GI22 Flashcards
1
Q
Gilbert’s syndrome.
A
- 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
Q
Crigler-Najjar syndrome Type 1:
- Characteristics
A
- Absent UDP-glucuronyl transferase
- Presents early in life (die soon after)
3
Q
Crigler-Najjar syndrome Type 1:
-Findings
A
- Jaundice
- Kernicterus
- Increased unconjugated bilirubin
4
Q
Crigler-Najjar syndrome Type 1:
-Treatment
A
Plasmapheresis & Phototherapy
5
Q
Crigler-Najjar syndrome Type 2.
A
- Less severe
- Responds to Phenobarbital (increases liver enzyme synthesis)
6
Q
Dubin-Johnson syndrome.
A
- Conjugated hyperbilirubinemia
- Defective liver secretion
- Grossly BLACK liver
- Benign
7
Q
Rotor’s syndrome.
A
- Similar to Dubin-Johnson
- Milder
- NO black liver
8
Q
Problem with bilirubin uptake
-Unconjugated bilirubinemia
A
Gilbert’s
9
Q
Problem with bilirubin congestion
-Unconjugated bilirubinemia
A
Crigler-Najjar
10
Q
Problem with excretion of conjugated bilirubin
- Conjugated bilirubinemia
A
Dubin-Johnson
