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11 Cards in this Set

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Gilbert's syndrome; What is the nature of defect?
Reduced activity of glucuronyl transferase
Gilbert's syndrome; What type of hyperbilirubinemia?
Unconjugated (indirect) bilirubin
Gilbert's syndrome; What are the clinical & pathologic characteristics?
Benign, asymptomatic hereditary jaundice. Hyperbilirubinemia increased by 24-36 hr fast. No tx required. Prognosis excellent
Dubin-Johnson/Rotor's syndrome; What is the nature of defect?
Faulty excretory function of hepatocytes.
Dubin-Johnson/Rotor's syndrome; What type of hyperbilirubinemia?
Conjugated (direct) bilirubin
Dubin-Johnson syndrome; What are the clinical & pathologic characteristics?
Benign, asymptomatic hereditary jaundice. Gallbladder does not visualize on oral cholecystography. Liver darkly pigmented on gross exam. Biopsy shows centrilobular brown pigment. Prognosis excellent.
Rotor's syndrome; What are the clinical & pathologic characteristics?
Similar to Dubin-Johnson syndrome, but liver is not pigmented & the gallbladder is visualized on oral cholecystorgraphy. Prognosis excellent.
Benign recurrent intrahepatic cholestasis/Recurrent jaundice of pregnancy; What is the nature of defect?
Cholestasis, often on a familial basis.
Benign recurrent intrahepatic cholestasis/Recurrent jaundice of pregnancy; What type of hyperbilirubinemia?
Unconjugated plus conjugated (total) bilirubin
Benign recurrent intrahepatic cholestasis; What are the clinical & pathologic characteristics?
Episodic attacks of jaundice, itching & malaise. Onset in early life & may persist for life. Alkaline phosphatase↑. Cholestasis found on liver biopsy. (Biopsy is normal during remission.) Prognosis excellent.
Recurrent jaundice of pregnancy; What are the clinical & pathologic characteristics?
Benign cholestatic jaundice of unknown cause, usually in 3rd trimester. Itching, GI sx, abnormal liver excretory function tests, cholestasis noted on liver biopsy, Prognosis excellent, but recurrence w/subsequent pregnancies or use of BC pills is characteristic.