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2043 Cards in this Set
- Front
- Back
Erythropoietin
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synthesized in peritubular capillaries
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Reticulocyte count
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measure of effective erythropoiesis; correct for degree of anemia
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Extramedullary hematopoiesis
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hematopoiesis outside bone marrow (e.g., spleen)
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Newborn physiologic anemia
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drop in Hb due to replacement of HbF RBCs with HbA
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Pregnancy
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Hb and Hct decreased; greater increase in plasma volume than RBC mass
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Anemia
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normal O2 saturation and arterial PO2
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MCV
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average volume of RBCs; useful for anemia classification
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MCHC
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average Hb concentration in RBCs
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MCHC
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↓ in microcytic anemias; ↑ in spherocytosis
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Thalassemias
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↓ MCV, ↑ RBC count
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RDW
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RBC size variation; ↑ iron deficiency; normal in other microcytic anemias
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Mature RBC
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anaerobic glycolysis; no mitochondria or HLA antigens
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Total iron binding capacity
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↑ iron deficiency; ↓ anemia chronic disease, sideroblastic anemia
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% Saturation
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↓ iron deficiency, anemia chronic disease; ↑ sideroblastic anemia
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Serum ferritin
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↓ iron deficiency; ↑ anemia chronic disease, sideroblastic anemia; normal thalassemia
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Microcytic anemias
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iron deficiency MC, anemic chronic disease, thalassemia, sideroblastic anemia
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Iron deficiency child
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MCC Meckel’s diverticulum
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Iron deficiency woman < 50
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MCC menorrhagia
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Iron deficiency man < 50
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MCC peptic ulcer disease
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Iron deficiency men/woman > 50
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MCC colon cancer
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Stages iron deficiency
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↓ ferritin; ↓ Fe and % saturation, ↑ TIBC; normocytic then microcytic anemia
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Anemia chronic disease
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MC anemia in malignancy and alcoholics
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α-Thalassemia trait
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AR; two α-globin gene deletions; normal Hb electrophoresis
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HbH disease
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three α-globin gene deletions; hemolytic anemia; four β-globin chains
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Hb Bart’s disease
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four α-globin gene deletions; four γ-globin chains
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β-Thalassemia minor
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AR; DNA splicing defect; ↑ HbA2 and F; ↓ HbA
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β-Thalassemia major
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nonsense mutation with stop codon; hemolytic anemia; ↑↑ HbF, ↑ HbA2
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Sideroblastic anemia
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defect in mitochondrial heme synthesis producing ringed sideroblasts
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Causes sideroblastic anemia
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alcohol, pyridoxine deficiency (isoniazid Rx of TB), Pb poisoning
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Pb poisoning
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inhibition ferrochelatase, d-aminolevulinic acid dehydrase, ribonuclease
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S/S Pb poisoning children
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growth retardation; Pb in epiphyses (lead lines); abdominal colic; encephalopathy
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S/S Pb poisoning adult
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peripheral neuropathy; proximal renal tubule damage (Fanconi’s syndrome)
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Lab Pb poisoning
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coarse basophilic stippling RBCs; ↓ MCV; ↑ blood Pb; ↑ d-aminolevulinic acid
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Vitamin B12
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animal products; requires intrinsic factor for reabsorption in terminal ileum
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Vitamin B12
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transfers methyl group to homocysteine
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R factor
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binds with B12 in mouth, removed by pancreatic enzymes in small intestine
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Vitamin B12
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involved in propionate metabolism; end-product succinyl CoA
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Causes B12 deficiency
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vegan, pernicious anemia MC, fish tapeworm, pancreatitis, bacterial overgrowth, Crohn’s disease
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Pernicious anemia
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autoimmune destruction parietal cells; chronic gastritis body/fundus; achlorhydria; ↑ gastrin
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Causes folate deficiency
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alcohol MCC, poor diet, drugs, malabsorption, pregnancy, goat milk
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Drugs and folate deficiency
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alcohol, OC, phenytoin, methotrexate, trimethoprim, 5-fluorouracil
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Intestinal conjugase in folate metabolism
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inhibited by phenytoin
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Jejunal uptake of monoglutamate form of folate
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inhibited by alcohol and OC
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Dihydrofolate reductase
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inhibited by methotrexate, trimethoprim
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Thymidylate synthetase
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inhibited by 5-fluorouracil
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Folate deficiency
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MCC of increased serum homocysteine
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Lab in B12/folate deficiency
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pancytopenia; hypersegmented neutrophils; ↑ homocysteine
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Lab findings unique to B12 deficiency
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↑ gastrin (pernicious anemia), ↑ methylmalonic acid
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B12 reabsorbed absorbed after administration of intrinsic factor
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PA
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B12 reabsorbed absorbed after administration of antibiotics
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bacterial overgrowth
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B12 reabsorbed absorbed after administration of pancreatic extract
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chronic pancreatitis
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Acute blood loss
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initially normal Hb and Hct; 0.9% saline uncovers RBC deficit
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Aplastic anemia
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drugs (e.g., phenylbutazone); infection (e.g., parvovirus); benzene
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Lab findings aplastic anemia
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pancytopenia; hypocellular bone marrow
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Anemia in renal disease
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normocytic; decreased EPO
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Extravascular hemolysis
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macrophage phagocytosis of RBCs; ↑ unconjugated bilirubin and urine UBG
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Intravascular hemolysis
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↓ serum haptoglobin; hemoglobinuria; hemosiderinuria
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Congenital spherocytosis
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AD; defect in spectrin; extravascular hemolysis; splenomegaly
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Blood findings in spherocytosis
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normocytic anemia; dense RBCs, ↑ MCHC, ↑ osmotic fragility
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PNH
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missing decay accelerating factor; complement destruction RBCs, neutrophils, platelets
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S/S PNH
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pancytopenia; hemoglobinuria; positive sugar water test and acidified serum test
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HbSS
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AR; missense mutation (valine for glutamic acid 6th positive β-globin chain)
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Causes of sickling
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↑ deoxyhemoglobin (hypoxemia, acidosis); HbS > 60%
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HbF
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inhibits sickling; hydroxyurea ↑ HbF
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Pathophysiology HbSS
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vasoocclusive crises, hemolytic anemia (extravascular)
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HbSS children
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dactylitis (6-9 months); Streptococcus pneumoniae sepsis (dysfunctional spleen)
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HbSS osteomyelitis
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Salmonella paratyphi
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HbSS complications
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aplastic crisis (parvovirus B-19), acute chest syndrome, autosplenectomy, calcium bilirubinate gallstones, priapism, aseptic necrosis
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HbAS
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microhematuria from sickling in renal medulla; renal papillary necrosis
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Hb electrophoresis
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HbAS-HbA 55-60%, HbS 40-45%; HbSS-HbS 90-95%, HbF 5-10%
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Blood findings in HbSS
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sickle cells; target cells; Howell-Jolly bodies (nuclear remnants)
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G6PD deficiency
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XR; oxidant damage (peroxide) to Hb (e.g., primaquine; dapsone; fava beans)
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Blood findings G6PD deficiency
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Heinz bodies (denatured Hb; special stain); bite cells
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Pyruvate kinase deficiency
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↓ ATP; RBCs dehydrated; ↑ 2,3-BPG (right-shifted OBC)
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Warm type AIHA
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IgG; extravascular hemolysis; e.g., SLE, drugs
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Cold type AIHA
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IgM intravascular hemolysis; e.g., CLL, Mycoplasma
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Penicillin
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IgG antibody against penicillin attached to RBC (type II hypersensitivity)
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Methyldopa
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drug alters Rh antigens; IgG antibody against Rh antigens (type II hypersensitivity)
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Quinidine
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drug-IgM IC; intravascular hemolysis; type III hypersensitivity
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Lab findings AIHA
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positive direct Coombs’; spherocytes
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Micro-macroangiopathic hemolysis
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mechanical damage causing intravascular hemolysis
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Causes of micro/macro hemolysis
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aortic stenosis (MCC), DIC, TIP, HUS
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Peripheral blood findings micro/macro hemolysis
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schistocytes; iron deficiency from hemoglobinuria
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Malaria
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intravascular hemolysis correlates with fever; falciparum-ring forms and gametocytes
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Leukemoid reaction
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exaggerated WBC response to infection; usually due to infection
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Leukoerythroblastic reaction
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marrow infiltrative disease peripheralizes myeloblasts/nucleated RBCs
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Causes of leukoerythroblastic reaction
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bone metastasis MCC, myelofibrosis
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Eosinophilia
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type I hypersensitivity (e.g., penicillin reaction); invasive helminthic infection
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Helminthes not producing eosinophilia
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pinworms, adult worms in ascariasis
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Atypical lymphocytes
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mononucleosis; CMV; toxoplasmosis; viral hepatitis; phenytoin
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Mononucleosis
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due to EBV; EBV attaches to CD21 receptors on B cells
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Clinical findings mono
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exudative tonsillitis, generalized lymphadenopathy, hepatosplenomegaly
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Lab findings mono
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atypical lymphocytosis; IgM heterophile antibodies against horse RBCs
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Lymphopenia
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T cell deficiencies (HIV); combined B/T deficiency (adenine deaminase deficiency)
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Lymphocytosis
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viral infections, whooping cough
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Corticosteroids
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lymphopenia, eosinopenia, neutrophilia
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Chronic MPD
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neoplastic stem cell disorder; splenomegaly; marrow fibrosis; risk for leukemia
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Examples of MPD
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polycythemia vera, myelofibrosis and myeloid metaplasia
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Relative polycythemia
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↓ plasma volume; ↑ RBC count; normal RBC mass
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Absolute polycythemia
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↓ RBC count and RBC mass
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Appropriate polycythemia
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hypoxic stimulus for EPO to generate RBCs
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Causes of appropriate absolute polycythemia
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lung disease, cyanotic heart disease, high altitude
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Appropriate absolute polycythemia
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normal plasma volume; ↑ RBC mass; ↓ SaO2; ↑ EPO
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Inappropriate absolute polycythemia
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no hypoxic stimulus for EPO
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Causes of inappropriate polycythemia
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ectopic secretion EPO, polycythemia vera
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Polycythemia vera
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↑ plasma volume and RBC mass; normal SaO2; ↓ EPO
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Ectopic EPO (renal cell carcinoma)
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normal plasma volume; ↑ RBC mass; normal SaO2; ↑ EPO
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Myelofibrosis myeloid metaplasia
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marrow fibrosis; extramedullary hematopoiesis; splenomegaly
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Lab findings in myelofibrosis
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tear drop RBCs; dry bone marrow aspirate (marrow fibrosis)
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Essential thrombocythemia
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MPO with increase in abnormal appearing platelets
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Myelodysplastic syndrome
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severe anemia in elderly; 30% develop leukemia; ringed sideroblasts
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Benzene
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aplastic anemia; acute leukemia
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Leukemia by age
|
ALL, newborn-14; AML, 15-60; CML, 40-60; CLL, >60
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Acute vs. chronic leukemia
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acute, blasts >30% in bone marrow; chronic, blasts <10% in bone marrow
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AML
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Auer rods in myeloblasts
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Acute promyelocytic leukemia
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t(15;17); defect in retinoic acid; Rx retinoic acid (↑ maturation); DIC
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Acute monocytic leukemia
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gum infiltration
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CML
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t(9;22) of ABL POC; Philadelphia chromosome 22; ↓ alkaline phosphatase score
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ALL
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early pre-B (80%); CALLA (CD10) and TdT positive; CNS and testicle involvement
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ALL
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t(12;21) offers good prognosis
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CLL
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B cell neoplasm; ↓ γ-globulins; MCC generalized lymphadenopathy patients> 60-yrs-old
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Adult T cell leukemia
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HTLV-1; CD4 T cells; skin infiltration; lytic bone lesions with hypercalcemia
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Hairy cell leukemia
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positive TRAP stain; splenomegaly; Rx with purine nucleosides
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Nodal sites
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germinal follicles, B cells; paracortex, T cells; sinuses, histiocytes
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Testicular cancer
|
metastasizes to para-aortic nodes
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Stomach cancer
|
metastasizes to left supraclavicular nodes (Virchow node)
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Phenytoin
|
atypical lymphocytosis
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Cat scratch disease
|
Bartonella henselae; granulomatous microabscesses
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Follicular B-cell lymphoma
|
t(14;18); overexpression of BCL-2 anti-apoptosis gene
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Burkitt lymphoma
|
t(8;14); EBV association; common childhood NHL; “starry sky” appearance
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Extra nodal lymphomas
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risk factors H. pylori (stomach); Sjogren’s syndrome
|
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Mycosis fungoides
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CD4 T cell neoplasm; skin lesions with Pautrier’s microabscesses
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Sezary syndrome
|
leukemic phase of mycosis fungoides
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Polyclonal gammopathy
|
sign of chronic inflammation
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Monoclonal gammopathy
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M component (spike); sign of plasma cell disorder
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Confirmatory tests
|
serum and urine immunoelectrophoresis; bone marrow aspirate
|
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Bence Jones protein
|
light chains in urine; predictive of a malignant plasma cell disorder
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Multiple myeloma
|
M spike; lytic bone lesions; pathologic fractures; hypercalcemia; renal failure
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MGUS
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MC monoclonal gammopathy; may progress to myeloma
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Findings in MGUS
|
elderly patient; no BJ protein; no malignant plasma cells
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Waldenstrom’s macroglobulinemia
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lymphoplasmacytic lymphoma; IgM M spike; hyperviscosity
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Hodgkin’s lymphoma
|
neoplastic component, Reed Stemberg (RS) cell; CD15 CD30 positive
|
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Lymphocyte predominant Hodgkin’s
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infrequent classic RS cells
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Nodular sclerosing Hodgkin’s
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female dominant; supraclavicular nodes + anterior mediastinal nodes
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Mixed cellularity Hodgkin’s
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male dominant; numerous RS cells; EBV association
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Hodgkin’s prognosis
|
stage of disease and type of Hodgkin’s most important factors
|
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Alkylating agents in Rx of Hodgkins
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↑ risk for second malignancies (leukemia; NHL)
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Langerhan’s histiocytes
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CD1 positive; Birbeck granules
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Letterer-Siwe disease
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malignant histiocytosis <2 yrs old; diffuse eczematous rash; organ involvement
|
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Hand-Christian-Christian disease
|
malignant; lytic skull lesions, diabetes insipidus, exophthalmos
|
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Eosinophilic granuloma
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benign histiocytosis; lytic bone lesions with pathologic fractures
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Mast cells
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release histamine (pruritus; swelling); metachromatic granules positive with toluidine blue
|
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Urticaria pigmentosum
|
localized mastocytosis; skin lesions swell and itch with scratching
|
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Amyloid
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twisted β-sheet; apple green birefringence with Congo red
|
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Primary amyloidosis
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AL amyloid derived from light chains; plasma cell disorders
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Secondary amyloidosis
|
AA amyloid derived from serum-associated amyloid; chronic infections
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Alzheimer’s disease
|
amyloid precursor protein gene product chromosome 21; amyloid-β
|
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Gaucher’s disease
|
macrophages have fibrillary appearance; deficiency glucocerebrosidase
|
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Niemann Pick’s disease
|
macrophages have soap bubble appearance; deficiency sphingomyelinase
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Hypersplenism
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splenomegaly; peripheral blood cytopenias; portal hypertension MCC
|
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Splenic dysfunction
|
Howell Jolly bodies; susceptible to Streptococcus pneumoniae sepsis
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Anticoagulants
|
tissue plasminogen activator, heparin, PGI2 ATIII, protein C/S
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Heparin
|
enhances ATIII activity (neutralizes all factors except V, VIII, fibrinogen)
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Protein C/S
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neutralize V and VIII
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Procoagulants
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coagulation factors, thromboxane A2 (platelet aggregation, vasoconstrictor)
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Protein C and S
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inactivate factors V and VIII; enhance fibrinolysis
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von Willebrand factor
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complexes with factor VIII to enhance VIII:C activity; platelet adhesion
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Platelets
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receptors for von Willebrand factor and fibrinogen; synthesize thromboxane A2
|
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GpIb
|
platelet receptor for von Willebrand factor
|
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GpIIb:IIIa
|
platelet receptor for fibrinogen
|
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Extrinsic system factor
|
VII
|
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Intrinsic system factors
|
XII, XI, IX, VIII
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Final common pathway factors
|
X, V, prothrombin (II), fibrinogen (I)
|
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Factor XIII
|
cross-links insoluble fibrin; strengthens fibrin clots
|
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Vitamin K-dependent factors
|
prothrombin, VII, IX, X, protein C and S
|
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Factors consumed in a clot
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fibrinogen, prothrombin, V, VIII; fluid is called serum
|
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Plasmin
|
cleaves fibrinogen and insoluble fibrin into degradation products
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Bleeding time
|
evaluates platelet function (adhesion, release reaction, aggregation)
|
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Aspirin
|
MCC of a prolonged bleeding time
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Tests for vWF
|
ristocetin cofactor assay; vWF antigen assay; agar electrophoresis
|
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PT
|
evaluates extrinsic pathway to fibrin clot
|
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PTT
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evaluates intrinsic pathway to stable fibrin clot
|
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Fibrinolysis tests
|
fibrin(ogen) degradation products; D-dimers (cross-linked insoluble fibrin)
|
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S/S platelet dysfunction
|
cannot form temporary plug; epistaxis; petechiae; bleeding from scratches
|
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Idiopathic thrombocytopenic purpura (ITP)
|
children; antibodies against GpIIb:IIIa; no splenomegaly
|
|
Chronic autoimmune thrombocytopenic purpura
|
SLE; antibodies against GpIIb:IIIa receptors
|
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Heparin
|
thrombocytopenia due to IgG antibody against heparin attached to PF4 on platelets
|
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PF4
|
heparin neutralizing factor
|
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HIV
|
thrombocytopenia MC hematologic abnormality; similar to ITP
|
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TTP
|
platelet thrombi develop in areas of endothelial damage in small vessels; consumption of platelets
|
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S/S
|
fever, thrombocytopenia, renal failure, hemolytic anemia with schistocytes, CNS deficits
|
|
Lab findings TTP
|
thrombocytopenia, prolonged bleeding time, normal PT and PTT
|
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HUS
|
similar to TTP; endothelial injury from Shiga-like toxin of 0157:H7 E. coli in undercooked beef
|
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S/S factor deficiency
|
no stable fibrin clot-late rebleeding; menorrhagia; GI bleeding; hemarthroses
|
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Hemophilia A
|
XR; hemarthroses; prolonged PTT, ↓ factor VIII activity, normal VIII antigen
|
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von Willebrand’s disease
|
AD; platelet adhesion defect + factor VIII deficiency
|
|
Lab findings in VWD
|
↓ vWF, VIII antigen, and VIII:C; prolonged bleeding time
|
|
Desmopressin acetate
|
Rx of choice for mild von Willebrand’s disease and hemophilia A
|
|
Circulating anticoagulants
|
antibodies destroy coagulation factors
|
|
Lab finding in circulating anticoagulant
|
prolonged PT and/or PTT corrected with mixing studies
|
|
Vitamin K deficiency
|
↓ epoxide reductase activity (↓ function vitamin K); hemorrhagic diathesis; ↑ PT
|
|
Causes vitamin K deficiency
|
antibiotics MC, newborn, malabsorption, warfarin
|
|
DIC
|
activation coagulation system from release of tissue thromboplastin and/or endothelial cell damage
|
|
DIC
|
consumption coagulation factors by fibrin clots; patient also anticoagulated
|
|
Causes
|
septic shock MCC, rattlesnake bite, massive trauma, amniotic fluid
|
|
S/S
|
bleeding from all scratches, holes, needle sites
|
|
Lab findings DIC
|
thrombocytopenia, ↑ PT and PTT, D-dimers (best test), anemia
|
|
Antiphospholipid antibodies
|
lupus anticoagulant and anticardiolipin antibodies; vessel thrombosis
|
|
Warfarin
|
inhibits epoxide reductase; PT best test but PTT also prolonged
|
|
Warfarin
|
full anticoagulation in 3 days when -carboxylated prothrombin disappears
|
|
Warfarin
|
ingredient in rat poison; danger to children in households with grandparents on warfarin
|
|
Rx warfarin over anticoagulation
|
intramuscular vitamin K (6-8 hrs), fresh frozen plasma (immediate)
|
|
Heparin
|
enhances ATIII; PTT best test but PT also prolonged
|
|
OC
|
estrogen ↑ coagulation factor synthesis and ATIII; predisposes to thrombosis
|
|
Factor V Leiden
|
MC hereditary thrombosis; resistant to degradation by protein C/S
|
|
ATIII deficiency
|
no prolongation of PTT with administration of heparin
|
|
Hemorrhagic skin necrosis
|
post-warfarin therapy in patient with heterozygote protein C deficiency
|
|
M cells
|
specialized cells that transfer foreign antigens to lymphocytes in Peyer’s patches
|
|
Blood group O
|
some patients have anti-AB-IgG antibodies; increased incidence duodenal ulcers
|
|
Blood group A
|
increased incidence of gastric carcinoma
|
|
Newborns
|
do not have natural blood group antibodies at birth (e.g., anti-A-lgM)
|
|
Elderly
|
may lose natural blood group antibodies; no hemolytic reaction to mismatched blood
|
|
Rh antigens
|
inherited in autosomal codominant fashion; Rh antigens include D, C, c, E, e
|
|
Atypical antibodies
|
antibodies against Rh or non-Rh blood group antigens (e.g., anti-D)
|
|
Duffy antigen
|
receptor for Plasmodium vivax; blacks often lack Duffy antigen
|
|
Antibody screen
|
indirect Coomb’s test; detects atypical antibodies in serum
|
|
Cytomegalovirus
|
MC infection transmitted by blood transfusion; MC antibody
|
|
Hepatitis C
|
MCC of post-transfusion hepatitis
|
|
Major crossmatch
|
patient serum reacted against donor RBCs; does not guarantee RBC survival
|
|
Universal donor
|
blood group O; no antigens on the surface of RBCs
|
|
Universal recipient
|
blood group AB; no natural blood group antibodies in serum
|
|
Packed RBC transfusion
|
raises Hb by 1 gm/dL and Hct by 3%
|
|
Cryoprecipitate
|
fibrinogen and factor VIII
|
|
Fresh frozen plasma
|
replacement for multiple factor deficiencies (e.g., cirrhosis, DIC)
|
|
Allergic transfusion reaction
|
type I IgE-mediated hypersensitivity reaction
|
|
Febrile transfusion reaction
|
recipient anti-HLA antibodies react against donor leukocytes
|
|
Intravascular HTR
|
transfusion of ABO incompatible blood (e.g., A person receives B blood)
|
|
Extravascular HTR
|
antibody attaches to donor RBCs; macrophage phagocytosis and hemolysis
|
|
Positive direct Coomb’s test
|
present in both types of hemolytic transfusion reactions
|
|
S/S
|
jaundice, no increase in Hb, hemoglobinuria
|
|
ABO HDN
|
mother O and baby A or B; transplacental passage of maternal anti-AB-IgG
|
|
ABO HDN
|
positive direct Coomb’s test; spherocytes; MCC unconjugated hyperbilirubinemia first 24 hrs
|
|
Rh HDN
|
mother Rh (D antigen) negative and fetus Rh (O antigen) positive
|
|
Rh HDN
|
no hemolysis in first Rh incompatible pregnancy
|
|
Rh HDN
|
maternal anti-D crosses placenta; potential for hydrops fetalis; high risk for kernicterus
|
|
Rh immune globulin
|
anti-D; coats D antigen site on fetal RBCs in maternal circulation
|
|
Rh HDN lab
|
positive direct Coomb’s; severe anemia and hyperbilirubinemia
|
|
ABO HDN
|
protects mother from Rh sensitization (development of anti-D antibodies)
|
|
O Rh negative mother with A Rh positive baby
|
A+ cells destroyed by mothers anti A-lgM
|
|
Blue fluorescent light
|
converts unconjugated bilirubin in skin into harmless water soluble dipyrrole
|
|
MV auscultation
|
apex
|
|
TV auscultation
|
left parasternal border
|
|
AV auscultation
|
right 2nd intercostal space
|
|
PV auscultation
|
left 2nd intercostal space
|
|
S1
|
closure MV and TV
|
|
S2
|
closure AV and PV
|
|
Inspiration
|
split in A2 and P2; due to increased blood in right side of heart
|
|
S3
|
abnormal; due to blood entering volume overloaded ventricle in early diastole
|
|
Causes S3
|
valve regurgitation; congestive heart failure
|
|
S4
|
abnormal; due to blood entering non-compliant ventricle with atrial contraction in late diastole
|
|
Causes S4
|
volume overloaded ventricle, hypertrophy
|
|
Murmurs
|
stretching valve ring or damage to valve
|
|
Inspiration
|
increases right sided abnormal heart sounds and murmurs
|
|
Expiration
|
increases left sided abnormal heart sounds and murmurs
|
|
Stenosis murmurs
|
problem in opening valve
|
|
Regurgitation murmurs
|
problem in closing valve
|
|
Valves opening in systole
|
AV and PV
|
|
Valves opening in diastole
|
MV and TV
|
|
Valves closing in systole
|
MV and TV
|
|
Valves closing in diastole
|
AV and PV
|
|
LDL
|
primary vehicle for carrying cholesterol
|
|
VLDL
|
primary vehicle for carrying liver-synthesized triglyceride
|
|
Familial hypercholesterolemia (type II)
|
AD; deficiency of LDL receptors; ↑ LDL
|
|
Type III hyperlipoproteinemia
|
deficiency apo E; ↑ remnants (chylomicron, intermediate density)
|
|
Type IV hyperlipoproteinemia
|
↑ VLDL; alcoholics
|
|
Apo B deficiency
|
deficiency apo B48 (chylomicrons) and B100 (VLDL); ↓ CH and TG
|
|
Clinical findings in apo B deficiency
|
malabsorption; hemolytic anemia
|
|
Atherosclerosis
|
reaction to injury of endothelial cells
|
|
Risk factors
|
smoking, ↑ LDL, ↑ homocysteine, Chlamydia pneumoniae infection
|
|
Cells involved
|
platelets, macrophages, smooth muscle cells, T cells with cytokine release
|
|
Fibrous plaque
|
pathognomonic lesion of atherosclerosis
|
|
C-reactive protein
|
marker of an inflammatory atheromatous plaque
|
|
Inflammatory atheromatous plaque
|
predisposes to platelet thrombosis
|
|
Increased plasma homocysteine
|
↑ vessel thrombosis; folate (MC)/vitamin B12 deficiency
|
|
Hyaline arteriolosclerosis
|
small vessel disease of DM and hypertension; excess protein in vessel wall
|
|
Mechanisms hyaline arteriolosclerosis in DM
|
non-enzymatic glycosylation
|
|
Non-enzymatic glycosylation
|
glucose attaches to amino acids in BM; causes ↑ permeability to protein
|
|
Mechanisms hyaline arteriolosclerosis in hypertension
|
pressure pushes proteins into vessel wall
|
|
Abdominal aortic aneurysm rupture
|
due to atherosclerosis; flank pain, hypotension, pulsatile mass
|
|
Syphilitic aneurysm
|
vasculitis of vasa vasorum of aortic arch; aortic regurgitation
|
|
Aortic dissection
|
due to hypertension and collagen tissue disorders (e.g., Marfan)
|
|
Cystic medial degeneration
|
elastic tissue degeneration creates spaces filled with mucopolysaccharides
|
|
Intimal tear in aorta
|
due to wall stress from hypertension and structural weakness
|
|
Types of dissection
|
proximal (MC); distal or combination of both
|
|
S/S proximal aortic dissection
|
chest pain radiating to back, lack of pulse; cardiac tamponade MC COD
|
|
Marfan’s
|
AD; fibrillin defect; aortic regurgitation/dissection; lens dislocation; MVP with sudden death
|
|
MC COD Marfan’s and Ehlers Danlos
|
aortic dissection
|
|
Phlebothrombosis
|
stasis of blood flow; deep veins below knee MC site
|
|
Pulmonary thromboembolism
|
emboli originate from femoral veins
|
|
Superficial migratory thrombophlebitis
|
sign of carcinoma of head of pancreas
|
|
Thoracic outlet syndrome
|
absent radial pulse with positional change
|
|
Turner’s syndrome
|
lymphedema hands/feet in newborn; preductal coarctation
|
|
Spider telangiectasia
|
arteriovenous fistula; due to hyperestrinism (cirrhosis, pregnancy)
|
|
Capillary hemangioma in newborn
|
regress with age; do not surgically remove
|
|
Kaposi’s sarcoma
|
HHV-8; vascular malignancy; MC cancer in AIDS
|
|
Bacillary angiomatosis
|
Bartonella henselae; vascular infection in AIDS
|
|
Small vessel vasculitis
|
palpable purpura; e.g., Henoch Schonlein purpura
|
|
Muscular artery vasculitis
|
vessel thrombosis with infarction; e.g., classical polyarteritis nodosa
|
|
Elastic artery vasculitis
|
absent pulse, stroke
|
|
Takayasu’s arteritis
|
pulseless disease; young Asian woman
|
|
Giant cell arteritis
|
temporal artery granulomatous vasculitis; ipsilateral blindness (ophthalmic artery)
|
|
Classical polyarteritis nodosa
|
muscular artery vasculitis with vessel thrombosis infarction
|
|
Path findings
|
vessel inflammation at different stages; aneurysms from vessel weakness
|
|
S/S
|
infarctions in kidneys, skin, GI tract, heart; HBsAg in 30%
|
|
Diagnosis
|
angiography identifies aneurysms and thrombosis
|
|
Kawasaki’s disease
|
coronary artery vasculitis/thrombosis/aneurysms in children
|
|
S/S
|
chest pain; desquamating rash; swelling hands/feet; cervical lymphadenopathy
|
|
Rx
|
IV γ-globulin
|
|
Buerger’s disease (thromboangiitis obliterans)
|
smoker’s digital vasculitis; digital infarction
|
|
Raynaud’s syndrome
|
digital vasculitis in PSS and CREST syndrome
|
|
S/S
|
digital pain; white-blue-red color changes
|
|
Cryoglobulinemia
|
protein gels in cold temperature; Raynaud’s syndrome; HCV association
|
|
S/S
|
acral cyanosis relieved by coming indoors
|
|
Wegener’s granulomatosis
|
association with c-ANCA; sinusitis, lung infarction, crescentic GN
|
|
Microscopic polyangiitis
|
palpable purpura; crescentic GN; association with p-ANCA
|
|
Henoch-Schönlein purpura
|
IgA-anti-IgA ICs; palpable purpura buttocks/legs; arthritis; IgA GN
|
|
Serum sickness vasculitis
|
e.g., horse antivenin in Rx of rattlesnake envenomation
|
|
Rocky Mountain spotted fever
|
tick borne
|
|
Meningococcemia
|
sepsis causes petechia/ecchymoses; potential for Waterhouse Friderichsen syndrome
|
|
Essential HTN blacks
|
defect in renal excretion of sodium; ↑ plasma volume, ↓ PRA
|
|
Renovascular HTN
|
atherosclerosis renal artery in men; fibromuscular hyperplasia renal artery women
|
|
S/S
|
epigastric bruit; ↑ PRA affected kidney, ↓ PRA unaffected kidney
|
|
Endocrine HTN
|
1º HPTH, Graves/hypothyroidism, Cushing’s, 1º aldosteronism, phaeochromocytoma
|
|
Hypertension
|
LVH MC complication; AMI MC COD followed by stroke and renal failure
|
|
Afterload
|
resistance ventricles contract against
|
|
Preload
|
volume ventricles must eject
|
|
Concentric LVH
|
increased afterload; e.g., essential HTN, aortic stenosis
|
|
LVH with dilation/hypertrophy
|
increased preload; e.g., valve regurgitation; left to right shunts
|
|
LHF
|
forward failure; pulmonary edema, pillow orthopnea, paroxysmal nocturnal dyspnea
|
|
Systolic dysfunction
|
LHF due to decreased ventricular contractility (ischemia)
|
|
Diastolic dysfunction
|
LHF due to decreased ventricular compliance (hypertrophy)
|
|
RHF
|
backward failure; ↑ venous hydrostatic pressure; neck vein distention, hepatomegaly, edema
|
|
ACE inhibitors
|
decrease afterload and preload in heart failure
|
|
Diuretics in CHF
|
reduce preload
|
|
Non-pharmacologic Rx in CHF
|
restrict salt and water
|
|
AMI
|
MC COD in United States; left anterior descending coronary artery thrombosis MCC
|
|
Exertional angina
|
coronary artery atherosclerosis; subendocardial ischemia; ST depression
|
|
Prinzmetal’s angina
|
coronary artery vasospasm; transmural ischemia; ST elevation
|
|
Sudden cardiac death
|
death within 1 hr of symptoms
|
|
Path findings
|
severe coronary artery atherosclerosis; absence of occlusive thrombosis
|
|
LAD coronary artery
|
anterior portion left ventricle, anterior 2/3rds IVS
|
|
RCA
|
posterior portion left ventricle and papillary muscle, inferior 1/3rd IVS, right ventricle
|
|
AMI
|
rupture of inflammatory plaque produces platelet thrombus
|
|
Ventricular fibrillation
|
MC COD in AMI
|
|
AMI
|
no gross changes until 24 hrs
|
|
S/S AMI
|
retrosternal pain radiating down arms, diaphoresis
|
|
AMI ruptures
|
3rd-7th day
|
|
Anterior wall rupture
|
MC type; LAD thrombosis; cardiac tamponade
|
|
Posteromedial papillary muscle rupture
|
RCA thrombosis; mitral regurgitation with LHF
|
|
IVS rupture
|
LAD thrombosis; left to right shunt; RHF
|
|
Mural thrombus
|
anterior AMI; danger embolization
|
|
Pericarditis
|
first week in transmural AMI; 6 wks later autoimmune
|
|
S/S
|
friction rub; leaning forward relieves pain
|
|
Ventricular aneurysms
|
late manifestation of AMI; precordial systolic bulge; CHF MC COD
|
|
Right ventricular infarction
|
RCA thrombosis; hypotension, RHF, preserved left ventricular function
|
|
Diagnosis of AMI
|
CK-MB and troponins; CK-MB absent by 3 days; troponins last 7-10 days
|
|
LDH isoenzymes
|
no longer used; LDH 1/2 flip indicates AMI
|
|
Reinfarction
|
reappearance CK-MB after 3 days
|
|
ECG findings in AMI
|
inverted T waves; ST elevation; Q waves
|
|
Ejection fraction
|
EF = stroke volume/left ventricular end-diastolic volume; 80/120 = 0.66
|
|
By-pass surgery
|
use internal mammary artery and saphenous veins (“arterialize” after 10 yrs)
|
|
Angioplasty complication
|
localized dissection with thrombosis
|
|
Umbilical vein
|
highest O2 saturation
|
|
Ductus arteriosis in fetus
|
shunts blood from pulmonary artery to aorta; PGE keeps it open
|
|
Ductus arteriosus in newborn
|
closes and becomes ligamentum arteriosum
|
|
Eisenmenger’s syndrome
|
cyanosis due to reversal of left to right shunt
|
|
VSD
|
MC congenital heart disease; ↑ SaO2 right ventricle (RV), pulmonary artery (PA)
|
|
ASD
|
patent foramen ovale; ↑ SaO2 right atrium (RA), RV, PA; MC adult congenital heart disease
|
|
Down syndrome
|
endocardial cushion defect (combined ASD and VSD)
|
|
PDA
|
machinery murmur; close with indomethacin; ↑ SaO2 PA
|
|
Tetralogy of Fallot
|
degree of pulmonic stenosis determines if cyanosis is present
|
|
Tetralogy of Fallot
|
↓ left ventricle, aorta
|
|
Tetralogy of Fallot
|
ASD and PDA are cardioprotective
|
|
Complete transposition
|
cyanosis; aorta empties RV; PA empties left ventricle
|
|
Complications cyanotic heart disease
|
2° polycythemia; infective endocarditis; metastatic abscesses
|
|
Pre-ductal coarctation
|
Turner’s syndrome
|
|
Post-ductal coarctation
|
constriction distal to ligamentum arteriosum
|
|
S/S
|
upper extremity HTN; claudication; rib-notching; activation RAA also causes HTN
|
|
Acute rheumatic fever
|
type II hypersensitivity; group A streptococcus pharyngeal infection
|
|
Acute rheumatic fever
|
sterile vegetations mitral valve (regurgitation); myocarditis with Aschoff nodule
|
|
S/S
|
polyarthritis (MC), carditis, erythema marginatum, rheumatoid nodules, chorea
|
|
Mitral stenosis
|
chronic rheumatic fever; opening snap followed by mid-diastolic rumble
|
|
Mitral stenosis
|
left atrial dilation hypertrophy - atrial fibrillation, thrombus, pulmonary edema, RHF
|
|
MVP
|
myxomatous degeneration of mitral valve; common in Marfan syndrome, Ehlers Danlos
|
|
S/S
|
mid-systolic click followed by a murmur; palpitations, chest pain, rupture of chordae
|
|
MVP click/murmur close to S1
|
decrease preload (stand, Valsalva, anxiety)
|
|
MVP click/murmur close to S2
|
increase preload (supine, squat, clench fist)
|
|
Mitral regurgitation
|
pansystolic murmur; S3 and S4 common
|
|
Causes
|
LHF, infective endocarditis, acute rheumatic fever
|
|
Aortic stenosis
|
systolic ejection murmur; syncope and angina with exercise; hemolytic anemia
|
|
Aortic stenosis murmur increased preload
|
worsens obstruction and increases murmur intensity
|
|
Aortic stenosis murmur decreased preload
|
decreases obstruction and decreases murmur intensity
|
|
Causes
|
bicuspid aortic valve; age-related sclerosis
|
|
Aortic regurgitation
|
bounding pulses; early diastolic blowing murmur
|
|
Austin Flint murmur
|
diastolic murmur; regurgitant flow on anterior leaflet mitral valve
|
|
Significance Austin Flint murmur
|
sign for AV replacement
|
|
Causes aortic regurgitation
|
essential HTN, infective endocarditis, acute rheumatic fever, dissection
|
|
Tricuspid regurgitation
|
pansystolic murmur ↑ intensity with inspiration
|
|
Causes
|
endocarditis IV drug abuse; RHF; carcinoid heart disease
|
|
Carcinoid heart disease
|
tricuspid regurgitation, pulmonic stenosis
|
|
Infective endocarditis (IE)
|
Streptococcus viridans MCC; Staphylococcus aureus MCC IVDA
|
|
IE prosthetic heart valve
|
Staphylococcus epidermidis (coagulase negative)
|
|
IE ulcerative bowel disease
|
Streptococcus bovis
|
|
S/S
|
IC vasculitis-Roth spot, splinter hemorrhages; regurgitant murmurs; metas1atic abscesses
|
|
Lab findings
|
positive blood culture Libman Sacks endocarditis
|
|
Coxsackievirus
|
MCC of myocarditis (lymphocyte infiltrate in myocardium) and pericarditis
|
|
Parasitic cause myocarditis
|
leishmania in Chagas disease
|
|
Pericardial effusion
|
all chamber pressures are uniformally increased
|
|
S/S
|
muffled heart sounds, pulsus paradoxus, inspiratory neck vein distention
|
|
Dx and Rx
|
echocardiogram, pericardiocentesis, respectively
|
|
Pulsus paradoxus
|
drop in blood pressure >10 mmHg with inspiration
|
|
Constrictive pericarditis
|
TB MCC worldwide; pericardial knock
|
|
Congestive cardiomyopathy
|
generalized chamber enlargement; low ejection fraction
|
|
Causes
|
postpartum, cardiotoxic drugs, hypothyroidism, alcohol
|
|
Hypertrophic cardiomyopathy
|
MCC of sudden death in young person (due to conduction defects)
|
|
Site of obstruction
|
anterior leaflet mitral valve drawn against asymmetric thickened IVS
|
|
Effect decreased preload on systolic murmur
|
worsens obstruction and increases murmur intensity
|
|
Effect increased preload on systolic murmur
|
reduces obstruction and decreases murmur intensity
|
|
Restrictive cardiomyopathy
|
decreased compliance
|
|
Causes
|
iron, amyloid, glycogen; sarcoidosis; tropical endocardial fibrosis
|
|
Cardiac myxoma
|
benign tumor left atrium; embolization; syncope
|
|
Cardiac rhabdomyoma
|
childhood tumor; association with tuberous sclerosis
|
|
U wave
|
hypokalemia; MCC diuretic therapy (e.g., thiazides; loop diuretics)
|
|
Peaked T wave
|
hyperkalemia; MCC renal failure
|
|
ST depression
|
subendocardial ischemia (e.g., classical angina pectoris)
|
|
ST elevation
|
transmural ischemia (e.g., AMI), pericarditis, ventricular aneurysm
|
|
Atrial fibrillation
|
MC chronic arrhythmia; absent P waves; danger for embolization
|
|
Ventricular premature beats
|
wide QRS complexes; MC arrhythmia in coronary care unit
|
|
Ventricular fibrillation
|
MCC of death in an AMI
|
|
Anterior AMI
|
Q waves in I and V1-V4
|
|
Inferior AMI
|
Q waves in II, III, and aVF; right coronary artery thrombosis.
|
|
Wolff-Parkinson-White
|
short PR interval with normal P wave; delta wave on upstroke of R wave
|
|
Alveolar O2 calculation
|
% O2 breathing (713) - PCO2/0.8
|
|
Increased A-a gradient
|
primary lung disease; left to right shunts in heart
|
|
Forced vital capacity
|
total amount of air expelled after a maximal inspiration
|
|
Forced expiratory volume/1 second (FEV1)
|
amount of air expelled in I second after maximal inspiration
|
|
Choanal atresia
|
cyanotic when breast feeding; turns pink when crying
|
|
Nasal polyps
|
allergic (MC; adults only), aspirin, cystic fibrosis
|
|
Nasal polyp in a child
|
requires sweat test to exclude cystic fibrosis
|
|
Triad asthma
|
patient on aspirin (pain syndrome) with nasal polyps, asthma
|
|
Obstructive sleep apnea (OSA)
|
snoring with intervals of apnea (respiratory acidosis with hypoxemia)
|
|
S/S
|
danger cor pulmonale; requires sleep test; Rx. O2 with continuous positive airway pressure
|
|
Sinusitis
|
maxillary sinusitis MC in adults; ethmoiditis MC in children; S. pneumoniae MC
|
|
Nasopharyngeal carcinoma
|
association with EBV; metastasize to cervical nodes
|
|
Laryngeal carcinoma
|
smoking MCC; hoarseness; squamous cell carcinoma
|
|
Resorption atelectasis
|
MCC of fever 24-36 hours after surgery
|
|
S/S
|
↓ percussion; absent fremitus, breath sounds; inspiratory lag; elevated diaphragm
|
|
RDS
|
decreased production surfactant; airway collapse; hyaline membranes
|
|
Type II pneumocytes
|
synthesize surfactant (lecithin, phosphatidylcholine); stored in lamellar bodies
|
|
Surfactant
|
reduces surface tension in airways; ↑ synthesis cortisol, thyroxine; ↓ synthesis insulin
|
|
Causes RDS
|
prematurity, maternal diabetes, C-section
|
|
Maternal diabetes
|
maternal hyperglycemia → fetal hyperglycemia → fetal insulin which ↓ surfactant
|
|
Complications RDS
|
O2 FR injury (blindness, bronchopulmonary dysplasia); necrotizing enterocolitis
|
|
Typical community acquired pneumonia
|
Streptococcus pneumoniae MCC
|
|
Typical pneumonia
|
bronchopneumonia, lobar pneumonia
|
|
S/S
|
productive cough; consolidation - ↓ percussion, ↑ tactile fremitus
|
|
Atypical community acquired pneumonia
|
interstitial pneumonia; Mycoplasma pneumoniae MCC
|
|
S/S
|
low grade fever, non-productive cough, no signs consolidation
|
|
Nosocomial pneumonia
|
Pseudomonas, aeruginosa MCC (respirators); others - S. aureus, E. coli
|
|
Rhinovirus
|
MCC common cold; hand to mouth transmission
|
|
Respiratory syncytial virus
|
MCC pneumonia and bronchiolitis in child
|
|
Parainfluenza virus
|
MCC croup in child; trachea area of obstruction
|
|
Cytomegalovirus
|
basophilic intranuclear inclusion surrounded by halo
|
|
Influenza
|
superimposed pneumonia with S. aureus increases mortality
|
|
Rubeola
|
Warthin-Finkeldey multinucleated giant cells
|
|
Chlamydia pneumoniae
|
atypical pneumonia; association with coronary artery disease
|
|
Chlamydia trachomatis
|
pneumonia in newborns; staccato cough; wheezing
|
|
Coxiella burnetii
|
only rickettsia without a vector
|
|
Mycoplasma pneumoniae pneumonia
|
crowded condition; cold agglutinins; azithromycin
|
|
Streptococcus pneumoniae pneumonia
|
gram positive diplococcus; azithromycin
|
|
Staphylococcus aureus pneumonia
|
tension pneumatocysts in children with cystic fibrosis
|
|
Corynebacterium diphtheriae
|
toxin produces ADP ribosylation of elongation factor 2
|
|
Haemophilus influenzae
|
exacerbation chronic bronchitis; acute epiglottis in children
|
|
Inspiratory stridor child
|
croup, epiglottitis
|
|
Pseudomonas aeruginosa
|
MCC of pneumonia and death in cystic fibrosis; green sputum
|
|
Klebsiella pneumoniae
|
mucoid sputum in alcoholic
|
|
Legionella pneumophila
|
silver stain; water coolers/mist (grocery produce, restaurants, zoo rain forest)
|
|
Mycobacterium tuberculosis
|
strict aerobe; MC COD due to infectious disease worldwide
|
|
Candida albicans
|
vessel invader; yeasts and pseudohyphae
|
|
Cryptococcus immitis
|
pigeon excreta; narrow-based bud
|
|
Aspergillus fumigatus
|
septate hyphae with fruiting body; fungus ball, extrinsic asthma, vessel invader
|
|
Mucor species
|
non-septate; vessel invader; frontal lobe abscess in diabetic ketoacidosis
|
|
Coccidioides immitis
|
Southwest deserts; inhale arthrospores in dust; spherule with endospores
|
|
S/S
|
erythema nodosum (painful nodules lower legs)
|
|
Histoplasma capsulatum
|
Ohio/central Mississippi river valley; excreta bats (spelunker), chickens
|
|
H. capsulatum
|
simulates TB; yeasts phagocytosed by macrophages
|
|
Blastomyces dermatitidis
|
overlaps histoplasmosis; broad-based buds; skin lesion simulates cancer
|
|
Pneumocystis carinii
|
cysts and trophozoites; pneumonia in HIV; Rx. trimethoprim/sulfamethoxazole
|
|
Primary TB
|
upper portion lower lobe, lower portion upper lobe
|
|
Primary TB
|
Ghon focus (subpleural caseation); Ghon complex (spread to hilar nodes)
|
|
Reactivation TB
|
cavitating lesion in upper lobe; kidney MC extrapulmonary site
|
|
Mycobacterium avium intracellulare (MAI)
|
atypical TB; MC TB in AIDS
|
|
CF
|
AR; 3 nucleotide deletion chromosome 7; defective CFTR (degraded in Golgi apparatus)
|
|
S/S
|
pneumonia, malabsorption, males sterile; + sweat test; P. aeruginosa pneumonia MC COD
|
|
Lung abscess
|
MCC aspiration oropharyngeal material (mixed aerobe/anaerobe); x-ray - air/fluid level
|
|
Aspiration sitting
|
posterobasal segment right lower lobe
|
|
Aspiration supine
|
superior segment right lower lobe
|
|
Aspiration right side
|
right middle lobe, posterior segment right upper lobe
|
|
Pulmonary thromboembolism
|
most derive from femoral vein
|
|
Bronchial artery
|
branch of aorta/intercostal artery; protects against developing pulmonary infarction
|
|
Saddle embolus
|
sudden death due to acute right heart strain
|
|
S/S pulmonary infarction
|
dyspnea and tachypnea; pleuritic chest pain; pleural effusion
|
|
Dx
|
ventilation/perfusion scan; respiratory alkalosis; hypoxemia
|
|
Pain on inspiration
|
pleuritic inflammation; pulmonary embolus, pneumonia, pneumothorax
|
|
Pathogenesis pulmonary hypertension (PH)
|
hypoxemia and respiratory acidosis
|
|
Hypoxemia + respiratory acidosis
|
vasoconstriction pulmonary vessels; vasodilation cerebral vessels
|
|
Causes PH
|
1° lung disease (COPD, restrictive), recurrent emboli, mitral stenosis, OSA, left-right shunts
|
|
Cor pulmonale
|
PH + RVH
|
|
S/S
|
dyspnea; accentuated P2 (PH); parasternal heave (RVH)
|
|
Restrictive lung disease
|
↓ compliance, ↑ elasticity; interstitial fibrosis/edema
|
|
Restrictive lung disease (RLD)
|
↓ all volumes and capacities; ↑ FEV1sec/FVC ratio
|
|
ARDS
|
RLD; non-cardiogenic pulmonary edema due to alveolar injury
|
|
ARDS
|
neutrophil destruction of type I and II pneumocytes; hyaline membranes
|
|
Causes
|
septic shock (MC), aspiration gastric contents, severe trauma
|
|
Pneumoconiosis
|
inhalation mineral dust causing interstitial fibrosis; particles <0.5 μm to reach alveoli
|
|
Caplan syndrome
|
pneumoconiosis + rheumatoid nodules in lungs
|
|
Coal worker’s
|
“black lung” disease; progressive massive fibrosis; no increased incidence cancer or TB
|
|
Silicosis
|
quartz; nodular opacities; foundry workers; ↑ incidence cancer and TB
|
|
Sources asbestos
|
roofing material, old buildings (9/11), pipe-fitter shipyard
|
|
Ferruginous bodies
|
asbestos fiber coated by iron
|
|
Asbestosis
|
benign pleural plaques (MC); bronchogenic carcinoma (MC cancer); mesothelioma
|
|
Mesothelioma
|
malignancy of serosa; no smoking association
|
|
Sarcoidosis
|
RLD; MC non-infectious lung and liver granulomatous disease
|
|
S/S
|
dyspnea, hilar adenopathy (non-caseating granulomas), uveitis, nodular skin lesions
|
|
Lab findings
|
↑ ACE, hypercalcemia (macrophages synthesize 1-α-hydroxylase)
|
|
Kveim test
|
intradermal injection sarcoid antigens causes skin reaction
|
|
Farmer’s lung
|
RLD; lung reaction against thermophilic bacteria in moldy hay
|
|
Silo filler’s disease
|
RLD; reaction against nitrogen dioxide in fermenting corn
|
|
Byssinosis
|
RLD; reaction against cotton, linen, hemp products in textile industry
|
|
Goodpasture’s syndrome
|
RLD; anti-BM antibodies; begins in lungs and ends in renal failure
|
|
Collagen vascular RLD
|
SLE, rheumatoid arthritis, systemic sclerosis
|
|
Drugs RLD
|
amiodarone, bleomycin, busulfan, cyclophosphamide, methotrexate, nitrofurantoin
|
|
Obstructive lung disease
|
↑ compliance, ↓ elasticity
|
|
Obstructive lung disease
|
↑ RV, TLC; ↓ TV, VC; ↓ FEV1sec and FVC; ↓ FEV1sec/FVC ratio
|
|
Obstructive lung disease
|
asthma, emphysema, chronic bronchitis, bronchiectasis
|
|
Asthma
|
extrinsic (type I hypersensitivity) and intrinsic types
|
|
S/S
|
expiratory wheezing (inflamed terminal bronchioles); LTC4,-D4,-E4 bronchoconstrictors
|
|
Charcot-Leyden crystals
|
derive from crystalline material in eosinophil granules
|
|
Lab findings
|
initial respiratory alkalosis; respiratory acidosis requires intubation
|
|
Emphysema
|
destruction elastic tissue respiratory unit; lung hyperinflation; smoking MCC; pink puffer
|
|
Respiratory unit
|
respiratory bronchiole, alveolar duct, alveoli
|
|
Radiograph emphysema
|
↑ AP diameter; depressed diaphragms; vertical heart
|
|
Pathogenesis
|
↓ AAT, ↑ neutrophil destruction of elastic tissue
|
|
Centriacinar emphysema
|
destruction/distention respiratory bronchioles upper lobe in smokers; THINK
|
|
Panacinar emphysema
|
destruction/distention entire respiratory unit lower lobes; AAT deficiency
|
|
Paraseptal emphysema
|
upper lobe destruction/distention alveolar ducts, alveoli; pneumothorax
|
|
Lab findings
|
normal to decreased PCO2 (respiratory alkalosis)
|
|
Chronic bronchitis
|
productive cough 3 months/2 consecutive years; blue bloater (cyanosis)
|
|
Site of obstruction
|
terminal bronchioles (proximal to respiratory unit)
|
|
Lab findings
|
respiratory acidosis/hypoxemia
|
|
Bronchiectasis
|
obstruction/infection key causes; dilated bronchioles extend to periphery
|
|
Causes
|
CF MCC, TB, immotile cilia syndrome
|
|
Immotile cilia syndrome
|
absent dynein arm in cilia; sinusitis, infertility, bronchiectasis, situs inversus
|
|
Central lung cancers
|
squamous cancer and small cell cancer; men > women
|
|
Peripheral lung cancers
|
adenocarcinoma; women > men
|
|
Squamous lung cancer
|
cavitate; secrete PTH-related protein
|
|
Small cell carcinoma
|
neuroendocrine tumor; secrete ACTH and ADH
|
|
Bronchioloalveolar carcinoma
|
no smoking relationship; lung consolidation resembling pneumonia
|
|
Scar carcinoma
|
usually adenocarcinoma developing in old TB scar
|
|
Bronchial carcinoid
|
low grade malignant; hemoptysis; rare cause carcinoid syndrome
|
|
Metastatic lung cancer
|
more common than primary cancer; breast cancer MCC
|
|
Pancoast tumor
|
squamous carcinoma posterior mediastinum; destruction superior cervical ganglion
|
|
S/S
|
Horner’s syndrome - lid lag, miosis, anhydrosis; lower brachial plexus injury
|
|
Solitary coin lesion
|
granuloma MCC
|
|
Superior vena caval syndrome
|
primary lung cancer obstructs vessel; venous congestion
|
|
Anterior mediastinal masses
|
thymoma; nodular sclerosing Hodgkin’s; teratomas
|
|
Posterior mediastinal masses
|
usually neurogenic tumors of ganglia
|
|
Myasthenia gravis
|
B cell hyperplasia of thymus MC abnormality; association with thymoma
|
|
Thymoma
|
association with hypogammaglobulinemia, autoimmune disease, pure RBC aplasia
|
|
Pleural effusions
|
transudates or exudates; CHF MCC
|
|
Spontaneous pneumothorax
|
rupture subpleural or intrapleural bleb; air/pleural cavity pressure same
|
|
S/S
|
pleuritic chest pain, dyspnea, tracheal shift ipsilateral side, absent breath sounds
|
|
Tension pneumothorax
|
flap-like pleural tear; increased pleural cavity pressure; compression atelectasis
|
|
S/S
|
as above except tracheal shift to opposite side
|
|
Cleft lip/palate
|
failure of fusion of facial processes
|
|
Herpes labialis
|
multinucleated giant cell with acidophilic intranuclear inclusions on Tzanck prep
|
|
Hairy leukoplakia
|
EBV glossitis; pre-AIDS defining lesion; not precursor to cancer
|
|
Mumps
|
bilateral parotitis; unilateral orchitis; ↑ amylase
|
|
Diphtheria
|
pseudomembrane pharynx and trachea with cervical lymphadenopathy
|
|
Congenital syphilis
|
notched central incisors
|
|
Actinomycosis
|
anaerobic gram + filamentous bacteria; complication extracted dental abscess
|
|
S/S
|
draining sinuses with sulfur granules
|
|
Exudative tonsillitis
|
majority are viral; 20% group A streptococcus
|
|
Oral thrush
|
common in newborn; pre-AIDS defining lesion; yeasts and pseudohyphae
|
|
Dental caries
|
Streptococcus mutans
|
|
Peutz-Jegher’s syndrome
|
mucosal pigmentation; hamartomatous polyps
|
|
Leukoplakia/erythroleukoplakia
|
biopsy to rule out squamous dysplasia or cancer
|
|
Squamous cell carcinoma
|
smoking and alcohol association; lower lip MC site
|
|
Smokeless tobacco
|
verrucoid squamous cell carcinoma
|
|
Gum hyperplasia
|
phenytoin, pregnancy, scurvy
|
|
Pleomorphic adenoma
|
MC benign tumor of salivary glands; parotid MC site
|
|
Mucoepidermoid carcinoma
|
MC malignant tumor major and minor salivary glands
|
|
Dysphagia for solids only
|
lesion obstructing esophagus; e.g., cancer, web
|
|
Plummer-Vinson syndrome
|
iron deficiency anemia causes esophageal web, glossitis, achlorhydria (↓ HCl in gastric acid)
|
|
Dysphagia for solids and liquids
|
motor abnormality; e.g., achalasia MCC, PSS or CREST syndrome
|
|
TE fistula
|
polyhydramnios; proximal esophagus ends blindly; distal esophagus derives from trachea
|
|
VATER syndrome
|
vertebral abnormalities, anal atresia, TE fistula, renal disease/radius abnormality
|
|
Zenker’s diverticulum
|
MC pulsion diverticulum of esophagus; halitosis (stinky breath, food gets stuck); near UES
|
|
GERD
|
relaxation of lower esophageal sphincter (LES) with acid reflux
|
|
GERD
|
MCC nocturnal cough and asthma
|
|
AIDS esophagitis
|
Candida MC, CMV, HSV
|
|
Barrett’s esophagus
|
glandular metaplasia distal esophagus in GERD
|
|
Complications of Barrett’s
|
precursor for adenocarcinoma, stricture
|
|
Esophageal varices
|
dilated left gastric vein; sign of portal hypertension due to cirrhosis
|
|
Mallory Weiss syndrome
|
tear of distal esophagus from retching in alcoholic or bulimic
|
|
Boerhaave’s syndrome
|
rupture of distal esophagus from retching; pneumomediastinum
|
|
Hamman’s mediastinal crunch
|
pneumomediastinum (air in subcutaneous tissue)
|
|
LES ganglion cells
|
contain VIP - relaxes LES
|
|
Achalasia
|
failure of LES relaxation (no VIP); absent ganglion cells in the myenteric plexus
|
|
S/S
|
aperistalsis/dilation of esophagus; regurgitation of undigested food at night
|
|
X-ray achalasia
|
bird’s beak appearance
|
|
Acquired achalasia
|
Chagas’ disease; leishmania destroy ganglion cells
|
|
Distal adenocarcinoma esophagus
|
MC primary cancer; due to Barrett’s esophagus
|
|
Squamous cell carcinoma of esophagus
|
smoking MCC; alcohol also causes
|
|
Melena
|
sign of upper GI bleed; acid changes Hb to hematin; peptic ulcer disease MCC
|
|
Hematemesis
|
vomiting blood; peptic ulcers MCC
|
|
Congenital pyloric stenosis
|
hypertrophy pyloric muscle; vomiting non-bile stained fluid in 2-4 weeks
|
|
Acute hemorrhagic (erosive) gastritis
|
NSAIDs MCC
|
|
Mucous barrier stomach
|
maintained by PGE; misoprostol PGE analog
|
|
Type A chronic gastritis
|
due to PA; achlorhydria with ↑ serum gastrin
|
|
Type B chronic gastritis
|
due to H. pylori; involves pylorus and antrum
|
|
H. pylori
|
curved rod; urease producer; MCC PUD, adenocarcinoma, gastric lymphoma
|
|
Gastric ulcer
|
lesser curvature pylorus and antrum; poor defense against acid; food aggravates pain
|
|
Duodenal ulcer
|
never malignant; ↑ acid production; food relieves pain
|
|
Perforated peptic ulcer
|
air under diaphragm causes pain in left shoulder
|
|
Menetrier’s disease
|
giant rugal hyperplasia; protein loss from increased mucus
|
|
Zollinger-Ellison syndrome
|
malignant islet cell tumor secreting gastrin; part of MEN I syndrome
|
|
S/S
|
PUD in usual locations; sometimes multiple ulcers
|
|
Hypergastrinemia
|
ZE, achlorhydria, gastric distention, H2 or proton blockers; renal failure
|
|
Leiomyoma
|
MC benign tumor of stomach
|
|
Intestinal type adenocarcinoma
|
H pylori related; ↓ incidence; lesser curvature pylorus/antrum
|
|
Diffuse type adenocarcinoma
|
linitis plastica; signet ring cells; Krukenberg tumors ovaries
|
|
Gastric lymphoma
|
stomach MC site for extranodal lymphomas; H. pylori associated
|
|
Malabsorption
|
steatorrhea; chronic pancreatitis, bile salt deficiency, small bowel disease
|
|
Causes bile salt deficiency
|
liver disease, bile salt resins, cholestasis, bacterial overgrowth, Crohn’s
|
|
D-xylose screen
|
failure to reabsorb xylose indicates small bowel disease
|
|
Calcification of pancreas
|
chronic pancreatitis cause of malabsorption
|
|
Celiac disease
|
autoimmune disease; antibodies against gliadin in gluten; flat villi
|
|
Celiac disease
|
association with dermatitis herpetiformis
|
|
Whipple’s disease
|
systemic infection; foamy macrophages with bacteria (PAS+ inclusions) in small bowel submucosa
|
|
S/S
|
fever, polyarthritis, skin pigmentation
|
|
Invasive diarrhea
|
Campylobacter jejuni MCC; positive fecal smear for leukocytes
|
|
Secretory diarrhea
|
loss isotonic fluid; enterotoxins from E. coli and V. cholerae
|
|
Osmotic diarrhea
|
hypotonic loss fluid; laxatives, lactase deficiency
|
|
Rotavirus
|
MCC diarrhea in children
|
|
Norwalk virus
|
MCC diarrhea in adults
|
|
Cytomegalovirus
|
common cause diarrhea in AIDS; MCC cholecystitis and pancreatitis in AIDS
|
|
Staphylococcus aureus
|
preformed toxin causes food poisoning; culture food
|
|
Bacillus cereus
|
preformed toxin in fried rice and tacos; gram positive rods in stool
|
|
Clostridium botulinum (adult)
|
preformed neurotoxin (blocks acetylcholine release); paralysis and mydriasis
|
|
Clostridium botulinum (child)
|
colonization of bowel with release of neurotoxin; eating honey
|
|
Clostridium difficile
|
pseudomembranous colitis; post-antibiotics; toxin assay stool; Rx metronidazole
|
|
Shigella sonnei
|
produces dysentery (bloody diarrhea); associated with HUS
|
|
Salmonella enteritidis
|
gastroenteritis; animal reservoirs - poultry, turtles
|
|
Salmonella paratyphi
|
sepsis; osteomyelitis in HbSS
|
|
Salmonella typhi
|
typhoid fever; human transmission; bradycardia, neutropenia, splenomegaly
|
|
Carrier state site
|
gallbladder
|
|
M. tuberculosis
|
MCC intestinal TB in United States (swallow TB); Peyer’s patch site of infection
|
|
Enterotoxigenic E. coli
|
secretory diarrhea (traveler’s diarrhea); toxin stimulates guanylate cyclase
|
|
Vibrio cholerae
|
secretory diarrhea; toxin stimulates adenylate cyclase to produce cAMP
|
|
Oral Rx cholera
|
solution must contain glucose to reabsorb Na+ (co-transport)
|
|
Yersinia enterocolitica
|
mesenteric lymphadenitis; sepsis in iron overload states
|
|
Entamoeba histolytica
|
dysentery; trophozoites phagocytose RBCs; liver abscess; Rx metronidazole
|
|
Cryptosporidium parvum
|
MCC diarrhea in AIDS; acid-fast oocysts
|
|
Giardia lamblia
|
MC protozoal cause of diarrhea; cause of malabsorption; Rx metronidazole
|
|
Trichuris trichiura
|
rectal prolapse in children
|
|
Enterobius vermicularis
|
anal pruritus; urethritis in girls; no eosinophilia
|
|
Ascaris lumbricoides
|
intestinal obstruction due to adult worms; no eosinophilia
|
|
Necator americanus
|
hookworm; iron deficiency anemia
|
|
Strongyloides stercoralis
|
rhabditiform larvae in stool not eggs
|
|
Diphyllobothrium latum
|
fish tapeworm; vitamin B12 deficiency
|
|
Signs of small bowel obstruction
|
colicky pain; constipation and obstipation
|
|
Radiograph small bowel obstruction
|
air-fluid levels on x-ray
|
|
MCC small bowel obstruction
|
adhesions from previous surgery
|
|
Duodenal atresia
|
vomiting bile-stained fluid at birth; double bubble sign; Down syndrome
|
|
Hirschsprung disease
|
absent ganglion cells in submucosal/myenteric plexus rectosigmoid
|
|
S/S
|
proximal bowel dilated but peristalses; no stool in rectal vault
|
|
Hirschsprung association
|
Down syndrome; Chagas disease
|
|
Intussusception
|
terminal ileum telescopes into cecum; obstruction plus bloody diarrhea
|
|
Meconium ileus
|
complication of cystic fibrosis
|
|
Indirect inguinal hernia
|
second MCC of small bowel obstruction; common in weight lifting
|
|
Gallstone ileus
|
obstruction of small bowel with gallstone + air in biliary tree
|
|
Volvulus
|
MC due to sigmoid colon twisting around mesentery
|
|
Direct inguinal hernia
|
protrudes through center of triangle of Hesselbach; no obstruction
|
|
Umbilical hernia
|
common in black children; may entrap bowel in adults
|
|
Sigmoid colon
|
MC site for polyps, cancer, diverticula
|
|
Small bowel infarction
|
diffuse abdominal pain with bloody diarrhea
|
|
Causes small bowel infarction
|
embolism (atrial fibrillation), thrombosis SMA or SMV
|
|
Ischemic colitis
|
splenic flexure pain with bloody diarrhea
|
|
Mesenteric angina
|
pain in splenic flexure 30 minutes after eating
|
|
Angiodysplasia
|
submucosal dilation of venules in cecum; cause of hematochezia
|
|
Hematochezia
|
massive loss of blood per rectum; diverticulosis MCC
|
|
Meckel’s diverticulum
|
persistence omphalomesenteric duct
|
|
S/S
|
bleeding MC (iron deficiency in children), diverticulitis
|
|
Meckel’s diverticulitis
|
mimics acute appendicitis; cannot differentiate without radionuclide scan
|
|
Sigmoid diverticulum
|
diverticulitis MC complication; MCC hematochezia and fistula formation
|
|
Diverticulitis
|
“left-sided acute appendicitis”
|
|
Ulcerative colitis
|
mucosal/submucosal ulceration; starts in rectum; crypt abscess; ↑ risk adenocarcinoma
|
|
S/S
|
left lower quadrant crampy pain with bloody diarrhea
|
|
UC associations
|
primary sclerosing cholangitis, seronegative HLA B27 + spondyloarthropathy
|
|
Crohn’s disease
|
transmural inflammation; terminal ileum involved 80%; granulomas; skip lesions
|
|
S/S
|
colicky pain and diarrhea; fistulas (anal, bowl to bowel)
|
|
Carcinoid tumor
|
appendix MC site; terminal ileum MC site for carcinoid syndrome
|
|
Carcinoid syndrome
|
liver metastasis; flushing/diarrhea due to serotonin; increased urine 5-HIAA
|
|
Tubular adenomas
|
precursor lesion colon cancer; size and number determine risk of malignancy
|
|
Villous adenoma
|
greatest risk for colon cancer (30%); secrete mucus rich in protein and potassium
|
|
Familial polyposis
|
AD with 100% penetrance for developing colon cancer
|
|
Gardner’s syndrome
|
AD, polyposis plus osteomas and desmoid tumors
|
|
Turcot’s syndrome
|
AD, polyposis plus brain tumors
|
|
Colorectal cancer
|
second MC cancer and cancer killer in adults
|
|
Left-sided colorectal cancer
|
obstruct; MC location rectosigmoid
|
|
Right-sided colorectal cancer
|
bleed
|
|
Acute appendicitis
|
due to lymphoid hyperplasia in children and obstruction by fecalith in adults
|
|
External hemorrhoids
|
thrombose
|
|
Internal hemorrhoids
|
bleed; prolapse out of rectum
|
|
Urobilinogen (UBG)
|
breakdown product CB in bowel (color of stool)
|
|
UBG
|
enterohepatic circulation to liver and kidney (color of urine)
|
|
Alcoholic liver disease
|
serum AST>ALT; ↑ serum GGT
|
|
Viral hepatitis
|
serum ALT>AST
|
|
Cholestasis markers
|
serum AP and GGT
|
|
Unconjugated bilirubin
|
macrophage degradation of heme; lipid soluble; never in urine
|
|
Conjugated bilirubin (CB)
|
water soluble; never normal in urine
|
|
% CB <20% (unconjugated)
|
Gilberts, spherocytosis, physiologic jaundice newborn, ABO/Rh HDN
|
|
Gilbert’s disease
|
AD; ↓ uptake and conjugation; bilirubin increases with fasting
|
|
Physiologic jaundice newborn
|
unconjugated hyperbilirubinemia; begins on day three
|
|
% CB 20-50%
|
viral/alcoholic hepatitis
|
|
% CB >50%
|
bile duct obstruction (intra or extrahepatic); carcinoma head of pancreas
|
|
Negative urine bilirubin + trace urobilinogen
|
normal urine
|
|
Positive urine bilirubin, absent urobilinogen
|
obstructive jaundice
|
|
Positive urine bilirubin + increased urobilinogen
|
hepatitis
|
|
Negative urine bilirubin + increased urobilinogen
|
extravascular hemolytic anemia
|
|
Markers of severity of liver disease
|
albumin, PT
|
|
Hepatitis A
|
protective antibodies; day care centers, jails, homosexuals, traveling; not chronic
|
|
Hepatitis B
|
protective antibodies; accidental needle stick, IVDA; hepatocellular carcinoma
|
|
Hepatitis C
|
no protective antibodies; post-transfusion hepatitis; chronic state; hepatocellular carcinoma
|
|
Hepatitis D
|
no protective antibodies; requires HBsAg to replicate
|
|
Anti-HBs alone
|
vaccination
|
|
Anti-HBs + anti-HBc-IgG
|
recovered from HBV
|
|
HBsAg + HBeAg + HBVDNA + anti-HBc-IgM
|
acute HBV/chronic HBV infective carrier if >6 months
|
|
Anti HBc-IgM alone
|
serologic gap; not infective
|
|
HBsAg + anti-HBc-IgM
|
chronic HBV healthy carrier
|
|
Fulminant hepatic failure
|
viral hepatitis and acetaminophen MCCs
|
|
Spontaneous peritonitis
|
E. coli in adults; S. pneumoniae in children; complication of ascites
|
|
Granulomatous hepatitis
|
TB MC bacteria
|
|
Amebiasis
|
Entamoeba histolytica; flash shaped ulcers in cecum; liver abscess; Rx
|
|
Echinococcosis
|
Echinococcus granulosis; sheep dog definitive host; man intermediate host
|
|
Schistosomiasis
|
Schistosoma mansoni; adult worms in portal vein; “pipe stem cirrhosis”
|
|
Clonorchiasis
|
Clonorchis sinensis; ingesting encysted larvae in fish; cholangiocarcinoma
|
|
Congestive hepatomegaly (centrilobular necrosis)
|
“nutmeg” liver; RHF MCC
|
|
Hepatic vein thrombosis
|
Budd-Chiari syndrome; painful hepatomegaly; ascites; portal hypertension
|
|
Portal vein thrombosis
|
ascites, portal hypertension, no hepatomegaly
|
|
Alcohol related disorders
|
fatty change; alcoholic hepatitis; cirrhosis
|
|
Hypertriglyceridemia in alcoholics
|
↑ synthesis of glycerol 3P (substrate for TG synthesis)
|
|
Hypoglycemia in alcoholics
|
↓ gluconeogenesis (↑ NADH causes pyruvate to convert to lactate)
|
|
Ketoacidosis in alcoholics
|
↑ lactate, ↑ ßOHB (acetyl CoA converted to AcAc and then ßOHB)
|
|
Primary biliary cirrhosis
|
granulomatous destruction triad bile ducts; anti-mitochondrial antibody
|
|
Primary sclerosing cholangitis
|
association with ulcerative colitis; MCC of cholangiocarcinoma
|
|
Extrahepatic biliary atresia
|
neonatal cholestasis
|
|
Drugs causing hepatitis
|
acetaminophen, isoniazid, halothane
|
|
Anabolic steroids
|
intrahepatic cholestasis
|
|
Estrogen/oral contraceptives
|
intrahepatic cholestasis; hepatic adenoma (intraperitoneal hemorrhage)
|
|
Methotrexate
|
liver fibrosis, fatty change
|
|
Liver angiosarcoma
|
vinyl chloride
|
|
Hemochromatosis
|
AR; increased iron reabsorption; liver target organ
|
|
S/S
|
cirrhosis; “bronze diabetes” - skin pigmentation + destruction of islet cells; malabsorption
|
|
Lab
|
↑ serum ferritin, iron, % saturation; ↓ TIBC
|
|
Wilson’s disease
|
AR disease; defect in copper excretion in bile and synthesis of ceruloplasmin
|
|
S/S
|
cirrhosis, movement disorder (necrosis in putamen), Kayser Fleisher ring (Descemet’s membrane)
|
|
Lab
|
↓ ceruloplasmin (causes ↓ total copper); ↑ serum/urine free copper
|
|
HELLP syndrome
|
pre-eclampsia; Hemolytic anemia, ELevated transaminases, Low Platelets
|
|
AAT deficiency in child
|
AR, cannot secrete AAT from liver cell; cirrhosis; hepatocellular carcinoma
|
|
Reye syndrome
|
coma and microvesicular fatty change post viral infection; increased ammonia
|
|
Cirrhosis
|
irreversible fibrosis; regenerative nodules; portal hypertension
|
|
Causes cirrhosis
|
alcohol (MC), HBV/HCV, hemochromatosis, Wilson’s, AAT deficiency, 1° biliary
|
|
Hepatic encephalopathy
|
mental status changes; ↑ serum ammonia
|
|
Portal hypertension
|
ascites; varices; splenomegaly; hemorrhoids; caput medusae
|
|
Cause of ascites
|
portal hypertension; hypoalbuminemia; secondary aldosteronism
|
|
Rx
|
use aldosterone blocker (acidosis increases loss ammonium in stool)
|
|
Hyperestrinism in men
|
gynecomastia; spider angiomas; female hair distribution
|
|
Lab findings cirrhosis
|
↓ BUN, glucose, sodium, potassium, calcium (↓ vitamin D); ↑ PT
|
|
Liver cell adenoma
|
estrogen related (steroids, oral contraceptives); intraperitoneal hemorrhage
|
|
Liver cancer
|
metastasis MC cancer; lung cancer MC primary site
|
|
Hepatocellular carcinoma
|
chronic HBV and HCV MCC; ↑ AFP; hepatic/portal vein invasion
|
|
Cholangiocarcinoma
|
primary sclerosing cholangitis MCC, C.C sinensis
|
|
Pathogenesis of cholesterol stones
|
bile with too much cholesterol and too little bile salts
|
|
Black pigment stones
|
sign of extravascular hemolytic anemia (spherocytosis, HbSS)
|
|
Acute cholecystitis
|
stone impacted in cystic duct; right upper quadrant colicky pain with radiation to shoulder
|
|
Chronic cholecystitis
|
chemical inflammation
|
|
Gallbladder cancer
|
risk factors - cholelithiasis and porcelain gallbladder
|
|
Acute pancreatitis
|
causes - alcohol and gallstones; ↑ amylase and lipase (more specific)
|
|
S/S
|
epigastric pain with radiation into back
|
|
Sentinel loop
|
localized ileus of duodenum due to acute pancreatitis
|
|
Pancreatic pseudocyst
|
abdominal mass; persistence of ↑ serum amylase >1 week
|
|
Chronic pancreatitis
|
alcohol abuse, CF; malabsorption, pain, type I diabetes
|
|
Pancreatic cancer
|
smoking MCC
|
|
S/S
|
jaundice/acholic (gray/pale) stools; palpable gallbladder; superficial migratory thrombophlebitis (Trousseau's sign); ↑ CA 19-9
|
|
First sign tubule cell dysfunction
|
inability to concentrate urine
|
|
Fixed specific gravity
|
chronic renal failure; cannot concentrate or dilute urine
|
|
Negative urine bilirubin + trace urobilinogen
|
normal urine
|
|
Positive urine bilirubin, absent urobilinogen
|
obstructive jaundice
|
|
Positive urine bilirubin + increased urobilinogen
|
hepatitis
|
|
Negative urine bilirubin + increased urobilinogen
|
extravascular hemolytic anemia
|
|
Positive urine nitrite + positive urine leukocyte esterase
|
urinary tract infection
|
|
Sterile pyuria
|
positive urine leukocyte esterase but negative standard culture; TB, C. trachomatis
|
|
Prerenal azotemia
|
↑ BUN and creatinine; ↓ renal blood flow (e.g. heart failure, hypovolemia)
|
|
Renal azotemia
|
↑ BUN and creatinine due to intrinsic renal disease (acute tubular necrosis)
|
|
Postrenal azotemia
|
↑ BUN and creatinine due to obstruction to urine flow
|
|
Serum BUN:creatinine ratio
|
<15:1 (renal failure); >15:1 (prerenal or postrenal azotemia)
|
|
BUN 80 mg/dL:creatinine 8 mg/dL
|
ratio 10/1 - renal failure
|
|
BUN 80 mg/dL:creatinine 2 mg/dL
|
ratio 40/1 - prerenal azotemia or postrenal azotemia
|
|
Creatinine clearance
|
measures GFR
|
|
Proteinuria
|
important sign of renal dysfunction
|
|
RBC casts
|
nephritic type of glomerulonephritis
|
|
WBC casts
|
acute pyelonephritis, acute tubulointerstitial nephritis
|
|
Fatty casts with Maltese crosses
|
nephrotic syndrome
|
|
Hyaline casts
|
normal unless associated with proteinuria
|
|
Renal tubular cell casts
|
acute tubular necrosis
|
|
Waxy or broad casts
|
chronic renal failure
|
|
Cystinuria
|
hexagonal crystals
|
|
Horseshoe kidney
|
Turner’s syndrome; lower poles fused
|
|
Renal dysplasia
|
MC childhood cystic disease; abnormal development; flank mass
|
|
Maternal oligohydramnios
|
fetal juvenile polycystic kidney disease; Potter’s facies in newborn
|
|
Adult polycystic kidney disease
|
AD; hypertension MC sign; cerebral berry aneurysms
|
|
Visceral epithelial cells
|
synthesize basement membrane
|
|
Glomerular BM
|
negative charge due to heparan sulfate
|
|
Nephritic syndrome
|
oliguria; RBC casts; hypertension; mild to moderate proteinuria
|
|
Nephrotic syndrome
|
proteinuria >3.5 g/day; ascites and pitting edema; fatty casts; fusion of podocytes
|
|
Immunofluorescence
|
linear (anti-glomerular BM antibodies); granular (IC deposition)
|
|
IgA GN
|
MC GN; usually nephritic; episodic hematuria; mesangial IC (lgA-anti-IgA) deposits
|
|
Post-streptococcal GN
|
nephritic; subepithelial deposits; skin/pharyngeal infections; anti-DNAase B
|
|
SLE type IV GN
|
nephritic; subendothelial deposits; anti-DNA antibodies
|
|
Crescentic GN
|
crescents from parietal cell proliferation; worst GN; Goodpasture’s, Wegener’s
|
|
Goodpasture’s
|
nephritic; anti-BM antibodies (glomerular + pulmonary capillary); crescentic GN
|
|
S/S
|
young male with hemoptysis progressing to renal failure
|
|
Minimal change disease (lipoid nephrosis)
|
MCC childhood nephrotic syndrome
|
|
Lipoid nephrosis
|
podocyte fusion; loss of negative charge in glomerular BM
|
|
Focal segmental glomerulosclerosis
|
nephrotic syndrome; AIDS and IV heroin abuse
|
|
Membranous GN
|
MCC adult nephrotic syndrome; subepithelial deposits; epimembranous spikes
|
|
Causes membranous GN
|
HBV, ACE inhibitors, cancer
|
|
Type I MPGN
|
nephrotic; subepithelial deposits; HCV association; tram tracks
|
|
Type II MPGN
|
nephrotic; C3 nephritic factor; intramembranous ICs (dense deposit disease)
|
|
DM nodular glomerulosclerosis
|
microalbuminuria first sign
|
|
DM glomerulosclerosis
|
nodules with collagen in mesangium; hyaline arteriolosclerosis of arterioles
|
|
ACE inhibitors
|
inhibit angiotensin II vasoconstriction of efferent arterioles
|
|
Alport’s syndrome
|
XD hereditary nephritis with sensorineural hearing loss
|
|
Ischemic ATN
|
prerenal azotemia MCC; renal tubular cell casts; BUN:creatinine ratio <15:1
|
|
Ischemic ATN
|
disruption of BM in proximal tubule and thick ascending limb
|
|
Nephrotoxic ATN
|
aminoglycosides, IVP dye, Pb/mercury poisoning
|
|
Nephrotoxic ATN
|
proximal tubule dysfunction; intact BM
|
|
Oliguria
|
prerenal azotemia, ATN, glomerulonephritis, postrenal azotemia
|
|
Acute pyelonephritis
|
vesicoureteral reflux with ascending infection; WBC casts, fever, flank pain
|
|
Chronic pyelonephritis
|
U-shaped scars overlying blunt calyces
|
|
Drug-induced tubulointerstitial nephritis
|
type I/IV reaction; e.g., penicillin
|
|
S/S
|
ARF, fever, rash, eosinophilia, eosinophiluria, WBC casts
|
|
Analgesic nephropathy
|
aspirin plus acetaminophen; renal papillary necrosis; IVP with ring defect
|
|
Myeloma kidney
|
BJ protein produces foreign body reaction in tubules
|
|
Urate nephropathy
|
prevent by giving allopurinol prior to chemotherapy
|
|
CRF
|
fixed specific gravity; BUN:creatinine <15:1; waxy and broad casts
|
|
Renal osteodystrophy CRF
|
hypovitaminosis D (no 1-α-hydroxylase); produces osteomalacia
|
|
Renal osteodystrophy CRF
|
osteoporosis from metabolic acidosis
|
|
Renal osteodystrophy CRF
|
secondary HPTH with increased osteoclastic activity
|
|
S/S CRF
|
pericarditis, prolonged bleeding time, normocytic anemia, pathologic fractures
|
|
Benign nephrosclerosis
|
kidney of hypertension; shrunken kidneys due to hyaline arteriolosclerosis
|
|
Malignant hypertension
|
renal failure; encephalopathy; BP >210/120 mm Hg; IV nitroprusside
|
|
Renal findings
|
necrotizing arteriolitis; “flea bitten” kidney; hyperplastic arteriolosclerosis
|
|
Renal infarction
|
pale infarcts; hematuria; common in polyarteritis nodosa
|
|
Hydronephrosis
|
renal stone MCC; atrophy of cortex/medulla; postrenal azotemia
|
|
Renal stones
|
most contain calcium (calcium oxalate/phosphate); hypercalciuria MC risk factor
|
|
S/S
|
colicky pain radiating into groin, hematuria; x-ray usually shows stone
|
|
Staghorn calculus
|
due to urease producing organisms (Proteus); alkaline urine pH; ammonia smell
|
|
Angiomyolipoma
|
hamartoma; associated with tuberous sclerosis
|
|
Renal cell carcinoma
|
smoking MCC; invasion renal vein/vena cava; lung, bone mets; yellow colored
|
|
S/S
|
flank mass, hematuria; ectopic hormones (EPO, PTH related peptide), left-sided varicocele
|
|
Renal pelvis transitional cell carcinoma
|
smoking MCC, phenacetin, aniline dyes, cyclophosphamide
|
|
Wilm’s tumor
|
hypertension, unilateral abdominal mass in child; aniridia/hemihypertrophy in AD types
|
|
Urine draining from umbilicus
|
persistent urachus
|
|
Retroperitoneal fibrosis
|
produces hydronephrosis
|
|
Bladder extrophy
|
abdominal wall defect + epispadias
|
|
Bladder diverticula
|
most commonly due to prostatic hyperplasia with urethral obstruction
|
|
Acute cystitis
|
E. coli; females > males; no fever, flank pain, or WBC casts
|
|
Bladder transitional cell carcinoma
|
smoking MCC, aniline dyes, cyclophosphamide; papillary
|
|
S/S
|
hematuria; hydronephrosis
|
|
Bladder adenocarcinoma
|
risk factors persistent urachus, extrophy
|
|
Bladder squamous cell carcinoma
|
Schistosoma hematobium infection
|
|
Hypospadias
|
ventral opening on penis due to failure closure of urethral folds
|
|
Epispadias
|
dorsal opening on penis due to defect in genital tubercle
|
|
Peyronie’s disease
|
painful curvature penis due to fibromatosis
|
|
Priapism
|
persistent/painful erection; HbSS
|
|
Squamous cell carcinoma penis
|
HPV and lack of circumcision most important risk factors
|
|
Cryptorchidism
|
undescended testis; risk for seminoma applies to cryptorchid testis and normal testis
|
|
Orchitis
|
mumps usually unilateral (infertility uncommon)
|
|
Epididymitis
|
<35 - N. gonorrhoeae, C. trachomatis; >35 - E. coli, P. aeruginosa
|
|
S/S
|
scrotal pain relieved by elevation of scrotum (Prehn’s sign)
|
|
Varicocele
|
left-sided scrotal mass; spermatic vein drains into left renal vein; infertility common
|
|
Varicocele
|
may be due to invasion of left renal vein by renal cell carcinoma
|
|
Hydrocele
|
persistent tunica vaginalis; scrotum transilluminates
|
|
Torsion of testicle
|
testicle high in canal; absent cremasteric reflex
|
|
Testicular cancer
|
unilateral painless mass that does not transilluminate
|
|
Risk factors
|
cryptorchid testis, Klinefelter’s, testicular feminization
|
|
Seminoma
|
MC cancer; radiosensitive; large cells with lymphoid infiltrate; small percentage have ↑hCG
|
|
Spermatocytic variant
|
>65 yrs of age
|
|
Embryonal carcinoma
|
hemorrhage/necrosis; hematogenous spread before lymphatic; ↑AFP, hCG
|
|
Yolk sac tumor
|
MC testicular cancer in boys; ↑AFP
|
|
Choriocarcinoma
|
most aggressive testicle cancer; ↑hCG
|
|
Teratoma
|
more often benign in children than adult
|
|
Teratocarcinoma
|
teratoma + embryonal carcinoma
|
|
Malignant lymphoma
|
MC type in elderly; metastasis not primary cancer
|
|
Prostate
|
DHT derived stimulation embryo; periurethral area - hyperplasia; peripheral area - cancer
|
|
Prostatitis
|
perineal pain, fever; WBCs at end of voiding
|
|
Benign prostatic hyperplasia
|
DHT/estrogen-mediated; glandular/smooth muscle hyperplasia
|
|
S/S
|
all men develop; urethral obstruction MC (hesitancy, dribbling, nocturia), hematuria, dysuria Rx
|
|
Prostate cancer
|
DHT-mediated; palpable with rectal exam; osteoblastic metastasis (↑ AP)
|
|
PSA
|
sensitive but not specific for prostate cancer; ↑ in hyperplasia
|
|
Kallmann’s syndrome
|
absent GnRH, anosmia, absence of taste
|
|
Impotence
|
failure to sustain an erection; psychogenic in most cases (erections present at night)
|
|
Erection
|
parasympathetic response
|
|
Ejaculation
|
sympathetic response
|
|
Leydig cell failure
|
↑ LH; ↓ testosterone, sperm count; normal FSH
|
|
Seminiferous tubule failure
|
↑ FSH (↓inhibin); ↓ sperm count; normal LH and testosterone
|
|
Leydig and seminiferous tubule failure
|
↑ FSH and LH; ↓ testosterone and sperm count
|
|
Y chromosome
|
determines genetic sex
|
|
Testosterone
|
develops seminal vesicles, epididymis, vas deferens
|
|
Dihydrotestosterone (DHT)
|
develops prostate and male external genitalia
|
|
Male pseudohermaphrodite
|
genetic male; phenotypically female
|
|
Testicular feminization
|
XR; deficient androgen receptors; MCC male pseudohermaphrodite
|
|
Klinefelter’s syndrome
|
XXY; 1 Barr body; female secondary sex characteristics
|
|
Herpes genitalis
|
recurrent painful vesicles; multinucleated squamous cells with intranuclear inclusions
|
|
Human papilloma virus
|
condyloma acuminata; koilocytosis (wrinkled nuclei surrounded by a halo)
|
|
Chlamydia trachomatis
|
metaplastic squamous cells with vacuoles containing elementary bodies
|
|
S/S
|
non-specific urethritis, cervicitis, PID, ophthalmia neonatorum
|
|
Neisseria gonorhoeae
|
urethritis, cervicitis, PID; ophthalmia neonatorum, gram negative diplococcus
|
|
Ophthalmia neonatorum first week
|
N gonorrhoeae
|
|
Ophthalmia neonatorum second week
|
C. trachomatis
|
|
Lymphogranuloma venereum
|
C. trachomatis subtype
|
|
S/S
|
scrotal/vulva lymphedema; granulomatous microabscesses; rectal strictures in females
|
|
Chancroid
|
painful ulcer, adenopathy, Hemophilus ducreyi
|
|
Granuloma inguinale
|
Calymmatobacterium granulomatis; raised ulceration but no lymphadenopathy
|
|
Treponema pallidum
|
spirochete; produces vasculitis of arterioles (plasma cell infiltrate)
|
|
Primary syphilis
|
painless chancre
|
|
Secondary syphilis
|
rash on palms/soles; condyloma lata; generalized adenopathy
|
|
Tertiary syphilis
|
neurosyphilis (e.g., tabes dorsalis), aortic arch aneurysm, gummas
|
|
RPR/VDRL
|
reagin antibodies against cardiolipin; ↓ titer with Rx of syphilis
|
|
RPR/VDRL
|
false positive with anticardiolipin antibodies (common in SLE)
|
|
FTA-ABS
|
confirmatory test for syphilis; not distinguish active from treated disease
|
|
FTA-ABS
|
remains positive after Rx
|
|
Trichomonas vaginalis
|
flagellate protozoan; cervicitis/vaginitis; Rx metronidazole both partners
|
|
Gardnerella vaginalis
|
vaginal pH >5; bacterial vaginosis; clue cells; Rx metronidazole
|
|
Candida vaginitis
|
white, curd-like discharge; DM, antibiotics, pregnancy; Rx fluconazole
|
|
Vulvar squamous cancer
|
MC vulvar cancer; HPV association
|
|
Vulvar leukoplakia
|
biopsy to R/O squamous dysplasia/cancer
|
|
Lichen sclerosis vulva
|
epidermal atrophy; slight risk for squamous cancer
|
|
Squamous hyperplasia vulva
|
leukoplakia; no cancer risk
|
|
Paget’s disease
|
intraepithelial adenocarcinoma (mucin production) of vulva
|
|
Malignant melanoma
|
vulva location; similar to Paget cells but not mucin positive
|
|
Gartner’s duct cyst
|
lateral wall vagina; persistent mesonephric duct
|
|
Embryonal rhabdomyosarcoma
|
bloody, grape-like vaginal mass young girl
|
|
Vaginal adenosis
|
maternal exposure to DES; precursor clear cell adenocarcinoma vagina
|
|
Vaginal squamous cancer
|
usually extension of cervical cancer
|
|
Rokitansky-Kiister-Hauser
|
absence of vagina and uterus
|
|
Nabothian cysts
|
endocervical glands covered by metaplastic squamous epithelium
|
|
Pathologic cervicitis
|
trichomonas, HSV-2, C. trachomatis (follicular cervicitis)
|
|
Cervical Pap
|
superficial squamous (estrogen), intermediate (progesterone), parabasal (no hormone)
|
|
Normal
|
70% superficial, 30% intermediate
|
|
Atrophic
|
100% parabasal cells
|
|
Hyperestrinism
|
100% superficial cells
|
|
Pregnancy
|
100% intermediate cells
|
|
Endocervical cells
|
sign of adequately performed Pap smear
|
|
Cervical polyp
|
bleeding after intercourse; non-neoplastic
|
|
Cervical dysplasia
|
begins in transformation zone; associated with low and high risk HPV
|
|
Risk factors cervical dysplasia/cancer
|
early onset sexual activity; multiple partners; smoking; OC
|
|
CIN
|
cervical intraepithelial dysplasia; mild, moderate, severe (in-situ)
|
|
Cervical cancer
|
↓ incidence (Pap smear); 45-yr-old; COD renal failure from obstruction of ureters
|
|
S/S
|
cervical discharge; bleeding after intercourse
|
|
Sequence to menarche
|
breast budding, growth spurt, pubic hair, axillary hair, menarche
|
|
Proliferative phase cycle
|
estrogen-dependent; ↑estrogen inhibits FSH and stimulates LH
|
|
Ovulation
|
day 14-I6; LH surge; subnuclear vacuoles; ↑body temperature
|
|
Secretory phase cycle
|
progesterone-dependent
|
|
Menses
|
drop in estrogen/progesterone stimulates apoptosis; plasmin prevents clotting
|
|
FSH
|
stimulates follicle and aromatase synthesis in granulosa cells
|
|
LH
|
stimulates androgen synthesis in proliferative phase and progesterone synthesis in secretory phase
|
|
Day 21
|
day of implantation of fertilized egg
|
|
Pregnancy
|
↑plasma volume > RBC mass; ↑GFR; ↑thyroxine/cortisol (increased binding proteins)
|
|
hCG
|
LH analogue produced by syncytiotrophoblast
|
|
hCG
|
stimulates corpus luteum of pregnancy to synthesize progesterone for 8-10 weeks
|
|
Estrone
|
estrogen of postmenopausal woman; aromatization of adrenal androstenedione
|
|
Estradiol
|
estrogen of non-pregnant woman in reproductive life; aromatization of testosterone
|
|
Estriol
|
estrogen of pregnancy
|
|
Menopause
|
↑ FSH (best screen; due to ↓estrogen), ↑LH
|
|
S/S
|
secondary amenorrhea, hot flushes
|
|
Hirsutism
|
↑ hair in normal areas
|
|
Virilization
|
hirsutism + male secondary sex characteristics (clitoromegaly)
|
|
Test for hirsutism/virilization
|
↑ testosterone - ovarian source; ↑DHEA-sulfate - adrenal source
|
|
Polycystic ovarian syndrome (POS)
|
↑ LH; ↓ FSH; ↑ estrogen and androgens
|
|
S/S
|
hirsutism, oligomenorrhea, infertility; enlarged ovaries with subcortical cysts; LH:FSH >2:1
|
|
Menorrhagia
|
excess menstrual flow; MCC iron deficiency in women
|
|
Dysmenorrhea
|
painful menses; 1° PGF2α, 2° endometriosis
|
|
DUB
|
bleeding related to hormone rather than anatomic causes
|
|
Anovulatory DUB
|
menarche and perimenopause; estrogen excess without progesterone
|
|
Ovulatory DUB
|
irregular shedding, inadequate luteal phase
|
|
Primary amenorrhea
|
no menses by 16 years old
|
|
Secondary amenorrhea
|
no menses for 3 months
|
|
Amenorrhea-hypothalamic/pituitary dysfunction
|
↓ FSH/LH; e.g., hypopituitarism
|
|
Amenorrhea-ovarian dysfunction
|
↑FSH/LH; e.g., Turner’s syndrome
|
|
Amenorrhea-end-organ disease
|
normal FSH/LH; e.g., imperforate hymen
|
|
Asherman syndrome
|
surgical removal of stratum basalis
|
|
Primary amenorrhea-normal secondary sex characteristics
|
constitutional delay MCC
|
|
Primary amenorrhea-lack secondary sex characteristics
|
Turner’s
|
|
Turner’s syndrome
|
XO; no Barr bodies; XO/XY types have gonadoblastomas; streak gonads (no eggs)
|
|
S/S
|
newborn with lymphedema hands/feet; cystic hygroma in neck (web); short stature; 1° amenorrhea
|
|
Secondary amenorrhea
|
pregnancy MCC; prolactinoma; anorexia nervosa; pituitary adenoma
|
|
Asherman syndrome
|
removal of stratum basalis causing scarring; secondary amenorrhea
|
|
Endometritis
|
group B streptococcus; intrauterine device (Actinomyces); chronic - plasma cells
|
|
Endometrial polyp
|
menorrhagia; not a precursor for endometrial cancer
|
|
Adenomyosis
|
functioning endometrial glands and stroma in myometrium; enlarged uterus
|
|
Endometriosis
|
functioning glands and stroma outside uterus; reverse menses; ovary MC site
|
|
S/S
|
dysmenorrhea, painful stooling, bowel obstruction; “powder burn” appearance
|
|
Endometrial hyperplasia
|
unopposed estrogen; simple/complex types; precursor endometrial cancer
|
|
Causes
|
obesity, estrogen Rx, polycystic ovarian syndrome
|
|
Endometrial cancer
|
obesity, nulliparity, estrogen Rx, early menarche/late menopause; OC protective
|
|
S/S
|
bleeding in postmenopausal woman
|
|
Leiomyoma uterus
|
menorrhagia, obstructive delivery; not a precursor for leiomyosarcoma
|
|
Leiomyosarcoma
|
MC sarcoma
|
|
Ectopic pregnancy
|
PID MC risk factor; intraperitoneal hemorrhage; screen with ß-hCG
|
|
Follicular cyst
|
MC ovarian mass in young woman
|
|
Risk factors ovarian tumors
|
nulliparity and genetic factors; OC protective
|
|
Serous ovarian tumors
|
surface-derived; ↑ bilaterality; psammoma bodies in malignant type
|
|
Mucinous ovarian tumors
|
surface-derived; pseudomyxoma peritonei in malignant type
|
|
Endometrioid carcinoma
|
resembles endometrial cancer; association with endometriosis
|
|
Cystic teratoma
|
MC benign germ cell tumor (<1% malignant); hair/teeth; calcifications
|
|
Dysgerminoma
|
MC malignant germ cell tumor; associated with streak gonads of Turners
|
|
Yolk sac tumor
|
MC germ cell tumor young girl; ↑AFP; Schiller-Duval bodies
|
|
Meigs syndrome
|
ovarian fibroma, ascites, right-sided pleural effusion
|
|
Granulosa tumor
|
low grade malignant; hyperestrinism, Call Exner bodies
|
|
Thecoma
|
benign; yellow color; hyperestrinism
|
|
Leydig cell and Sertoli cell tumors
|
hyperandrogenism
|
|
Gonadoblastoma
|
XY phenotype of Turner’s
|
|
Krukenberg tumors
|
metastatic stomach cancer; signet ring cells
|
|
Single umbilical artery
|
↑ incidence congenital defects
|
|
Syncytiotrophoblast
|
lining of villi; produces hCG and human placental lactogen
|
|
Human placental lactogen
|
responsible for mild glucose intolerance in pregnancy
|
|
Abruptio placenta
|
retroplacental clot; painful bleeding; hypertension, cocaine, smoking
|
|
Placenta previa
|
placenta implanted over cervical os; painless bleeding
|
|
Placenta accreta
|
direct implantation into myometrium without intervening decidua; hysterectomy
|
|
Twin placenta
|
monochorionic always identical twins; dichorionic may be identical or fraternal
|
|
Siamese twins
|
monoamniotic monochorionic twin placenta
|
|
Enlarged placenta
|
DM, Rh HDN, syphilis
|
|
Complete mole
|
benign neoplasm of chorionic villi; dilated villi; no embryo; 46 XX (both male)
|
|
S/S
|
preeclampsia in first trimester; ↑ incidence choriocarcinoma
|
|
Partial mole
|
embryo present; 68 XXY; no transformation into choriocarcinoma
|
|
Choriocarcinoma
|
malignancy of trophoblastic tissue (syncytiotrophoblast, cytotrophoblast)
|
|
Risk factors
|
complete mole (MC), spontaneous abortion, normal pregnancy
|
|
S/S
|
↑hCG; lung metastasis; good prognosis
|
|
Chorioamnionitis
|
group B streptococcus (S. agalactiae) infection
|
|
Preeclampsia
|
abnormal placentation causing placental ischemia; ↑ in vasoconstrictors (ATII)
|
|
S/S
|
hypertension, proteinuria, pitting edema; begins in third trimester
|
|
Spontaneous abortion
|
50% have karyotype deformity (trisomy 16)
|
|
Amniotic fluid
|
fetal urine
|
|
Polyhydramnios
|
TE fistula, duodenal atresia, open neural tube defects
|
|
Oligohydramnios
|
infantile polycystic disease
|
|
↑ Serum AFP
|
open neural tube defect
|
|
↓ Serum AFP
|
Down syndrome
|
|
Urine estriol
|
fetal adrenal, placental, maternal liver involved in its production
|
|
Down syndrome triad
|
↑ ß-hCG, ↓ serum AFP, ↓ urine estriol
|
|
Fibrocystic change
|
MC breast mass <50-yrs-old; atypical hyperplasia cancer risk; lumpy, painful breasts
|
|
Sclerosing adenosis
|
component of FCC; involves terminal lobules often has microcalcifications
|
|
Fibroadenoma
|
benign stromal tumor; MC movable mass in women <35-yrs-old
|
|
Intraductal papilloma
|
benign tumor lactiferous duct/sinus; MCC bloody nipple discharge <50-yr-old
|
|
Invasive ductal cancer
|
MCC breast mass in woman >50-yrs-old
|
|
Breast cancer risk
|
unopposed estrogen; family history first-degree relatives
|
|
Breast cancer
|
painless mass upper outer quadrant in woman >50-yrs-old
|
|
Mammography
|
screening test to detect non-palpable masses
|
|
Palpable breast mass
|
order fine needle aspiration (not a mammogram)
|
|
Ductal carcinoma in situ
|
necrotic centers (comedo); microcalcifications common
|
|
Paget’s disease of breast
|
invasive ductal cancer into nipple; Paget’s cells similar to vulvar Paget’s
|
|
Medullary carcinoma
|
bulky tumor with large cells and lymphoid infiltrate; more common in Pt with BRCA 1 mutation
|
|
Inflammatory carcinoma
|
orange peel appearance; lymphatics blocked by tumor (lymphedema)
|
|
Lobular cancer
|
MC cancer of terminal lobule; ↑ bilaterality
|
|
Phyllodes tumor
|
low grade malignant tumor of stroma
|
|
ER-PR positive tumors
|
tumors responding to hormones; candidate for tamoxifen (anti-estrogen)
|
|
ERB-B2 oncogene positive breast cancer
|
aggressive breast cancer
|
|
Gynecomastia
|
estrogen stimulation of male breast
|
|
Gynecomastia
|
normal in newborn, puberty (no surgery), old age; (micronodular) cirrhosis MC pathologic cause
|
|
Overactive endocrine syndrome
|
most often adenomas; use suppression tests (most do not suppress)
|
|
Tumors that suppress
|
prolactinoma (bromocriptine), pituitary Cushings (high dose dexamethasone)
|
|
Underactive endocrine syndrome
|
autoimmune destruction MCC; stimulation tests
|
|
Hypopituitarism adults
|
non-functioning adenoma MCC, Sheehan’s postpartum necrosis (stop lactation)
|
|
Hypopituitarism in children
|
craniopharyngioma (Rathke’s pouch remnant) MCC; visual field defects
|
|
S/S ↓ FSH and LH
|
amenorrhea, ↓ testosterone in male
|
|
Growth hormone functions
|
muscle growth, gluconeogenesis; release of insulin growth factor (IGF)
|
|
IGF
|
synthesized in liver; bone and cartilage growth
|
|
S/S ↓ GH/IGF in children
|
growth retardation; ↓ height and weight
|
|
Sleep and arginine infusion
|
stimulation tests for GH and IGF
|
|
S/S ↓ GH/IGF in adults
|
hypoglycemia
|
|
S/S ↓ TSH
|
secondary hypothyroidism; ↓ T4, ↓ TSH; muscle weakness, dry skin
|
|
S/S ↓ ACTH
|
secondary hypocortisolism; ↓ cortisol, ↓ ACTH; fatigue; hypoglycemia
|
|
Metyrapone
|
stimulation test for ACTH reserve
|
|
Metyrapone
|
blocks adrenal 11-hydroxylase → ↑ ACTH and 11-deoxycortisol (proximal to block)
|
|
Metyrapone test ↓ ACTH and 11-deoxycortisol
|
pituitary/hypothalamic dysfunction
|
|
Metyrapone test ↑ ACTH and 11-deoxycortisol
|
Addison's disease
|
|
Diabetes insipidus
|
loss ADH (central), refractory to ADH (nephrogenic); always diluting urine
|
|
Central diabetes insipidus (CDI)
|
↓ UOsm and ↑ POsm with water deprivation; vasopressin causes ↑ UOsm > 50%
|
|
Causes CDI
|
pituitary stalk transection, hypothalamic lesion (site for ADH synthesis)
|
|
Nephrogenic diabetes insipidus (NDI)
|
↓ UOsm and ↑ POsm with water deprivation; vasopressin causes ↑ UOsm < 50%
|
|
Causes NDI
|
lithium, demeclocycline, nephrocalcinosis, severe hypokalemia
|
|
Gigantism
|
GH secreting pituitary adenoma before epiphyses have fused
|
|
Acromegaly
|
GH secreting pituitary adenoma after epiphyses have fused
|
|
S/S acromegaly
|
cardiomyopathy; large hands, feet, jaw; hyperglycemia
|
|
Prolactin
|
inhibited by dopamine
|
|
Prolactinoma
|
MC pituitary tumor; secondary amenorrhea and galactorrhea; prolactin inhibits GnRH
|
|
Rx
|
surgery or bromocriptine (dopamine analog)
|
|
Other causes hyperprolactinemia
|
primary hypothyroidism, drugs
|
|
Inappropriate ADH syndrome
|
hyponatremia <120 mEq/L; ↑ UOsm (always concentrating urine)
|
|
Causes
|
small cell carcinoma lung, CNS injury, chlorpropamide
|
|
Rx
|
restrict water; demeclocycline in small cell carcinoma
|
|
Serum T4
|
↑ or ↓ in free hormone or thyroid binding globulin (TBG)
|
|
↑ Serum T4 and normal TSH
|
↑ TBG; due to ↑ in estrogen
|
|
↑ Serum T4 and ↓ TSH
|
thyrotoxicosis
|
|
↓ Serum T4 and normal TSH
|
↓ TBG; due to anabolic steroids
|
|
↓ Serum T4 and ↑ TSH
|
primary hypothyroidism
|
|
↓ Serum T4 and ↓ TSH
|
secondary hypothyroidism
|
|
TSH
|
negative feedback with T4 and T3; best screening test
|
|
I131 uptake
|
↑ in Graves; ↓ in thyroiditis, patient taking excess thyroid, hypothyroidism
|
|
Cold nodule
|
non-functioning nodule; no uptake I131
|
|
Hot nodule
|
functioning nodule; ↑ uptake I131
|
|
Thyroglossal duct cyst
|
midline cystic mass
|
|
Branchial cleft cyst
|
cyst in anterolateral neck
|
|
Acute/subacute thyroiditis
|
painful thyroid; early thyrotoxicosis; ↓ I131 uptake
|
|
Hashimoto’s thyroiditis
|
MCC hypothyroidism; HLA Dr3/Dr5; inhibitory IgG TSH receptor antibody
|
|
Hashimoto’s thyroiditis
|
↑ anti microsomal and thyroglobulin antibodies
|
|
S/S
|
muscle weakness, periorbital puffiness, ↓ reflexes, diastolic hypertension, constipation, dry skin
|
|
Lab
|
↓ T4, ↑ TSH
|
|
Cretinism
|
maternal hypothyroidism before fetal thyroid developed, genetic disorder
|
|
S/S
|
mental retardation; short stature and increased weight; coarse skin
|
|
Thyrotoxicosis
|
any cause ↑ thyroid hormone activity; Graves disease, excess hormone, thyroiditis
|
|
Hyperthyroidism
|
↑ synthesis thyroid hormone; Graves disease and toxic nodular goiter
|
|
Graves disease
|
autoantibody against TSH receptor (type II reaction); HLA Dr3
|
|
S/S unique to Graves
|
exophthalmos, pretibial myxedema
|
|
S/S thyrotoxicosis
|
tachycardia/atrial fibrillation, systolic hypertension, diarrhea, brisk reflexes
|
|
Lab thyrotoxicosis
|
↑ T4, ↓ TSH, ↑ glucose, ↑ calcium
|
|
I131 uptake
|
↑ Graves, toxic nodular goiter; ↓ thyroiditis, excess hormone, hypothyroidism
|
|
Rx Graves disease
|
ß-blocker; drug to decrease hormone synthesis (propylthiouracil)
|
|
Toxic nodular goiter
|
hyperthyroidism; develops out of a multinodular goiter; no exophthalmos
|
|
Goiter
|
enlarged thyroid; iodine deficiency MCC; relative thyroid hormone deficiency
|
|
S/S
|
rapid enlargement due to hemorrhage into cyst; Rx thyroxine
|
|
Solitary thyroid nodule woman
|
most often benign (cyst)
|
|
Solitary thyroid nodule man or child
|
often malignant
|
|
Papillary carcinoma thyroid
|
MC thyroid cancer; radiation exposure; psammoma bodies
|
|
Follicular carcinoma thyroid
|
invades blood vessels
|
|
Medullary carcinoma thyroid
|
parafollicular cells; calcitonin; amyloid (calcitonin conversion)
|
|
MEN I syndrome
|
3 P's; pituitary tumor, parathyroid adenoma, pancreatic tumor (ZE or ß-islet cell tumor)
|
|
MEN IIa syndrome
|
2 P's; medullary carcinoma thyroid, pheochromocytoma, parathyroid adenoma
|
|
MEN IIb syndrome
|
1 P; medullary carcinoma thyroid, pheochromocytoma, mucosal neuromas
|
|
Alkalotic pH
|
tetany with normal total calcium, ↓ ionized calcium and ↑ PTH
|
|
Hypoalbuminemia
|
↓ total calcium, normal ionized calcium and PTH
|
|
Tetany
|
↓ ionized calcium level; threshold potential comes closer to resting potential
|
|
S/S
|
thumb adducts into palm, twitching after tapping of facial nerve
|
|
PTH
|
maintains ionized Ca2+; ↑ Ca2+ renal reabsorption; ↓ phosphate/bicarbonate reabsorption in kidneys
|
|
Primary HPTH
|
↑ Ca2+, hypophosphatemia, ↑ PTH
|
|
Cause
|
adenoma MCC, hyperplasia, cancer
|
|
S/S
|
renal stone, peptic ulcers, pancreatitis, hypertension, metastatic calcification
|
|
Secondary HPTH
|
↓ Ca2+, ↑ PTH; hypovitaminosis D from renal failure MCC
|
|
Malignancy-induced hypercalcemia
|
↑ Ca2+, ↓ PTH; all other non-parathyroid causes same results
|
|
Causes hypercalcemia
|
osteolytic lesions, sarcoidosis, ↑ vitamin D, PTH-related peptide, myeloma
|
|
Tertiary HPTH
|
hypercalcemia developing from secondary HPTH
|
|
Primary hypoparathyroidism
|
↓ Ca2+ and ↓ PTH
|
|
Causes
|
previous thyroid surgery, autoimmune, DiGeorge syndrome
|
|
S/S
|
tetany; calcification basal ganglia
|
|
Pseudohypoparathyroidism
|
↓ Ca2+ with normal to ↑ PTH; end-organ resistance to PTH
|
|
Other causes ↓ Ca2+
|
hypomagnesemia (↓ PTH), ↓ vitamin D, DiGeorge
|
|
↓ Ca2+ and ↓ PTH
|
primary hypoparathyroidism
|
|
↓ Ca2+ and ↑ PTH
|
secondary hyperparathyroidism
|
|
↑ Ca2+ and ↑ PTH
|
primary hyperparathyroidism
|
|
↑ Ca2+ and ↓ PTH
|
malignancy induced hypercalcemia; other causes hypercalcemia
|
|
Waterhouse-Friderichsen syndrome
|
meningococcemia with bilateral adrenal hemorrhage due to DIC
|
|
Addison’s disease
|
autoimmune destruction adrenal cortex MCC, adrenogenital syndrome, metastasis
|
|
S/S
|
hypotension (salt loss), hyperpigmentation (ACTH), hypoglycemia
|
|
Lab
|
↓ sodium, ↓ cortisol, ↑ potassium, ↑ ACTH
|
|
Adrenogenital syndrome
|
AR; enzyme deficiency; hypocortisolism; hyperpigmentation from ↑ ACTH
|
|
21-Hydroxylase deficiency
|
↑ 17 KS, ↓ 17 OH, lose salt, hypotension; female pseudohermaphrodite
|
|
11-Hydroxylase deficiency
|
↑ 17 KS, ↑ 17 OH, retain salt, hypertension; female pseudohermaphrodite
|
|
17-Hydroxylase deficiency
|
↓ 17 KS, ↓ 17 OH, retain salt, hypertension; male pseudohermaphrodite
|
|
MCC Cushings
|
long-term corticosteroid therapy
|
|
Tests Cushings syndrome
|
low/high dose dexamethasone suppression; urine free cortisol (best test)
|
|
Normal dexamethasone suppression
|
cortisol analogue; ↓ ACTH and ↑ cortisol
|
|
Pituitary Cushings
|
MCC Cushing’s; ACTH secreting pituitary tumor
|
|
Lab
|
low dose dexamethasone not suppress cortisol; high dose suppresses
|
|
Adrenal Cushings
|
adrenal adenoma secreting cortisol; suppressed ACTH
|
|
Lab
|
no suppression with low/high dose dexamethasone
|
|
Ectopic Cushings
|
ACTH secreting small cell carcinoma of lung; high ACTH and cortisol levels
|
|
Lab
|
no suppression with low/high dose dexamethasone
|
|
S/S Cushings
|
purple stria, truncal obesity, hypertension, DM
|
|
Primary aldosteronism
|
benign adenoma in zona glomerulosa
|
|
S/S
|
hypertension and muscle weakness (hypokalemia), no pitting edema
|
|
Lab
|
hypernatremia, hypokalemia, metabolic alkalosis, ↑ urine K+ and Na+
|
|
Pheochromocytoma
|
benign tumor in adrenal medulla in adults
|
|
Associations
|
von Hippel Lindau, neurofibromatosis, MEN IIa and IIb
|
|
S/S
|
labile hypertension, anxiety, sweating, headache
|
|
Lab
|
↑ 24 hr urine for VMA and metanephrines
|
|
Neuroblastoma
|
malignant tumor adrenal medulla child; widespread metastasis; hypertension
|
|
ß-islet cell tumor (insulinoma)
|
benign tumor; hypoglycemia, ↑ insulin and C-peptide
|
|
Patient taking excess insulin
|
hypoglycemia, ↑ insulin, ↓ C-peptide
|
|
Glucagonoma
|
malignant α-islet cell tumor; hyperglycemia and rash
|
|
Zollinger Ellison syndrome
|
malignant islet cell tumor secreting gastrin; peptic ulcers
|
|
Somatostatinoma
|
malignant δ islet cell tumor; DM, malabsorption, cholelithiasis, achlorhydria
|
|
VIPoma
|
malignant islet cell tumor; diarrhea, hypokalemia, achlorhydria
|
|
DM
|
organ damage correlates with glycemic control
|
|
Type 1
|
young, thin person; no insulin; HLA DR3/4; insulitis; islet cell antibodies; ketoacidosis
|
|
Type 2
|
older person; obese; relative insulin deficiency (↓ insulin receptors, postreceptor problems)
|
|
Type 2
|
family history; fibrotic islet cells with amyloid; hyperosmolar nonketotic coma
|
|
↑ Non-enzymatic glycosylation
|
glucose attaches to amino acids in basement membranes
|
|
Non-enzymatic glycosylation
|
↑ vessel permeability producing hyaline arteriolosclerosis
|
|
Osmotic damage
|
glucose converted into sorbitol by aldose reductase
|
|
Osmotic damage
|
lens (cataracts), Schwann cell (neuropathy), pericytes retinal vessels (microaneurysms)
|
|
Pathogenesis hyperglycemia
|
↑ gluconeogenesis (most important), glycogenolysis
|
|
Pathogenesis hyperlipidemia
|
no insulin to stimulate capillary lipoprotein lipase; ↑ chylomicrons/VLDL
|
|
Pathogenesis ketoacidosis
|
↑ oxidation fatty acids with excess acetyl CoA; liver synthesis ketone bodies
|
|
Most commons due to DM
|
neuropathy, blindness, CRF, hyperglycemia, non-traumatic amputation
|
|
Glycosylated HbA1c
|
measure of long term glycemic control (8-12 weeks)
|
|
Gestational DM
|
↑ placental size, human placental lactogen
|
|
Complications
|
macrosomia (↑ muscle/fat from insulin), RDS, newborn hypoglycemia (↑ insulin)
|
|
Hypoglycemia
|
insulin/oral hypoglycemics MCC, liver disease; carnitine deficiency
|
|
Carnitine deficiency
|
no ß-oxidation of fatty acids; all cells compete for glucose
|
|
Monosodium urate crystals (MSU)
|
yellow when parallel to slow ray of compensator
|
|
Calcium pyrophosphate crystals (pseudogout)
|
blue when parallel to slow ray of compensator
|
|
Osteoarthritis
|
degeneration articular cartilage; subchondral cysts; eburnation; osteophytes at margins
|
|
Joints
|
weight bearing (femoral head); DIP joint (Heberden’s nodes), PIP joints (Bouchard nodes)
|
|
Neuropathic joint
|
2° to neurologic disease; DM, syringomyelia, tabes dorsalis
|
|
Erythropoietin
|
synthesized in peritubular capillaries
|
|
Reticulocyte count
|
measure of effective erythropoiesis; correct for degree of anemia
|
|
Extramedullary hematopoiesis
|
hematopoiesis outside bone marrow (e.g., spleen)
|
|
Newborn physiologic anemia
|
drop in Hb due to replacement of HbF RBCs with HbA
|
|
Pregnancy
|
Hb and Hct decreased; greater increase in plasma volume than RBC mass
|
|
Anemia
|
normal O2 saturation and arterial PO2
|
|
MCV
|
average volume of RBCs; useful for anemia classification
|
|
MCHC
|
average Hb concentration in RBCs
|
|
MCHC
|
↓ in microcytic anemias; ↑ in spherocytosis
|
|
Thalassemias
|
↓ MCV, ↑ RBC count
|
|
RDW
|
RBC size variation; ↑ iron deficiency; normal in other microcytic anemias
|
|
Mature RBC
|
anaerobic glycolysis; no mitochondria or HLA antigens
|
|
Total iron binding capacity
|
↑ iron deficiency; ↓ anemia chronic disease, sideroblastic anemia
|
|
% Saturation
|
↓ iron deficiency, anemia chronic disease; ↑ sideroblastic anemia
|
|
Serum ferritin
|
↓ iron deficiency; ↑ anemia chronic disease, sideroblastic anemia; normal thalassemia
|
|
Microcytic anemias
|
iron deficiency MC, anemic chronic disease, thalassemia, sideroblastic anemia
|
|
Iron deficiency child
|
MCC Meckel’s diverticulum
|
|
Iron deficiency woman < 50
|
MCC menorrhagia
|
|
Iron deficiency man < 50
|
MCC peptic ulcer disease
|
|
Iron deficiency men/woman > 50
|
MCC colon cancer
|
|
Stages iron deficiency
|
↓ ferritin; ↓ Fe and % saturation, ↑ TIBC; normocytic then microcytic anemia
|
|
Anemia chronic disease
|
MC anemia in malignancy and alcoholics
|
|
α-Thalassemia trait
|
AR; two α-globin gene deletions; normal Hb electrophoresis
|
|
HbH disease
|
three α-globin gene deletions; hemolytic anemia; four β-globin chains
|
|
Hb Bart’s disease
|
four α-globin gene deletions; four γ-globin chains
|
|
β-Thalassemia minor
|
AR; DNA splicing defect; ↑ HbA2 and F; ↓ HbA
|
|
β-Thalassemia major
|
nonsense mutation with stop codon; hemolytic anemia; ↑↑ HbF, ↑ HbA2
|
|
Sideroblastic anemia
|
defect in mitochondrial heme synthesis producing ringed sideroblasts
|
|
Causes sideroblastic anemia
|
alcohol, pyridoxine deficiency (isoniazid Rx of TB), Pb poisoning
|
|
Pb poisoning
|
inhibition ferrochelatase, d-aminolevulinic acid dehydrase, ribonuclease
|
|
S/S Pb poisoning children
|
growth retardation; Pb in epiphyses (lead lines); abdominal colic; encephalopathy
|
|
S/S Pb poisoning adult
|
peripheral neuropathy; proximal renal tubule damage (Fanconi’s syndrome)
|
|
Lab Pb poisoning
|
coarse basophilic stippling RBCs; ↓ MCV; ↑ blood Pb; ↑ d-aminolevulinic acid
|
|
Vitamin B12
|
animal products; requires intrinsic factor for reabsorption in terminal ileum
|
|
Vitamin B12
|
transfers methyl group to homocysteine
|
|
R factor
|
binds with B12 in mouth, removed by pancreatic enzymes in small intestine
|
|
Vitamin B12
|
involved in propionate metabolism; end-product succinyl CoA
|
|
Causes B12 deficiency
|
vegan, pernicious anemia MC, fish tapeworm, pancreatitis, bacterial overgrowth, Crohn’s disease
|
|
Pernicious anemia
|
autoimmune destruction parietal cells; chronic gastritis body/fundus; achlorhydria; ↑ gastrin
|
|
Causes folate deficiency
|
alcohol MCC, poor diet, drugs, malabsorption, pregnancy, goat milk
|
|
Drugs and folate deficiency
|
alcohol, OC, phenytoin, methotrexate, trimethoprim, 5-fluorouracil
|
|
Intestinal conjugase in folate metabolism
|
inhibited by phenytoin
|
|
Jejunal uptake of monoglutamate form of folate
|
inhibited by alcohol and OC
|
|
Dihydrofolate reductase
|
inhibited by methotrexate, trimethoprim
|
|
Thymidylate synthetase
|
inhibited by 5-fluorouracil
|
|
Folate deficiency
|
MCC of increased serum homocysteine
|
|
Lab in B12/folate deficiency
|
pancytopenia; hypersegmented neutrophils; ↑ homocysteine
|
|
Lab findings unique to B12 deficiency
|
↑ gastrin (pernicious anemia), ↑ methylmalonic acid
|
|
B12 reabsorbed absorbed after administration of intrinsic factor
|
PA
|
|
B12 reabsorbed absorbed after administration of antibiotics
|
bacterial overgrowth
|
|
B12 reabsorbed absorbed after administration of pancreatic extract
|
chronic pancreatitis
|
|
Acute blood loss
|
initially normal Hb and Hct; 0.9% saline uncovers RBC deficit
|
|
Aplastic anemia
|
drugs (e.g., phenylbutazone); infection (e.g., parvovirus); benzene
|
|
Lab findings aplastic anemia
|
pancytopenia; hypocellular bone marrow
|
|
Anemia in renal disease
|
normocytic; decreased EPO
|
|
Extravascular hemolysis
|
macrophage phagocytosis of RBCs; ↑ unconjugated bilirubin and urine UBG
|
|
Intravascular hemolysis
|
↓ serum haptoglobin; hemoglobinuria; hemosiderinuria
|
|
Congenital spherocytosis
|
AD; defect in spectrin; extravascular hemolysis; splenomegaly
|
|
Blood findings in spherocytosis
|
normocytic anemia; dense RBCs, ↑ MCHC, ↑ osmotic fragility
|
|
PNH
|
missing decay accelerating factor; complement destruction RBCs, neutrophils, platelets
|
|
S/S PNH
|
pancytopenia; hemoglobinuria; positive sugar water test and acidified serum test
|
|
HbSS
|
AR; missense mutation (valine for glutamic acid 6th positive β-globin chain)
|
|
Causes of sickling
|
↑ deoxyhemoglobin (hypoxemia, acidosis); HbS > 60%
|
|
HbF
|
inhibits sickling; hydroxyurea ↑ HbF
|
|
Pathophysiology HbSS
|
vasoocclusive crises, hemolytic anemia (extravascular)
|
|
HbSS children
|
dactylitis (6-9 months); Streptococcus pneumoniae sepsis (dysfunctional spleen)
|
|
HbSS osteomyelitis
|
Salmonella paratyphi
|
|
HbSS complications
|
aplastic crisis (parvovirus B-19), acute chest syndrome, autosplenectomy, calcium bilirubinate gallstones, priapism, aseptic necrosis
|
|
HbAS
|
microhematuria from sickling in renal medulla; renal papillary necrosis
|
|
Hb electrophoresis
|
HbAS-HbA 55-60%, HbS 40-45%; HbSS-HbS 90-95%, HbF 5-10%
|
|
Blood findings in HbSS
|
sickle cells; target cells; Howell-Jolly bodies (nuclear remnants)
|
|
G6PD deficiency
|
XR; oxidant damage (peroxide) to Hb (e.g., primaquine; dapsone; fava beans)
|
|
Blood findings G6PD deficiency
|
Heinz bodies (denatured Hb; special stain); bite cells
|
|
Pyruvate kinase deficiency
|
↓ ATP; RBCs dehydrated; ↑ 2,3-BPG (right-shifted OBC)
|
|
Warm type AIHA
|
IgG; extravascular hemolysis; e.g., SLE, drugs
|
|
Cold type AIHA
|
IgM intravascular hemolysis; e.g., CLL, Mycoplasma
|
|
Penicillin
|
IgG antibody against penicillin attached to RBC (type II hypersensitivity)
|
|
Methyldopa
|
drug alters Rh antigens; IgG antibody against Rh antigens (type II hypersensitivity)
|
|
Quinidine
|
drug-IgM IC; intravascular hemolysis; type III hypersensitivity
|
|
Lab findings AIHA
|
positive direct Coombs’; spherocytes
|
|
Micro-macroangiopathic hemolysis
|
mechanical damage causing intravascular hemolysis
|
|
Causes of micro/macro hemolysis
|
aortic stenosis (MCC), DIC, TIP, HUS
|
|
Peripheral blood findings micro/macro hemolysis
|
schistocytes; iron deficiency from hemoglobinuria
|
|
Malaria
|
intravascular hemolysis correlates with fever; falciparum-ring forms and gametocytes
|
|
Leukemoid reaction
|
exaggerated WBC response to infection; usually due to infection
|
|
Leukoerythroblastic reaction
|
marrow infiltrative disease peripheralizes myeloblasts/nucleated RBCs
|
|
Causes of leukoerythroblastic reaction
|
bone metastasis MCC, myelofibrosis
|
|
Eosinophilia
|
type I hypersensitivity (e.g., penicillin reaction); invasive helminthic infection
|
|
Helminthes not producing eosinophilia
|
pinworms, adult worms in ascariasis
|
|
Atypical lymphocytes
|
mononucleosis; CMV; toxoplasmosis; viral hepatitis; phenytoin
|
|
Mononucleosis
|
due to EBV; EBV attaches to CD21 receptors on B cells
|
|
Clinical findings mono
|
exudative tonsillitis, generalized lymphadenopathy, hepatosplenomegaly
|
|
Lab findings mono
|
atypical lymphocytosis; IgM heterophile antibodies against horse RBCs
|
|
Lymphopenia
|
T cell deficiencies (HIV); combined B/T deficiency (adenine deaminase deficiency)
|
|
Lymphocytosis
|
viral infections, whooping cough
|
|
Corticosteroids
|
lymphopenia, eosinopenia, neutrophilia
|
|
Chronic MPD
|
neoplastic stem cell disorder; splenomegaly; marrow fibrosis; risk for leukemia
|
|
Examples of MPD
|
polycythemia vera, myelofibrosis and myeloid metaplasia
|
|
Relative polycythemia
|
↓ plasma volume; ↑ RBC count; normal RBC mass
|
|
Absolute polycythemia
|
↓ RBC count and RBC mass
|
|
Appropriate polycythemia
|
hypoxic stimulus for EPO to generate RBCs
|
|
Causes of appropriate absolute polycythemia
|
lung disease, cyanotic heart disease, high altitude
|
|
Appropriate absolute polycythemia
|
normal plasma volume; ↑ RBC mass; ↓ SaO2; ↑ EPO
|
|
Inappropriate absolute polycythemia
|
no hypoxic stimulus for EPO
|
|
Causes of inappropriate polycythemia
|
ectopic secretion EPO, polycythemia vera
|
|
Polycythemia vera
|
↑ plasma volume and RBC mass; normal SaO2; ↓ EPO
|
|
Ectopic EPO (renal cell carcinoma)
|
normal plasma volume; ↑ RBC mass; normal SaO2; ↑ EPO
|
|
Myelofibrosis myeloid metaplasia
|
marrow fibrosis; extramedullary hematopoiesis; splenomegaly
|
|
Lab findings in myelofibrosis
|
tear drop RBCs; dry bone marrow aspirate (marrow fibrosis)
|
|
Essential thrombocythemia
|
MPO with increase in abnormal appearing platelets
|
|
Myelodysplastic syndrome
|
severe anemia in elderly; 30% develop leukemia; ringed sideroblasts
|
|
Benzene
|
aplastic anemia; acute leukemia
|
|
Leukemia by age
|
ALL, newborn-14; AML, 15-60; CML, 40-60; CLL, >60
|
|
Acute vs. chronic leukemia
|
acute, blasts >30% in bone marrow; chronic, blasts <10% in bone marrow
|
|
AML
|
Auer rods in myeloblasts
|
|
Acute promyelocytic leukemia
|
t(15;17); defect in retinoic acid; Rx retinoic acid (↑ maturation); DIC
|
|
Acute monocytic leukemia
|
gum infiltration
|
|
CML
|
t(9;22) of ABL POC; Philadelphia chromosome 22; ↓ alkaline phosphatase score
|
|
ALL
|
early pre-B (80%); CALLA (CD10) and TdT positive; CNS and testicle involvement
|
|
ALL
|
t(12;21) offers good prognosis
|
|
CLL
|
B cell neoplasm; ↓ γ-globulins; MCC generalized lymphadenopathy patients> 60-yrs-old
|
|
Adult T cell leukemia
|
HTLV-1; CD4 T cells; skin infiltration; lytic bone lesions with hypercalcemia
|
|
Hairy cell leukemia
|
positive TRAP stain; splenomegaly; Rx with purine nucleosides
|
|
Nodal sites
|
germinal follicles, B cells; paracortex, T cells; sinuses, histiocytes
|
|
Testicular cancer
|
metastasizes to para-aortic nodes
|
|
Stomach cancer
|
metastasizes to left supraclavicular nodes (Virchow node)
|
|
Phenytoin
|
atypical lymphocytosis
|
|
Cat scratch disease
|
Bartonella henselae; granulomatous microabscesses
|
|
Follicular B-cell lymphoma
|
t(14;18); overexpression of BCL-2 anti-apoptosis gene
|
|
Burkitt lymphoma
|
t(8;14); EBV association; common childhood NHL; “starry sky” appearance
|
|
Extra nodal lymphomas
|
risk factors H. pylori (stomach); Sjogren’s syndrome
|
|
Mycosis fungoides
|
CD4 T cell neoplasm; skin lesions with Pautrier’s microabscesses
|
|
Sezary syndrome
|
leukemic phase of mycosis fungoides
|
|
Polyclonal gammopathy
|
sign of chronic inflammation
|
|
Monoclonal gammopathy
|
M component (spike); sign of plasma cell disorder
|
|
Confirmatory tests
|
serum and urine immunoelectrophoresis; bone marrow aspirate
|
|
Bence Jones protein
|
light chains in urine; predictive of a malignant plasma cell disorder
|
|
Multiple myeloma
|
M spike; lytic bone lesions; pathologic fractures; hypercalcemia; renal failure
|
|
MGUS
|
MC monoclonal gammopathy; may progress to myeloma
|
|
Findings in MGUS
|
elderly patient; no BJ protein; no malignant plasma cells
|
|
Waldenstrom’s macroglobulinemia
|
lymphoplasmacytic lymphoma; IgM M spike; hyperviscosity
|
|
Hodgkin’s lymphoma
|
neoplastic component, Reed Stemberg (RS) cell; CD15 CD30 positive
|
|
Lymphocyte predominant Hodgkin’s
|
infrequent classic RS cells
|
|
Nodular sclerosing Hodgkin’s
|
female dominant; supraclavicular nodes + anterior mediastinal nodes
|
|
Mixed cellularity Hodgkin’s
|
male dominant; numerous RS cells; EBV association
|
|
Hodgkin’s prognosis
|
stage of disease and type of Hodgkin’s most important factors
|
|
Alkylating agents in Rx of Hodgkins
|
↑ risk for second malignancies (leukemia; NHL)
|
|
Langerhan’s histiocytes
|
CD1 positive; Birbeck granules
|
|
Letterer-Siwe disease
|
malignant histiocytosis <2 yrs old; diffuse eczematous rash; organ involvement
|
|
Hand-Christian-Christian disease
|
malignant; lytic skull lesions, diabetes insipidus, exophthalmos
|
|
Eosinophilic granuloma
|
benign histiocytosis; lytic bone lesions with pathologic fractures
|
|
Mast cells
|
release histamine (pruritus; swelling); metachromatic granules positive with toluidine blue
|
|
Urticaria pigmentosum
|
localized mastocytosis; skin lesions swell and itch with scratching
|
|
Amyloid
|
twisted β-sheet; apple green birefringence with Congo red
|
|
Primary amyloidosis
|
AL amyloid derived from light chains; plasma cell disorders
|
|
Secondary amyloidosis
|
AA amyloid derived from serum-associated amyloid; chronic infections
|
|
Alzheimer’s disease
|
amyloid precursor protein gene product chromosome 21; amyloid-β
|
|
Gaucher’s disease
|
macrophages have fibrillary appearance; deficiency glucocerebrosidase
|
|
Niemann Pick’s disease
|
macrophages have soap bubble appearance; deficiency sphingomyelinase
|
|
Hypersplenism
|
splenomegaly; peripheral blood cytopenias; portal hypertension MCC
|
|
Splenic dysfunction
|
Howell Jolly bodies; susceptible to Streptococcus pneumoniae sepsis
|
|
Anticoagulants
|
tissue plasminogen activator, heparin, PGI2 ATIII, protein C/S
|
|
Heparin
|
enhances ATIII activity (neutralizes all factors except V, VIII, fibrinogen)
|
|
Protein C/S
|
neutralize V and VIII
|
|
Procoagulants
|
coagulation factors, thromboxane A2 (platelet aggregation, vasoconstrictor)
|
|
Protein C and S
|
inactivate factors V and VIII; enhance fibrinolysis
|
|
von Willebrand factor
|
complexes with factor VIII to enhance VIII:C activity; platelet adhesion
|
|
Platelets
|
receptors for von Willebrand factor and fibrinogen; synthesize thromboxane A2
|
|
GpIb
|
platelet receptor for von Willebrand factor
|
|
GpIIb:IIIa
|
platelet receptor for fibrinogen
|
|
Extrinsic system factor
|
VII
|
|
Intrinsic system factors
|
XII, XI, IX, VIII
|
|
Final common pathway factors
|
X, V, prothrombin (II), fibrinogen (I)
|
|
Factor XIII
|
cross-links insoluble fibrin; strengthens fibrin clots
|
|
Vitamin K-dependent factors
|
prothrombin, VII, IX, X, protein C and S
|
|
Factors consumed in a clot
|
fibrinogen, prothrombin, V, VIII; fluid is called serum
|
|
Plasmin
|
cleaves fibrinogen and insoluble fibrin into degradation products
|
|
Bleeding time
|
evaluates platelet function (adhesion, release reaction, aggregation)
|
|
Aspirin
|
MCC of a prolonged bleeding time
|
|
Tests for vWF
|
ristocetin cofactor assay; vWF antigen assay; agar electrophoresis
|
|
PT
|
evaluates extrinsic pathway to fibrin clot
|
|
PTT
|
evaluates intrinsic pathway to stable fibrin clot
|
|
Fibrinolysis tests
|
fibrin(ogen) degradation products; D-dimers (cross-linked insoluble fibrin)
|
|
S/S platelet dysfunction
|
cannot form temporary plug; epistaxis; petechiae; bleeding from scratches
|
|
Idiopathic thrombocytopenic purpura (ITP)
|
children; antibodies against GpIIb:IIIa; no splenomegaly
|
|
Chronic autoimmune thrombocytopenic purpura
|
SLE; antibodies against GpIIb:IIIa receptors
|
|
Heparin
|
thrombocytopenia due to IgG antibody against heparin attached to PF4 on platelets
|
|
PF4
|
heparin neutralizing factor
|
|
HIV
|
thrombocytopenia MC hematologic abnormality; similar to ITP
|
|
TTP
|
platelet thrombi develop in areas of endothelial damage in small vessels; consumption of platelets
|
|
S/S
|
fever, thrombocytopenia, renal failure, hemolytic anemia with schistocytes, CNS deficits
|
|
Lab findings TTP
|
thrombocytopenia, prolonged bleeding time, normal PT and PTT
|
|
HUS
|
similar to TTP; endothelial injury from Shiga-like toxin of 0157:H7 E. coli in undercooked beef
|
|
S/S factor deficiency
|
no stable fibrin clot-late rebleeding; menorrhagia; GI bleeding; hemarthroses
|
|
Hemophilia A
|
XR; hemarthroses; prolonged PTT, ↓ factor VIII activity, normal VIII antigen
|
|
von Willebrand’s disease
|
AD; platelet adhesion defect + factor VIII deficiency
|
|
Lab findings in VWD
|
↓ vWF, VIII antigen, and VIII:C; prolonged bleeding time
|
|
Desmopressin acetate
|
Rx of choice for mild von Willebrand’s disease and hemophilia A
|
|
Circulating anticoagulants
|
antibodies destroy coagulation factors
|
|
Lab finding in circulating anticoagulant
|
prolonged PT and/or PTT corrected with mixing studies
|
|
Vitamin K deficiency
|
↓ epoxide reductase activity (↓ function vitamin K); hemorrhagic diathesis; ↑ PT
|
|
Causes vitamin K deficiency
|
antibiotics MC, newborn, malabsorption, warfarin
|
|
DIC
|
activation coagulation system from release of tissue thromboplastin and/or endothelial cell damage
|
|
DIC
|
consumption coagulation factors by fibrin clots; patient also anticoagulated
|
|
Causes
|
septic shock MCC, rattlesnake bite, massive trauma, amniotic fluid
|
|
S/S
|
bleeding from all scratches, holes, needle sites
|
|
Lab findings DIC
|
thrombocytopenia, ↑ PT and PTT, D-dimers (best test), anemia
|
|
Antiphospholipid antibodies
|
lupus anticoagulant and anticardiolipin antibodies; vessel thrombosis
|
|
Warfarin
|
inhibits epoxide reductase; PT best test but PTT also prolonged
|
|
Warfarin
|
full anticoagulation in 3 days when -carboxylated prothrombin disappears
|
|
Warfarin
|
ingredient in rat poison; danger to children in households with grandparents on warfarin
|
|
Rx warfarin over anticoagulation
|
intramuscular vitamin K (6-8 hrs), fresh frozen plasma (immediate)
|
|
Heparin
|
enhances ATIII; PTT best test but PT also prolonged
|
|
OC
|
estrogen ↑ coagulation factor synthesis and ATIII; predisposes to thrombosis
|
|
Factor V Leiden
|
MC hereditary thrombosis; resistant to degradation by protein C/S
|
|
ATIII deficiency
|
no prolongation of PTT with administration of heparin
|
|
Hemorrhagic skin necrosis
|
post-warfarin therapy in patient with heterozygote protein C deficiency
|
|
M cells
|
specialized cells that transfer foreign antigens to lymphocytes in Peyer’s patches
|
|
Blood group O
|
some patients have anti-AB-IgG antibodies; increased incidence duodenal ulcers
|
|
Blood group A
|
increased incidence of gastric carcinoma
|
|
Newborns
|
do not have natural blood group antibodies at birth (e.g., anti-A-lgM)
|
|
Elderly
|
may lose natural blood group antibodies; no hemolytic reaction to mismatched blood
|
|
Rh antigens
|
inherited in autosomal codominant fashion; Rh antigens include D, C, c, E, e
|
|
Atypical antibodies
|
antibodies against Rh or non-Rh blood group antigens (e.g., anti-D)
|
|
Duffy antigen
|
receptor for Plasmodium vivax; blacks often lack Duffy antigen
|
|
Antibody screen
|
indirect Coomb’s test; detects atypical antibodies in serum
|
|
Cytomegalovirus
|
MC infection transmitted by blood transfusion; MC antibody
|
|
Hepatitis C
|
MCC of post-transfusion hepatitis
|
|
Major crossmatch
|
patient serum reacted against donor RBCs; does not guarantee RBC survival
|
|
Universal donor
|
blood group O; no antigens on the surface of RBCs
|
|
Universal recipient
|
blood group AB; no natural blood group antibodies in serum
|
|
Packed RBC transfusion
|
raises Hb by 1 gm/dL and Hct by 3%
|
|
Cryoprecipitate
|
fibrinogen and factor VIII
|
|
Fresh frozen plasma
|
replacement for multiple factor deficiencies (e.g., cirrhosis, DIC)
|
|
Allergic transfusion reaction
|
type I IgE-mediated hypersensitivity reaction
|
|
Febrile transfusion reaction
|
recipient anti-HLA antibodies react against donor leukocytes
|
|
Intravascular HTR
|
transfusion of ABO incompatible blood (e.g., A person receives B blood)
|
|
Extravascular HTR
|
antibody attaches to donor RBCs; macrophage phagocytosis and hemolysis
|
|
Positive direct Coomb’s test
|
present in both types of hemolytic transfusion reactions
|
|
S/S
|
jaundice, no increase in Hb, hemoglobinuria
|
|
ABO HDN
|
mother O and baby A or B; transplacental passage of maternal anti-AB-IgG
|
|
ABO HDN
|
positive direct Coomb’s test; spherocytes; MCC unconjugated hyperbilirubinemia first 24 hrs
|
|
Rh HDN
|
mother Rh (D antigen) negative and fetus Rh (O antigen) positive
|
|
Rh HDN
|
no hemolysis in first Rh incompatible pregnancy
|
|
Rh HDN
|
maternal anti-D crosses placenta; potential for hydrops fetalis; high risk for kernicterus
|
|
Rh immune globulin
|
anti-D; coats D antigen site on fetal RBCs in maternal circulation
|
|
Rh HDN lab
|
positive direct Coomb’s; severe anemia and hyperbilirubinemia
|
|
ABO HDN
|
protects mother from Rh sensitization (development of anti-D antibodies)
|
|
O Rh negative mother with A Rh positive baby
|
A+ cells destroyed by mothers anti A-lgM
|
|
Blue fluorescent light
|
converts unconjugated bilirubin in skin into harmless water soluble dipyrrole
|
|
MV auscultation
|
apex
|
|
TV auscultation
|
left parasternal border
|
|
AV auscultation
|
right 2nd intercostal space
|
|
PV auscultation
|
left 2nd intercostal space
|
|
S1
|
closure MV and TV
|
|
S2
|
closure AV and PV
|
|
Inspiration
|
split in A2 and P2; due to increased blood in right side of heart
|
|
S3
|
abnormal; due to blood entering volume overloaded ventricle in early diastole
|
|
Causes S3
|
valve regurgitation; congestive heart failure
|
|
S4
|
abnormal; due to blood entering non-compliant ventricle with atrial contraction in late diastole
|
|
Causes S4
|
volume overloaded ventricle, hypertrophy
|
|
Murmurs
|
stretching valve ring or damage to valve
|
|
Inspiration
|
increases right sided abnormal heart sounds and murmurs
|
|
Expiration
|
increases left sided abnormal heart sounds and murmurs
|
|
Stenosis murmurs
|
problem in opening valve
|
|
Regurgitation murmurs
|
problem in closing valve
|
|
Valves opening in systole
|
AV and PV
|
|
Valves opening in diastole
|
MV and TV
|
|
Valves closing in systole
|
MV and TV
|
|
Valves closing in diastole
|
AV and PV
|
|
LDL
|
primary vehicle for carrying cholesterol
|
|
VLDL
|
primary vehicle for carrying liver-synthesized triglyceride
|
|
Familial hypercholesterolemia (type II)
|
AD; deficiency of LDL receptors; ↑ LDL
|
|
Type III hyperlipoproteinemia
|
deficiency apo E; ↑ remnants (chylomicron, intermediate density)
|
|
Type IV hyperlipoproteinemia
|
↑ VLDL; alcoholics
|
|
Apo B deficiency
|
deficiency apo B48 (chylomicrons) and B100 (VLDL); ↓ CH and TG
|
|
Clinical findings in apo B deficiency
|
malabsorption; hemolytic anemia
|
|
Atherosclerosis
|
reaction to injury of endothelial cells
|
|
Risk factors
|
smoking, ↑ LDL, ↑ homocysteine, Chlamydia pneumoniae infection
|
|
Cells involved
|
platelets, macrophages, smooth muscle cells, T cells with cytokine release
|
|
Fibrous plaque
|
pathognomonic lesion of atherosclerosis
|
|
C-reactive protein
|
marker of an inflammatory atheromatous plaque
|
|
Inflammatory atheromatous plaque
|
predisposes to platelet thrombosis
|
|
Increased plasma homocysteine
|
↑ vessel thrombosis; folate (MC)/vitamin B12 deficiency
|
|
Hyaline arteriolosclerosis
|
small vessel disease of DM and hypertension; excess protein in vessel wall
|
|
Mechanisms hyaline arteriolosclerosis in DM
|
non-enzymatic glycosylation
|
|
Non-enzymatic glycosylation
|
glucose attaches to amino acids in BM; causes ↑ permeability to protein
|
|
Mechanisms hyaline arteriolosclerosis in hypertension
|
pressure pushes proteins into vessel wall
|
|
Abdominal aortic aneurysm rupture
|
due to atherosclerosis; flank pain, hypotension, pulsatile mass
|
|
Syphilitic aneurysm
|
vasculitis of vasa vasorum of aortic arch; aortic regurgitation
|
|
Aortic dissection
|
due to hypertension and collagen tissue disorders (e.g., Marfan)
|
|
Cystic medial degeneration
|
elastic tissue degeneration creates spaces filled with mucopolysaccharides
|
|
Intimal tear in aorta
|
due to wall stress from hypertension and structural weakness
|
|
Types of dissection
|
proximal (MC); distal or combination of both
|
|
S/S proximal aortic dissection
|
chest pain radiating to back, lack of pulse; cardiac tamponade MC COD
|
|
Marfan’s
|
AD; fibrillin defect; aortic regurgitation/dissection; lens dislocation; MVP with sudden death
|
|
MC COD Marfan’s and Ehlers Danlos
|
aortic dissection
|
|
Phlebothrombosis
|
stasis of blood flow; deep veins below knee MC site
|
|
Pulmonary thromboembolism
|
emboli originate from femoral veins
|
|
Superficial migratory thrombophlebitis
|
sign of carcinoma of head of pancreas
|
|
Thoracic outlet syndrome
|
absent radial pulse with positional change
|
|
Turner’s syndrome
|
lymphedema hands/feet in newborn; preductal coarctation
|
|
Spider telangiectasia
|
arteriovenous fistula; due to hyperestrinism (cirrhosis, pregnancy)
|
|
Capillary hemangioma in newborn
|
regress with age; do not surgically remove
|
|
Kaposi’s sarcoma
|
HHV-8; vascular malignancy; MC cancer in AIDS
|
|
Bacillary angiomatosis
|
Bartonella henselae; vascular infection in AIDS
|
|
Small vessel vasculitis
|
palpable purpura; e.g., Henoch Schonlein purpura
|
|
Muscular artery vasculitis
|
vessel thrombosis with infarction; e.g., classical polyarteritis nodosa
|
|
Elastic artery vasculitis
|
absent pulse, stroke
|
|
Takayasu’s arteritis
|
pulseless disease; young Asian woman
|
|
Giant cell arteritis
|
temporal artery granulomatous vasculitis; ipsilateral blindness (ophthalmic artery)
|
|
Classical polyarteritis nodosa
|
muscular artery vasculitis with vessel thrombosis infarction
|
|
Path findings
|
vessel inflammation at different stages; aneurysms from vessel weakness
|
|
S/S
|
infarctions in kidneys, skin, GI tract, heart; HBsAg in 30%
|
|
Diagnosis
|
angiography identifies aneurysms and thrombosis
|
|
Kawasaki’s disease
|
coronary artery vasculitis/thrombosis/aneurysms in children
|
|
S/S
|
chest pain; desquamating rash; swelling hands/feet; cervical lymphadenopathy
|
|
Rx
|
IV γ-globulin
|
|
Buerger’s disease (thromboangiitis obliterans)
|
smoker’s digital vasculitis; digital infarction
|
|
Raynaud’s syndrome
|
digital vasculitis in PSS and CREST syndrome
|
|
S/S
|
digital pain; white-blue-red color changes
|
|
Cryoglobulinemia
|
protein gels in cold temperature; Raynaud’s syndrome; HCV association
|
|
S/S
|
acral cyanosis relieved by coming indoors
|
|
Wegener’s granulomatosis
|
association with c-ANCA; sinusitis, lung infarction, crescentic GN
|
|
Microscopic polyangiitis
|
palpable purpura; crescentic GN; association with p-ANCA
|
|
Henoch-Schönlein purpura
|
IgA-anti-IgA ICs; palpable purpura buttocks/legs; arthritis; IgA GN
|
|
Serum sickness vasculitis
|
e.g., horse antivenin in Rx of rattlesnake envenomation
|
|
Rocky Mountain spotted fever
|
tick borne
|
|
Meningococcemia
|
sepsis causes petechia/ecchymoses; potential for Waterhouse Friderichsen syndrome
|
|
Essential HTN blacks
|
defect in renal excretion of sodium; ↑ plasma volume, ↓ PRA
|
|
Renovascular HTN
|
atherosclerosis renal artery in men; fibromuscular hyperplasia renal artery women
|
|
S/S
|
epigastric bruit; ↑ PRA affected kidney, ↓ PRA unaffected kidney
|
|
Endocrine HTN
|
1º HPTH, Graves/hypothyroidism, Cushing’s, 1º aldosteronism, phaeochromocytoma
|
|
Hypertension
|
LVH MC complication; AMI MC COD followed by stroke and renal failure
|
|
Afterload
|
resistance ventricles contract against
|
|
Preload
|
volume ventricles must eject
|
|
Concentric LVH
|
increased afterload; e.g., essential HTN, aortic stenosis
|
|
LVH with dilation/hypertrophy
|
increased preload; e.g., valve regurgitation; left to right shunts
|
|
LHF
|
forward failure; pulmonary edema, pillow orthopnea, paroxysmal nocturnal dyspnea
|
|
Systolic dysfunction
|
LHF due to decreased ventricular contractility (ischemia)
|
|
Diastolic dysfunction
|
LHF due to decreased ventricular compliance (hypertrophy)
|
|
RHF
|
backward failure; ↑ venous hydrostatic pressure; neck vein distention, hepatomegaly, edema
|
|
ACE inhibitors
|
decrease afterload and preload in heart failure
|
|
Diuretics in CHF
|
reduce preload
|
|
Non-pharmacologic Rx in CHF
|
restrict salt and water
|
|
AMI
|
MC COD in United States; left anterior descending coronary artery thrombosis MCC
|
|
Exertional angina
|
coronary artery atherosclerosis; subendocardial ischemia; ST depression
|
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Prinzmetal’s angina
|
coronary artery vasospasm; transmural ischemia; ST elevation
|
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Sudden cardiac death
|
death within 1 hr of symptoms
|
|
Path findings
|
severe coronary artery atherosclerosis; absence of occlusive thrombosis
|
|
LAD coronary artery
|
anterior portion left ventricle, anterior 2/3rds IVS
|
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RCA
|
posterior portion left ventricle and papillary muscle, inferior 1/3rd IVS, right ventricle
|
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AMI
|
rupture of inflammatory plaque produces platelet thrombus
|
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Ventricular fibrillation
|
MC COD in AMI
|
|
AMI
|
no gross changes until 24 hrs
|
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S/S AMI
|
retrosternal pain radiating down arms, diaphoresis
|
|
AMI ruptures
|
3rd-7th day
|
|
Anterior wall rupture
|
MC type; LAD thrombosis; cardiac tamponade
|
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Posteromedial papillary muscle rupture
|
RCA thrombosis; mitral regurgitation with LHF
|
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IVS rupture
|
LAD thrombosis; left to right shunt; RHF
|
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Mural thrombus
|
anterior AMI; danger embolization
|
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Pericarditis
|
first week in transmural AMI; 6 wks later autoimmune
|
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S/S
|
friction rub; leaning forward relieves pain
|
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Ventricular aneurysms
|
late manifestation of AMI; precordial systolic bulge; CHF MC COD
|
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Right ventricular infarction
|
RCA thrombosis; hypotension, RHF, preserved left ventricular function
|
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Diagnosis of AMI
|
CK-MB and troponins; CK-MB absent by 3 days; troponins last 7-10 days
|
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LDH isoenzymes
|
no longer used; LDH 1/2 flip indicates AMI
|
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Reinfarction
|
reappearance CK-MB after 3 days
|
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ECG findings in AMI
|
inverted T waves; ST elevation; Q waves
|
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Ejection fraction
|
EF = stroke volume/left ventricular end-diastolic volume; 80/120 = 0.66
|
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By-pass surgery
|
use internal mammary artery and saphenous veins (“arterialize” after 10 yrs)
|
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Angioplasty complication
|
localized dissection with thrombosis
|
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Umbilical vein
|
highest O2 saturation
|
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Ductus arteriosis in fetus
|
shunts blood from pulmonary artery to aorta; PGE keeps it open
|
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Ductus arteriosus in newborn
|
closes and becomes ligamentum arteriosum
|
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Eisenmenger’s syndrome
|
cyanosis due to reversal of left to right shunt
|
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VSD
|
MC congenital heart disease; ↑ SaO2 right ventricle (RV), pulmonary artery (PA)
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ASD
|
patent foramen ovale; ↑ SaO2 right atrium (RA), RV, PA; MC adult congenital heart disease
|
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Down syndrome
|
endocardial cushion defect (combined ASD and VSD)
|
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PDA
|
machinery murmur; close with indomethacin; ↑ SaO2 PA
|
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Tetralogy of Fallot
|
degree of pulmonic stenosis determines if cyanosis is present
|
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Tetralogy of Fallot
|
↓ left ventricle, aorta
|
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Tetralogy of Fallot
|
ASD and PDA are cardioprotective
|
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Complete transposition
|
cyanosis; aorta empties RV; PA empties left ventricle
|
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Complications cyanotic heart disease
|
2° polycythemia; infective endocarditis; metastatic abscesses
|
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Pre-ductal coarctation
|
Turner’s syndrome
|
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Post-ductal coarctation
|
constriction distal to ligamentum arteriosum
|
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S/S
|
upper extremity HTN; claudication; rib-notching; activation RAA also causes HTN
|
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Acute rheumatic fever
|
type II hypersensitivity; group A streptococcus pharyngeal infection
|
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Acute rheumatic fever
|
sterile vegetations mitral valve (regurgitation); myocarditis with Aschoff nodule
|
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S/S
|
polyarthritis (MC), carditis, erythema marginatum, rheumatoid nodules, chorea
|
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Mitral stenosis
|
chronic rheumatic fever; opening snap followed by mid-diastolic rumble
|
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Mitral stenosis
|
left atrial dilation hypertrophy - atrial fibrillation, thrombus, pulmonary edema, RHF
|
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MVP
|
myxomatous degeneration of mitral valve; common in Marfan syndrome, Ehlers Danlos
|
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S/S
|
mid-systolic click followed by a murmur; palpitations, chest pain, rupture of chordae
|
|
MVP click/murmur close to S1
|
decrease preload (stand, Valsalva, anxiety)
|
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MVP click/murmur close to S2
|
increase preload (supine, squat, clench fist)
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Mitral regurgitation
|
pansystolic murmur; S3 and S4 common
|
|
Causes
|
LHF, infective endocarditis, acute rheumatic fever
|
|
Aortic stenosis
|
systolic ejection murmur; syncope and angina with exercise; hemolytic anemia
|
|
Aortic stenosis murmur increased preload
|
worsens obstruction and increases murmur intensity
|
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Aortic stenosis murmur decreased preload
|
decreases obstruction and decreases murmur intensity
|
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Causes
|
bicuspid aortic valve; age-related sclerosis
|
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Aortic regurgitation
|
bounding pulses; early diastolic blowing murmur
|
|
Austin Flint murmur
|
diastolic murmur; regurgitant flow on anterior leaflet mitral valve
|
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Significance Austin Flint murmur
|
sign for AV replacement
|
|
Causes aortic regurgitation
|
essential HTN, infective endocarditis, acute rheumatic fever, dissection
|
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Tricuspid regurgitation
|
pansystolic murmur ↑ intensity with inspiration
|
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Causes
|
endocarditis IV drug abuse; RHF; carcinoid heart disease
|
|
Carcinoid heart disease
|
tricuspid regurgitation, pulmonic stenosis
|
|
Infective endocarditis (IE)
|
Streptococcus viridans MCC; Staphylococcus aureus MCC IVDA
|
|
IE prosthetic heart valve
|
Staphylococcus epidermidis (coagulase negative)
|
|
IE ulcerative bowel disease
|
Streptococcus bovis
|
|
S/S
|
IC vasculitis-Roth spot, splinter hemorrhages; regurgitant murmurs; metas1atic abscesses
|
|
Lab findings
|
positive blood culture Libman Sacks endocarditis
|
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Coxsackievirus
|
MCC of myocarditis (lymphocyte infiltrate in myocardium) and pericarditis
|
|
Parasitic cause myocarditis
|
leishmania in Chagas disease
|
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Pericardial effusion
|
all chamber pressures are uniformally increased
|
|
S/S
|
muffled heart sounds, pulsus paradoxus, inspiratory neck vein distention
|
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Dx and Rx
|
echocardiogram, pericardiocentesis, respectively
|
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Pulsus paradoxus
|
drop in blood pressure >10 mmHg with inspiration
|
|
Constrictive pericarditis
|
TB MCC worldwide; pericardial knock
|
|
Congestive cardiomyopathy
|
generalized chamber enlargement; low ejection fraction
|
|
Causes
|
postpartum, cardiotoxic drugs, hypothyroidism, alcohol
|
|
Hypertrophic cardiomyopathy
|
MCC of sudden death in young person (due to conduction defects)
|
|
Site of obstruction
|
anterior leaflet mitral valve drawn against asymmetric thickened IVS
|
|
Effect decreased preload on systolic murmur
|
worsens obstruction and increases murmur intensity
|
|
Effect increased preload on systolic murmur
|
reduces obstruction and decreases murmur intensity
|
|
Restrictive cardiomyopathy
|
decreased compliance
|
|
Causes
|
iron, amyloid, glycogen; sarcoidosis; tropical endocardial fibrosis
|
|
Cardiac myxoma
|
benign tumor left atrium; embolization; syncope
|
|
Cardiac rhabdomyoma
|
childhood tumor; association with tuberous sclerosis
|
|
U wave
|
hypokalemia; MCC diuretic therapy (e.g., thiazides; loop diuretics)
|
|
Peaked T wave
|
hyperkalemia; MCC renal failure
|
|
ST depression
|
subendocardial ischemia (e.g., classical angina pectoris)
|
|
ST elevation
|
transmural ischemia (e.g., AMI), pericarditis, ventricular aneurysm
|
|
Atrial fibrillation
|
MC chronic arrhythmia; absent P waves; danger for embolization
|
|
Ventricular premature beats
|
wide QRS complexes; MC arrhythmia in coronary care unit
|
|
Ventricular fibrillation
|
MCC of death in an AMI
|
|
Anterior AMI
|
Q waves in I and V1-V4
|
|
Inferior AMI
|
Q waves in II, III, and aVF; right coronary artery thrombosis.
|
|
Wolff-Parkinson-White
|
short PR interval with normal P wave; delta wave on upstroke of R wave
|
|
Alveolar O2 calculation
|
% O2 breathing (713) - PCO2/0.8
|
|
Increased A-a gradient
|
primary lung disease; left to right shunts in heart
|
|
Forced vital capacity
|
total amount of air expelled after a maximal inspiration
|
|
Forced expiratory volume/1 second (FEV1)
|
amount of air expelled in I second after maximal inspiration
|
|
Choanal atresia
|
cyanotic when breast feeding; turns pink when crying
|
|
Nasal polyps
|
allergic (MC; adults only), aspirin, cystic fibrosis
|
|
Nasal polyp in a child
|
requires sweat test to exclude cystic fibrosis
|
|
Triad asthma
|
patient on aspirin (pain syndrome) with nasal polyps, asthma
|
|
Obstructive sleep apnea (OSA)
|
snoring with intervals of apnea (respiratory acidosis with hypoxemia)
|
|
S/S
|
danger cor pulmonale; requires sleep test; Rx. O2 with continuous positive airway pressure
|
|
Sinusitis
|
maxillary sinusitis MC in adults; ethmoiditis MC in children; S. pneumoniae MC
|
|
Nasopharyngeal carcinoma
|
association with EBV; metastasize to cervical nodes
|
|
Laryngeal carcinoma
|
smoking MCC; hoarseness; squamous cell carcinoma
|
|
Resorption atelectasis
|
MCC of fever 24-36 hours after surgery
|
|
S/S
|
↓ percussion; absent fremitus, breath sounds; inspiratory lag; elevated diaphragm
|
|
RDS
|
decreased production surfactant; airway collapse; hyaline membranes
|
|
Type II pneumocytes
|
synthesize surfactant (lecithin, phosphatidylcholine); stored in lamellar bodies
|
|
Surfactant
|
reduces surface tension in airways; ↑ synthesis cortisol, thyroxine; ↓ synthesis insulin
|
|
Causes RDS
|
prematurity, maternal diabetes, C-section
|
|
Maternal diabetes
|
maternal hyperglycemia → fetal hyperglycemia → fetal insulin which ↓ surfactant
|
|
Complications RDS
|
O2 FR injury (blindness, bronchopulmonary dysplasia); necrotizing enterocolitis
|
|
Typical community acquired pneumonia
|
Streptococcus pneumoniae MCC
|
|
Typical pneumonia
|
bronchopneumonia, lobar pneumonia
|
|
S/S
|
productive cough; consolidation - ↓ percussion, ↑ tactile fremitus
|
|
Atypical community acquired pneumonia
|
interstitial pneumonia; Mycoplasma pneumoniae MCC
|
|
S/S
|
low grade fever, non-productive cough, no signs consolidation
|
|
Nosocomial pneumonia
|
Pseudomonas, aeruginosa MCC (respirators); others - S. aureus, E. coli
|
|
Rhinovirus
|
MCC common cold; hand to mouth transmission
|
|
Respiratory syncytial virus
|
MCC pneumonia and bronchiolitis in child
|
|
Parainfluenza virus
|
MCC croup in child; trachea area of obstruction
|
|
Cytomegalovirus
|
basophilic intranuclear inclusion surrounded by halo
|
|
Influenza
|
superimposed pneumonia with S. aureus increases mortality
|
|
Rubeola
|
Warthin-Finkeldey multinucleated giant cells
|
|
Chlamydia pneumoniae
|
atypical pneumonia; association with coronary artery disease
|
|
Chlamydia trachomatis
|
pneumonia in newborns; staccato cough; wheezing
|
|
Coxiella burnetii
|
only rickettsia without a vector
|
|
Mycoplasma pneumoniae pneumonia
|
crowded condition; cold agglutinins; azithromycin
|
|
Streptococcus pneumoniae pneumonia
|
gram positive diplococcus; azithromycin
|
|
Staphylococcus aureus pneumonia
|
tension pneumatocysts in children with cystic fibrosis
|
|
Corynebacterium diphtheriae
|
toxin produces ADP ribosylation of elongation factor 2
|
|
Haemophilus influenzae
|
exacerbation chronic bronchitis; acute epiglottis in children
|
|
Inspiratory stridor child
|
croup, epiglottitis
|
|
Pseudomonas aeruginosa
|
MCC of pneumonia and death in cystic fibrosis; green sputum
|
|
Klebsiella pneumoniae
|
mucoid sputum in alcoholic
|
|
Legionella pneumophila
|
silver stain; water coolers/mist (grocery produce, restaurants, zoo rain forest)
|
|
Mycobacterium tuberculosis
|
strict aerobe; MC COD due to infectious disease worldwide
|
|
Candida albicans
|
vessel invader; yeasts and pseudohyphae
|
|
Cryptococcus immitis
|
pigeon excreta; narrow-based bud
|
|
Aspergillus fumigatus
|
septate hyphae with fruiting body; fungus ball, extrinsic asthma, vessel invader
|
|
Mucor species
|
non-septate; vessel invader; frontal lobe abscess in diabetic ketoacidosis
|
|
Coccidioides immitis
|
Southwest deserts; inhale arthrospores in dust; spherule with endospores
|
|
S/S
|
erythema nodosum (painful nodules lower legs)
|
|
Histoplasma capsulatum
|
Ohio/central Mississippi river valley; excreta bats (spelunker), chickens
|
|
H. capsulatum
|
simulates TB; yeasts phagocytosed by macrophages
|
|
Blastomyces dermatitidis
|
overlaps histoplasmosis; broad-based buds; skin lesion simulates cancer
|
|
Pneumocystis carinii
|
cysts and trophozoites; pneumonia in HIV; Rx. trimethoprim/sulfamethoxazole
|
|
Primary TB
|
upper portion lower lobe, lower portion upper lobe
|
|
Primary TB
|
Ghon focus (subpleural caseation); Ghon complex (spread to hilar nodes)
|
|
Reactivation TB
|
cavitating lesion in upper lobe; kidney MC extrapulmonary site
|
|
Mycobacterium avium intracellulare (MAI)
|
atypical TB; MC TB in AIDS
|
|
CF
|
AR; 3 nucleotide deletion chromosome 7; defective CFTR (degraded in Golgi apparatus)
|
|
S/S
|
pneumonia, malabsorption, males sterile; + sweat test; P. aeruginosa pneumonia MC COD
|
|
Lung abscess
|
MCC aspiration oropharyngeal material (mixed aerobe/anaerobe); x-ray - air/fluid level
|
|
Aspiration sitting
|
posterobasal segment right lower lobe
|
|
Aspiration supine
|
superior segment right lower lobe
|
|
Aspiration right side
|
right middle lobe, posterior segment right upper lobe
|
|
Pulmonary thromboembolism
|
most derive from femoral vein
|
|
Bronchial artery
|
branch of aorta/intercostal artery; protects against developing pulmonary infarction
|
|
Saddle embolus
|
sudden death due to acute right heart strain
|
|
S/S pulmonary infarction
|
dyspnea and tachypnea; pleuritic chest pain; pleural effusion
|
|
Dx
|
ventilation/perfusion scan; respiratory alkalosis; hypoxemia
|
|
Pain on inspiration
|
pleuritic inflammation; pulmonary embolus, pneumonia, pneumothorax
|
|
Pathogenesis pulmonary hypertension (PH)
|
hypoxemia and respiratory acidosis
|
|
Hypoxemia + respiratory acidosis
|
vasoconstriction pulmonary vessels; vasodilation cerebral vessels
|
|
Causes PH
|
1° lung disease (COPD, restrictive), recurrent emboli, mitral stenosis, OSA, left-right shunts
|
|
Cor pulmonale
|
PH + RVH
|
|
S/S
|
dyspnea; accentuated P2 (PH); parasternal heave (RVH)
|
|
Restrictive lung disease
|
↓ compliance, ↑ elasticity; interstitial fibrosis/edema
|
|
Restrictive lung disease (RLD)
|
↓ all volumes and capacities; ↑ FEV1sec/FVC ratio
|
|
ARDS
|
RLD; non-cardiogenic pulmonary edema due to alveolar injury
|
|
ARDS
|
neutrophil destruction of type I and II pneumocytes; hyaline membranes
|
|
Causes
|
septic shock (MC), aspiration gastric contents, severe trauma
|
|
Pneumoconiosis
|
inhalation mineral dust causing interstitial fibrosis; particles <0.5 μm to reach alveoli
|
|
Caplan syndrome
|
pneumoconiosis + rheumatoid nodules in lungs
|
|
Coal worker’s
|
“black lung” disease; progressive massive fibrosis; no increased incidence cancer or TB
|
|
Silicosis
|
quartz; nodular opacities; foundry workers; ↑ incidence cancer and TB
|
|
Sources asbestos
|
roofing material, old buildings (9/11), pipe-fitter shipyard
|
|
Ferruginous bodies
|
asbestos fiber coated by iron
|
|
Asbestosis
|
benign pleural plaques (MC); bronchogenic carcinoma (MC cancer); mesothelioma
|
|
Mesothelioma
|
malignancy of serosa; no smoking association
|
|
Sarcoidosis
|
RLD; MC non-infectious lung and liver granulomatous disease
|
|
S/S
|
dyspnea, hilar adenopathy (non-caseating granulomas), uveitis, nodular skin lesions
|
|
Lab findings
|
↑ ACE, hypercalcemia (macrophages synthesize 1-α-hydroxylase)
|
|
Kveim test
|
intradermal injection sarcoid antigens causes skin reaction
|
|
Farmer’s lung
|
RLD; lung reaction against thermophilic bacteria in moldy hay
|
|
Silo filler’s disease
|
RLD; reaction against nitrogen dioxide in fermenting corn
|
|
Byssinosis
|
RLD; reaction against cotton, linen, hemp products in textile industry
|
|
Goodpasture’s syndrome
|
RLD; anti-BM antibodies; begins in lungs and ends in renal failure
|
|
Collagen vascular RLD
|
SLE, rheumatoid arthritis, systemic sclerosis
|
|
Drugs RLD
|
amiodarone, bleomycin, busulfan, cyclophosphamide, methotrexate, nitrofurantoin
|
|
Obstructive lung disease
|
↑ compliance, ↓ elasticity
|
|
Obstructive lung disease
|
↑ RV, TLC; ↓ TV, VC; ↓ FEV1sec and FVC; ↓ FEV1sec/FVC ratio
|
|
Obstructive lung disease
|
asthma, emphysema, chronic bronchitis, bronchiectasis
|
|
Asthma
|
extrinsic (type I hypersensitivity) and intrinsic types
|
|
S/S
|
expiratory wheezing (inflamed terminal bronchioles); LTC4,-D4,-E4 bronchoconstrictors
|
|
Charcot-Leyden crystals
|
derive from crystalline material in eosinophil granules
|
|
Lab findings
|
initial respiratory alkalosis; respiratory acidosis requires intubation
|
|
Emphysema
|
destruction elastic tissue respiratory unit; lung hyperinflation; smoking MCC; pink puffer
|
|
Respiratory unit
|
respiratory bronchiole, alveolar duct, alveoli
|
|
Radiograph emphysema
|
↑ AP diameter; depressed diaphragms; vertical heart
|
|
Pathogenesis
|
↓ AAT, ↑ neutrophil destruction of elastic tissue
|
|
Centriacinar emphysema
|
destruction/distention respiratory bronchioles upper lobe in smokers; THINK
|
|
Panacinar emphysema
|
destruction/distention entire respiratory unit lower lobes; AAT deficiency
|
|
Paraseptal emphysema
|
upper lobe destruction/distention alveolar ducts, alveoli; pneumothorax
|
|
Lab findings
|
normal to decreased PCO2 (respiratory alkalosis)
|
|
Chronic bronchitis
|
productive cough 3 months/2 consecutive years; blue bloater (cyanosis)
|
|
Site of obstruction
|
terminal bronchioles (proximal to respiratory unit)
|
|
Lab findings
|
respiratory acidosis/hypoxemia
|
|
Bronchiectasis
|
obstruction/infection key causes; dilated bronchioles extend to periphery
|
|
Causes
|
CF MCC, TB, immotile cilia syndrome
|
|
Immotile cilia syndrome
|
absent dynein arm in cilia; sinusitis, infertility, bronchiectasis, situs inversus
|
|
Central lung cancers
|
squamous cancer and small cell cancer; men > women
|
|
Peripheral lung cancers
|
adenocarcinoma; women > men
|
|
Squamous lung cancer
|
cavitate; secrete PTH-related protein
|
|
Small cell carcinoma
|
neuroendocrine tumor; secrete ACTH and ADH
|
|
Bronchioloalveolar carcinoma
|
no smoking relationship; lung consolidation resembling pneumonia
|
|
Scar carcinoma
|
usually adenocarcinoma developing in old TB scar
|
|
Bronchial carcinoid
|
low grade malignant; hemoptysis; rare cause carcinoid syndrome
|
|
Metastatic lung cancer
|
more common than primary cancer; breast cancer MCC
|
|
Pancoast tumor
|
squamous carcinoma posterior mediastinum; destruction superior cervical ganglion
|
|
S/S
|
Horner’s syndrome - lid lag, miosis, anhydrosis; lower brachial plexus injury
|
|
Solitary coin lesion
|
granuloma MCC
|
|
Superior vena caval syndrome
|
primary lung cancer obstructs vessel; venous congestion
|
|
Anterior mediastinal masses
|
thymoma; nodular sclerosing Hodgkin’s; teratomas
|
|
Posterior mediastinal masses
|
usually neurogenic tumors of ganglia
|
|
Myasthenia gravis
|
B cell hyperplasia of thymus MC abnormality; association with thymoma
|
|
Thymoma
|
association with hypogammaglobulinemia, autoimmune disease, pure RBC aplasia
|
|
Pleural effusions
|
transudates or exudates; CHF MCC
|
|
Spontaneous pneumothorax
|
rupture subpleural or intrapleural bleb; air/pleural cavity pressure same
|
|
S/S
|
pleuritic chest pain, dyspnea, tracheal shift ipsilateral side, absent breath sounds
|
|
Tension pneumothorax
|
flap-like pleural tear; increased pleural cavity pressure; compression atelectasis
|
|
S/S
|
as above except tracheal shift to opposite side
|
|
Cleft lip/palate
|
failure of fusion of facial processes
|
|
Herpes labialis
|
multinucleated giant cell with acidophilic intranuclear inclusions on Tzanck prep
|
|
Hairy leukoplakia
|
EBV glossitis; pre-AIDS defining lesion; not precursor to cancer
|
|
Mumps
|
bilateral parotitis; unilateral orchitis; ↑ amylase
|
|
Diphtheria
|
pseudomembrane pharynx and trachea with cervical lymphadenopathy
|
|
Congenital syphilis
|
notched central incisors
|
|
Actinomycosis
|
anaerobic gram + filamentous bacteria; complication extracted dental abscess
|
|
S/S
|
draining sinuses with sulfur granules
|
|
Exudative tonsillitis
|
majority are viral; 20% group A streptococcus
|
|
Oral thrush
|
common in newborn; pre-AIDS defining lesion; yeasts and pseudohyphae
|
|
Dental caries
|
Streptococcus mutans
|
|
Peutz-Jegher’s syndrome
|
mucosal pigmentation; hamartomatous polyps
|
|
Leukoplakia/erythroleukoplakia
|
biopsy to rule out squamous dysplasia or cancer
|
|
Squamous cell carcinoma
|
smoking and alcohol association; lower lip MC site
|
|
Smokeless tobacco
|
verrucoid squamous cell carcinoma
|
|
Gum hyperplasia
|
phenytoin, pregnancy, scurvy
|
|
Pleomorphic adenoma
|
MC benign tumor of salivary glands; parotid MC site
|
|
Mucoepidermoid carcinoma
|
MC malignant tumor major and minor salivary glands
|
|
Dysphagia for solids only
|
lesion obstructing esophagus; e.g., cancer, web
|
|
Plummer-Vinson syndrome
|
iron deficiency anemia causes esophageal web, glossitis, achlorhydria (↓ HCl in gastric acid)
|
|
Dysphagia for solids and liquids
|
motor abnormality; e.g., achalasia MCC, PSS or CREST syndrome
|
|
TE fistula
|
polyhydramnios; proximal esophagus ends blindly; distal esophagus derives from trachea
|
|
VATER syndrome
|
vertebral abnormalities, anal atresia, TE fistula, renal disease/radius abnormality
|
|
Zenker’s diverticulum
|
MC pulsion diverticulum of esophagus; halitosis (stinky breath, food gets stuck); near UES
|
|
GERD
|
relaxation of lower esophageal sphincter (LES) with acid reflux
|
|
GERD
|
MCC nocturnal cough and asthma
|
|
AIDS esophagitis
|
Candida MC, CMV, HSV
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Barrett’s esophagus
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glandular metaplasia distal esophagus in GERD
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Complications of Barrett’s
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precursor for adenocarcinoma, stricture
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Esophageal varices
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dilated left gastric vein; sign of portal hypertension due to cirrhosis
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Mallory Weiss syndrome
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tear of distal esophagus from retching in alcoholic or bulimic
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Boerhaave’s syndrome
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rupture of distal esophagus from retching; pneumomediastinum
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Hamman’s mediastinal crunch
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pneumomediastinum (air in subcutaneous tissue)
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LES ganglion cells
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contain VIP - relaxes LES
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Achalasia
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failure of LES relaxation (no VIP); absent ganglion cells in the myenteric plexus
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S/S
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aperistalsis/dilation of esophagus; regurgitation of undigested food at night
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X-ray achalasia
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bird’s beak appearance
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Acquired achalasia
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Chagas’ disease; leishmania destroy ganglion cells
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Distal adenocarcinoma esophagus
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MC primary cancer; due to Barrett’s esophagus
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Squamous cell carcinoma of esophagus
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smoking MCC; alcohol also causes
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Melena
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sign of upper GI bleed; acid changes Hb to hematin; peptic ulcer disease MCC
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Hematemesis
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vomiting blood; peptic ulcers MCC
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Congenital pyloric stenosis
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hypertrophy pyloric muscle; vomiting non-bile stained fluid in 2-4 weeks
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Acute hemorrhagic (erosive) gastritis
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NSAIDs MCC
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Mucous barrier stomach
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maintained by PGE; misoprostol PGE analog
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Type A chronic gastritis
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due to PA; achlorhydria with ↑ serum gastrin
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Type B chronic gastritis
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due to H. pylori; involves pylorus and antrum
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H. pylori
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curved rod; urease producer; MCC PUD, adenocarcinoma, gastric lymphoma
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Gastric ulcer
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lesser curvature pylorus and antrum; poor defense against acid; food aggravates pain
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Duodenal ulcer
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never malignant; ↑ acid production; food relieves pain
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Perforated peptic ulcer
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air under diaphragm causes pain in left shoulder
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Menetrier’s disease
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giant rugal hyperplasia; protein loss from increased mucus
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Zollinger-Ellison syndrome
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malignant islet cell tumor secreting gastrin; part of MEN I syndrome
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S/S
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PUD in usual locations; sometimes multiple ulcers
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Hypergastrinemia
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ZE, achlorhydria, gastric distention, H2 or proton blockers; renal failure
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Leiomyoma
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MC benign tumor of stomach
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Intestinal type adenocarcinoma
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H pylori related; ↓ incidence; lesser curvature pylorus/antrum
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Diffuse type adenocarcinoma
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linitis plastica; signet ring cells; Krukenberg tumors ovaries
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Gastric lymphoma
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stomach MC site for extranodal lymphomas; H. pylori associated
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Malabsorption
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steatorrhea; chronic pancreatitis, bile salt deficiency, small bowel disease
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Causes bile salt deficiency
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liver disease, bile salt resins, cholestasis, bacterial overgrowth, Crohn’s
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D-xylose screen
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failure to reabsorb xylose indicates small bowel disease
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Calcification of pancreas
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chronic pancreatitis cause of malabsorption
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Celiac disease
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autoimmune disease; antibodies against gliadin in gluten; flat villi
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Celiac disease
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association with dermatitis herpetiformis
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Whipple’s disease
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systemic infection; foamy macrophages with bacteria (PAS+ inclusions) in small bowel submucosa
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S/S
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fever, polyarthritis, skin pigmentation
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Invasive diarrhea
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Campylobacter jejuni MCC; positive fecal smear for leukocytes
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Secretory diarrhea
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loss isotonic fluid; enterotoxins from E. coli and V. cholerae
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Osmotic diarrhea
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hypotonic loss fluid; laxatives, lactase deficiency
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Rotavirus
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MCC diarrhea in children
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Norwalk virus
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MCC diarrhea in adults
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Cytomegalovirus
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common cause diarrhea in AIDS; MCC cholecystitis and pancreatitis in AIDS
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Staphylococcus aureus
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preformed toxin causes food poisoning; culture food
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Bacillus cereus
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preformed toxin in fried rice and tacos; gram positive rods in stool
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Clostridium botulinum (adult)
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preformed neurotoxin (blocks acetylcholine release); paralysis and mydriasis
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Clostridium botulinum (child)
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colonization of bowel with release of neurotoxin; eating honey
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Clostridium difficile
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pseudomembranous colitis; post-antibiotics; toxin assay stool; Rx metronidazole
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Shigella sonnei
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produces dysentery (bloody diarrhea); associated with HUS
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Salmonella enteritidis
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gastroenteritis; animal reservoirs - poultry, turtles
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Salmonella paratyphi
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sepsis; osteomyelitis in HbSS
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Salmonella typhi
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typhoid fever; human transmission; bradycardia, neutropenia, splenomegaly
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Carrier state site
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gallbladder
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M. tuberculosis
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MCC intestinal TB in United States (swallow TB); Peyer’s patch site of infection
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Enterotoxigenic E. coli
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secretory diarrhea (traveler’s diarrhea); toxin stimulates guanylate cyclase
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Vibrio cholerae
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secretory diarrhea; toxin stimulates adenylate cyclase to produce cAMP
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Oral Rx cholera
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solution must contain glucose to reabsorb Na+ (co-transport)
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Yersinia enterocolitica
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mesenteric lymphadenitis; sepsis in iron overload states
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Entamoeba histolytica
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dysentery; trophozoites phagocytose RBCs; liver abscess; Rx metronidazole
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Cryptosporidium parvum
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MCC diarrhea in AIDS; acid-fast oocysts
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Giardia lamblia
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MC protozoal cause of diarrhea; cause of malabsorption; Rx metronidazole
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Trichuris trichiura
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rectal prolapse in children
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Enterobius vermicularis
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anal pruritus; urethritis in girls; no eosinophilia
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Ascaris lumbricoides
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intestinal obstruction due to adult worms; no eosinophilia
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Necator americanus
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hookworm; iron deficiency anemia
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Strongyloides stercoralis
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rhabditiform larvae in stool not eggs
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Diphyllobothrium latum
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fish tapeworm; vitamin B12 deficiency
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Signs of small bowel obstruction
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colicky pain; constipation and obstipation
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Radiograph small bowel obstruction
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air-fluid levels on x-ray
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MCC small bowel obstruction
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adhesions from previous surgery
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Duodenal atresia
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vomiting bile-stained fluid at birth; double bubble sign; Down syndrome
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Hirschsprung disease
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absent ganglion cells in submucosal/myenteric plexus rectosigmoid
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S/S
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proximal bowel dilated but peristalses; no stool in rectal vault
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Hirschsprung association
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Down syndrome; Chagas disease
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Intussusception
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terminal ileum telescopes into cecum; obstruction plus bloody diarrhea
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Meconium ileus
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complication of cystic fibrosis
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Indirect inguinal hernia
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second MCC of small bowel obstruction; common in weight lifting
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Gallstone ileus
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obstruction of small bowel with gallstone + air in biliary tree
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Volvulus
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MC due to sigmoid colon twisting around mesentery
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Direct inguinal hernia
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protrudes through center of triangle of Hesselbach; no obstruction
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Umbilical hernia
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common in black children; may entrap bowel in adults
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Sigmoid colon
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MC site for polyps, cancer, diverticula
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Small bowel infarction
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diffuse abdominal pain with bloody diarrhea
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Causes small bowel infarction
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embolism (atrial fibrillation), thrombosis SMA or SMV
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Ischemic colitis
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splenic flexure pain with bloody diarrhea
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Mesenteric angina
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pain in splenic flexure 30 minutes after eating
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Angiodysplasia
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submucosal dilation of venules in cecum; cause of hematochezia
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Hematochezia
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massive loss of blood per rectum; diverticulosis MCC
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Meckel’s diverticulum
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persistence omphalomesenteric duct
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S/S
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bleeding MC (iron deficiency in children), diverticulitis
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Meckel’s diverticulitis
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mimics acute appendicitis; cannot differentiate without radionuclide scan
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Sigmoid diverticulum
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diverticulitis MC complication; MCC hematochezia and fistula formation
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Diverticulitis
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“left-sided acute appendicitis”
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Ulcerative colitis
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mucosal/submucosal ulceration; starts in rectum; crypt abscess; ↑ risk adenocarcinoma
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S/S
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left lower quadrant crampy pain with bloody diarrhea
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UC associations
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primary sclerosing cholangitis, seronegative HLA B27 + spondyloarthropathy
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Crohn’s disease
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transmural inflammation; terminal ileum involved 80%; granulomas; skip lesions
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S/S
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colicky pain and diarrhea; fistulas (anal, bowl to bowel)
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Carcinoid tumor
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appendix MC site; terminal ileum MC site for carcinoid syndrome
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Carcinoid syndrome
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liver metastasis; flushing/diarrhea due to serotonin; increased urine 5-HIAA
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Tubular adenomas
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precursor lesion colon cancer; size and number determine risk of malignancy
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Villous adenoma
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greatest risk for colon cancer (30%); secrete mucus rich in protein and potassium
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Familial polyposis
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AD with 100% penetrance for developing colon cancer
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Gardner’s syndrome
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AD, polyposis plus osteomas and desmoid tumors
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Turcot’s syndrome
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AD, polyposis plus brain tumors
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Colorectal cancer
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second MC cancer and cancer killer in adults
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Left-sided colorectal cancer
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obstruct; MC location rectosigmoid
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Right-sided colorectal cancer
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bleed
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Acute appendicitis
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due to lymphoid hyperplasia in children and obstruction by fecalith in adults
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External hemorrhoids
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thrombose
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Internal hemorrhoids
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bleed; prolapse out of rectum
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Urobilinogen (UBG)
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breakdown product CB in bowel (color of stool)
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UBG
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enterohepatic circulation to liver and kidney (color of urine)
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Alcoholic liver disease
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serum AST>ALT; ↑ serum GGT
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Viral hepatitis
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serum ALT>AST
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Cholestasis markers
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serum AP and GGT
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Unconjugated bilirubin
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macrophage degradation of heme; lipid soluble; never in urine
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Conjugated bilirubin (CB)
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water soluble; never normal in urine
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% CB <20% (unconjugated)
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Gilberts, spherocytosis, physiologic jaundice newborn, ABO/Rh HDN
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Gilbert’s disease
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AD; ↓ uptake and conjugation; bilirubin increases with fasting
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Physiologic jaundice newborn
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unconjugated hyperbilirubinemia; begins on day three
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% CB 20-50%
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viral/alcoholic hepatitis
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% CB >50%
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bile duct obstruction (intra or extrahepatic); carcinoma head of pancreas
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Negative urine bilirubin + trace urobilinogen
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normal urine
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Positive urine bilirubin, absent urobilinogen
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obstructive jaundice
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Positive urine bilirubin + increased urobilinogen
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hepatitis
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Negative urine bilirubin + increased urobilinogen
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extravascular hemolytic anemia
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Markers of severity of liver disease
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albumin, PT
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Hepatitis A
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protective antibodies; day care centers, jails, homosexuals, traveling; not chronic
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Hepatitis B
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protective antibodies; accidental needle stick, IVDA; hepatocellular carcinoma
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Hepatitis C
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no protective antibodies; post-transfusion hepatitis; chronic state; hepatocellular carcinoma
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Hepatitis D
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no protective antibodies; requires HBsAg to replicate
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Anti-HBs alone
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vaccination
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Anti-HBs + anti-HBc-IgG
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recovered from HBV
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HBsAg + HBeAg + HBVDNA + anti-HBc-IgM
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acute HBV/chronic HBV infective carrier if >6 months
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Anti HBc-IgM alone
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serologic gap; not infective
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HBsAg + anti-HBc-IgM
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chronic HBV healthy carrier
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Fulminant hepatic failure
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viral hepatitis and acetaminophen MCCs
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Spontaneous peritonitis
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E. coli in adults; S. pneumoniae in children; complication of ascites
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Granulomatous hepatitis
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TB MC bacteria
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Amebiasis
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Entamoeba histolytica; flash shaped ulcers in cecum; liver abscess; Rx
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Echinococcosis
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Echinococcus granulosis; sheep dog definitive host; man intermediate host
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Schistosomiasis
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Schistosoma mansoni; adult worms in portal vein; “pipe stem cirrhosis”
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Clonorchiasis
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Clonorchis sinensis; ingesting encysted larvae in fish; cholangiocarcinoma
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Congestive hepatomegaly (centrilobular necrosis)
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“nutmeg” liver; RHF MCC
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Hepatic vein thrombosis
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Budd-Chiari syndrome; painful hepatomegaly; ascites; portal hypertension
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Portal vein thrombosis
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ascites, portal hypertension, no hepatomegaly
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Alcohol related disorders
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fatty change; alcoholic hepatitis; cirrhosis
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Hypertriglyceridemia in alcoholics
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↑ synthesis of glycerol 3P (substrate for TG synthesis)
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Hypoglycemia in alcoholics
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↓ gluconeogenesis (↑ NADH causes pyruvate to convert to lactate)
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Ketoacidosis in alcoholics
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↑ lactate, ↑ ßOHB (acetyl CoA converted to AcAc and then ßOHB)
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Primary biliary cirrhosis
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granulomatous destruction triad bile ducts; anti-mitochondrial antibody
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Primary sclerosing cholangitis
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association with ulcerative colitis; MCC of cholangiocarcinoma
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Extrahepatic biliary atresia
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neonatal cholestasis
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Drugs causing hepatitis
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acetaminophen, isoniazid, halothane
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Anabolic steroids
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intrahepatic cholestasis
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Estrogen/oral contraceptives
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intrahepatic cholestasis; hepatic adenoma (intraperitoneal hemorrhage)
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Methotrexate
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liver fibrosis, fatty change
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Liver angiosarcoma
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vinyl chloride
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Hemochromatosis
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AR; increased iron reabsorption; liver target organ
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S/S
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cirrhosis; “bronze diabetes” - skin pigmentation + destruction of islet cells; malabsorption
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Lab
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↑ serum ferritin, iron, % saturation; ↓ TIBC
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Wilson’s disease
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AR disease; defect in copper excretion in bile and synthesis of ceruloplasmin
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S/S
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cirrhosis, movement disorder (necrosis in putamen), Kayser Fleisher ring (Descemet’s membrane)
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Lab
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↓ ceruloplasmin (causes ↓ total copper); ↑ serum/urine free copper
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HELLP syndrome
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pre-eclampsia; Hemolytic anemia, ELevated transaminases, Low Platelets
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AAT deficiency in child
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AR, cannot secrete AAT from liver cell; cirrhosis; hepatocellular carcinoma
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Reye syndrome
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coma and microvesicular fatty change post viral infection; increased ammonia
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Cirrhosis
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irreversible fibrosis; regenerative nodules; portal hypertension
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Causes cirrhosis
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alcohol (MC), HBV/HCV, hemochromatosis, Wilson’s, AAT deficiency, 1° biliary
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Hepatic encephalopathy
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mental status changes; ↑ serum ammonia
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Portal hypertension
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ascites; varices; splenomegaly; hemorrhoids; caput medusae
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Cause of ascites
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portal hypertension; hypoalbuminemia; secondary aldosteronism
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Rx
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use aldosterone blocker (acidosis increases loss ammonium in stool)
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Hyperestrinism in men
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gynecomastia; spider angiomas; female hair distribution
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Lab findings cirrhosis
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↓ BUN, glucose, sodium, potassium, calcium (↓ vitamin D); ↑ PT
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Liver cell adenoma
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estrogen related (steroids, oral contraceptives); intraperitoneal hemorrhage
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Liver cancer
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metastasis MC cancer; lung cancer MC primary site
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Hepatocellular carcinoma
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chronic HBV and HCV MCC; ↑ AFP; hepatic/portal vein invasion
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Cholangiocarcinoma
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primary sclerosing cholangitis MCC, C.C sinensis
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Pathogenesis of cholesterol stones
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bile with too much cholesterol and too little bile salts
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Black pigment stones
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sign of extravascular hemolytic anemia (spherocytosis, HbSS)
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Acute cholecystitis
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stone impacted in cystic duct; right upper quadrant colicky pain with radiation to shoulder
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Chronic cholecystitis
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chemical inflammation
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Gallbladder cancer
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risk factors - cholelithiasis and porcelain gallbladder
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Acute pancreatitis
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causes - alcohol and gallstones; ↑ amylase and lipase (more specific)
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S/S
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epigastric pain with radiation into back
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Sentinel loop
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localized ileus of duodenum due to acute pancreatitis
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Pancreatic pseudocyst
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abdominal mass; persistence of ↑ serum amylase >1 week
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Chronic pancreatitis
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alcohol abuse, CF; malabsorption, pain, type I diabetes
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Pancreatic cancer
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smoking MCC
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S/S
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jaundice/acholic (gray/pale) stools; palpable gallbladder; superficial migratory thrombophlebitis (Trousseau's sign); ↑ CA 19-9
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