Specific disorders of bilirubin Flashcards
Disease associated with unconjugated pre-hepatic bilirubin
Hemolytic disease
Laboratory findings in hemolytic disease
Increased B1, normal B2
Defect in hemolytic disease
Increased RBC destruction leading to increased bilirubin production
Diseases with unconjugated hepatic bilirubin
Gilbert syndrome, Lucey-Driscoll syndrome, Physiologic jaundice of the newborn, Crigler-Najjar syndrome
Laboratory findings in unconjugated hepatic diseases
Increased B1, normal B2
Diseases with mixed hepatic bilirubin elevation
Viral hepatitis, Cirrhosis, Hepatic carcinoma
Laboratory findings in mixed hepatic diseases
Increased B1 and B2
Diseases with conjugated/mixed hepatic bilirubin elevation
Dubin-Johnson syndrome, Rotor syndrome
Laboratory findings in Dubin-Johnson and Rotor syndromes
Normal/increased B1, increased B2
Characteristic finding that shows dark pigmentation in hepatocytes
Dubin-Johnson syndrome
Characteristic finding that shows positive cytosolic inclusion bodies
Rotor syndrome
Disease associated with conjugated post-hepatic bilirubin
Biliary obstruction
Laboratory findings in biliary obstruction
Normal B1, increased B2
Defect that is associated with transport defect coupled with UDPGT deficiency
Gilbert syndrome
Defect that is associated with circulating inhibitor of bilirubin conjugation
Lucey-Driscoll syndrome
Defect seen in Physiologic jaundice of the newborn
Transient UDPGT deficiency
Defect in Crigler-Najjar syndrome
Total or partial UDPGT deficiency
Consequence of total UDPGT deficiency in Crigler-Najjar syndrome
Stillbirth (homozygous)
Diseases involving a conjugation defect requiring phototherapy
Lucey-Driscoll syndrome, Physiologic jaundice of the newborn, Crigler-Najjar syndrome
Defect in viral hepatitis, cirrhosis, and hepatic carcinoma
Decreased conjugation and bile flow interference
Defect in Dubin-Johnson and Rotor syndromes
Secretory defect
Cause of biliary obstruction
Extrahepatic cholestasis (cholelithiasis, tumors, strictures)