GASTROENTEROLOGY LITERATURE REVIEW SESSION
September 20, 2001
Banerjee S, Owen C, Chopra S. Sickle Cell Hepatopathy. Hepatology 2001;33:1021-1028.
1. When interpreting abnormal liver enzyme test results in patients with sickle cell disease:
a. Elevated levels of AST reflect ischemic liver injury
b. elevated bilirubin is universally present in SS patients, levels correlate with
LDH levels
c. ALT is the preferred marker of liver injury
d. Alkaline phosphatase elevations during a pain crisis is usually of bone origin
e. Elevated LDH reflect hepatocyte lysis and death
2. Typical findings on liver biopsy from patients with SS disease include
a. increased copper content
b. intrasinusoidal sickling of red cells
c. Kupfer cell hyperplasia
d. erythrophagocytosis
e. sinusoidal dilation
f. hemosiderosis
True or False
3. Acute onset of RUQ pain, worsening jaundice, tender hepatomegaly and elevated liver enzymes in a patients with sickle cell crisis suggest ascending cholangitis.
4. Very high bilirubin levels, mostly conjugated, not associated with encephalopathy, coagulopathy or renal dysfunction is termed “benign hyperbilirubinemia” and typically resolves spontaneously.
5. Massive splenomegaly is a typical finding in adult SS patients with cirrhosis and portal hypertension
6. Hepatic infarcts during sickle cell crisis may lead to development of hepatic abscesses
7. Rapidly progressive hepatomegaly and decreasing hemoglobin levels associated with RUQ pain indicate intra-hepatic bleeding and is an indication for immediate surgery in patients with SS disease.
8. Elective cholecystectomy should be performed in most patients with SS and gallstones to prevent diagnostic confusion when presenting with painful crisis.
9. Sickle cell intrahepatic cholestasis is characterized by
a. extremely high levels of bilirubin, over 50% conjugated
b. impaired renal function
c. the presence of common bile duct stones
d. often associated with encephalopathy
e. coagulopathy and thrombocytopenia are common
10. Zinc in sickle cell disease
a. may be deficient due to renal loses and/or chelation therapy
b. low zinc levels may lead to hyperammonemia and encephalopathy
c. supplementation may decrease number of sickle cell crisis
d. should be supplemented in cirrhotic patients with SS disease