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hyperbilirubinemia

Etiology: === unconjugated hyperbilirubinemia === 1) overproduction of bilirubin a) hemolysis 1] intravascular: DIC 2] extravascular: hemoglobinopathy, G6PD deficiency, autoimmune hemolytic anemia 3] bilirubin rarely increases above 4 mg/dL (except neonatal jaundice) b) ineffective erythropoiesis c) hematoma d) blood transfusions 2) decreased hepatic uptake a) prolonged fasting b) sepsis 3) hereditary unconjugated hyperbilirubinemia a) Gilbert's syndrome (jaundice during times of stress) [4] b) Crigler-Najjar syndrome, type I c) Crigler-Najjar syndrome, type II 4) pharmacologic agents a) chloramphenicol: neonatal hyperbilirubinemia b) vitamin K: neonatal hyperbilirubinemia 5) 5-beta-pregnane-3-alpha, 20-alpha-diol: breast milk jaundice 6) physiologic jaundice of the newborn is secondary to inefficient uptake of bilirubin by immature hepatocytes 7) neonatal jaundice a) polycythemia b) pyloric stenosis c) congenital hypothyroidism === conjugated hyperbilirubinemia === 1) inherited disorders a) Dubin-Johnson syndrome b) Rotor syndrome c) recurrent (benign) intrahepatic cholestasis 2) hepatocellular diseases a) viral hepatitis: 1] most common cause of jaundice in young adults 2] serum unconjugated bilirubin 0-20 mg/dL 3] serum transaminases 10-50 times upper limit of normal 4] hepatitis A, hepatitis B, hepatitis C, hepatitis E, Epstein Barr virus, Cytomegalovirus b) alcoholic hepatitis 1] serum AST (SGOT) 6-10 times upper limit of normal 2] serum ALT (SGPT) 2-3 times upper limit of normal c) ischemic hepatitis - serum transaminase 100 times upper limit of normal d) cirrhosis: 1/3 of jaundice in patients age 30-60 years 1] alcoholic cirrhosis - most common etiology in US 2] hepatitis B - most common etiology worldwide 3] cardiac cirrhosis 4] alpha-1 antitrypsin deficiency 5] hemochromatosis 6] Wilson's disease e) malignancy: hepatocellular, metastatic f) abscesses: pyogenic, amebic g) granulomatous infiltration of the liver 1] tuberculosis 2] sarcoidosis 3] lymphoma 4] Mycobacterium avium intracellulare h) toxoplasmosis i) Pneumocystis carinii j) Echinococcus 3) biliary diseases a) biliary cirrhosis: primary, secondary b) primary sclerosing cholangitis c) gallstone disease d) cholangiocarcinoma & ampullary carcinoma e) biliary atresia in neonate 4) pancreatic disease a) pancreas-related stricture of the common bile duct b) tumor of the head of the pancreas 5) pharmacologic agents a) drug-induced hepatitis: - amoxicillin clavulanate (most common) - isoniazid (INH), NSAIDs, Bactrim, zidovudine (AZT), acetaminophen b) drug-induced cholestasis: - perchlorperazine, haloperidol (Haldol), estrogens, androgens, rifampin, allopurinol, sulfonylurea, chlorpromazine 6) sepsis 7) postoperative jaundice 8) galactosemia in neonate 9) cholestatic jaundice of pregnancy 10) helminth infection: Clonorchis, Ascaris Clinical manifestations: 1) jaundice 2) symptoms associated with specific etiologies a) manifestations of cirrhosis - palmar erythema - spider angiomas - gynecomastia - testicular atrophy - ascites - splenomegaly b) manifestations of biliary obstruction - palpable gall bladder - abdominal mass c) manifestations of hepatocellular dysfunction - hepatomegaly - right upper quadrant tenderness d) Kayser-Fleischer rings in Wilson's disease e) edema associated with cardiac cirrhosis 3) weight loss Laboratory: 1) complete blood count (CBC) 2) liver profile a) increased serum alkaline phosphatase 3X upper limit of indicates biliary obstruction b) increased serum transaminases 5X upper limit of normal indicates hepatocellular dysfunction 3) prothrombin time 4) tests for viral hepatitis (hepatitis serology) a) hepatitis A serology b) hepatitis B serology c) hepatitis C serology d) hepatitis E serology e) Epstein Barr virus: 1] heterophile antibody test 2] EBV serology f) serology for Cytomegalovirus 5) special studies as indicated a) antimitochondrial antibodies for primary biliary cirrhosis b) iron studies for hemochromatosis: serum ferritin, serum iron, TIBC, % transferrin saturation c) antinuclear & anti-smooth muscle antibodies for autoimmune hepatitis Special laboratory: - endoscopic retrograde cholangiopancreatography (ERCP) a) invasive procedure b) provides specimen for cytology (suspected malignancy) c) therapeutic modality: 1] removal of common bile duct stone 2] stent placement 3] sphincterotomy Radiology: 1) abdominal ultrasound in all cases of suspected biliary obstruction 2) abdominal CT Management: 1) general measures a) control itching - cholestyramine 1 packet TID - diphenhydramine (Benadryl) b) phenobarbital 60-180 mg/day may reduce serum bilirubin c) abstinence from alcohol 2) treatment of underlying cause a) obstructive jaundice is treated surgically b) Wilson's disease is treated with penicillamine c) hemochromatosis is treated with phlebotomy d) interferon is occasionally used to treat chronic hepatitis B & hepatitis C

Related

bilirubin jaundice (icterus) kernicterus; neonatal jaundice; neonatal hyperbilirubinemia

General

sign/symptom

References

  1. Harrison's Principles of Internal Medicine, 13th ed. Companion Handbook, Isselbacher et al (eds), McGraw-Hill Inc. NY, 1995, pg 830.
  2. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 361-62, 371-73
  3. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 253
  4. NEJM Knowledge+ Gastroenterology