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Flashcards in GI22 Deck (10):
1

Gilbert's syndrome.

-Mildly decreased UDP-glucuronyl transferase or decreased bilirubin uptake
-Asymptomatic
-Elevated unconjugated bilirubin without overt hemolysis
-Bilirubin increases with Fasting and Stress

*NO clinical consequence

2

Crigler-Najjar syndrome Type 1:
- Characteristics

-Absent UDP-glucuronyl transferase
-Presents early in life (die soon after)

3

Crigler-Najjar syndrome Type 1:
-Findings

-Jaundice
-Kernicterus
-Increased unconjugated bilirubin

4

Crigler-Najjar syndrome Type 1:
-Treatment

Plasmapheresis & Phototherapy

5

Crigler-Najjar syndrome Type 2.

-Less severe
-Responds to Phenobarbital (increases liver enzyme synthesis)

6

Dubin-Johnson syndrome.

-Conjugated hyperbilirubinemia
-Defective liver secretion
-Grossly BLACK liver
-Benign

7

Rotor's syndrome.

-Similar to Dubin-Johnson
-Milder
-NO black liver

8

Problem with bilirubin uptake
-Unconjugated bilirubinemia

Gilbert's

9

Problem with bilirubin congestion
-Unconjugated bilirubinemia

Crigler-Najjar

10

Problem with excretion of conjugated bilirubin
- Conjugated bilirubinemia

Dubin-Johnson