Sickle cell intrahepatic cholestasis with cholelithiasis.
Sickle cell intrahepatic cholestasis (SCIC) is a rare complication seen in sickle cell patients who present with sudden onset of RUQ pain, progressive hepatomegaly, mild elevation of transaminases, coagulopathy, and extreme hyperbilirubinemia. Early recognition of this entity is essential to avoid life-threatening complications. Diagnosis can be challenging given the overlap in clinical presentation with other conditions affecting the hepatobiliary biliary system in sickle cell anemia such as hepatitis, cholecystitis, and hepatic crisis. Treatment is currently limited to exchange transfusion. The authors present two patients with SCIC and cholelithiasis; the clinical picture of one is complicated by choledocholithiasis.
Irizarry
Karina
K
Department of Pediatrics, University of South Florida College of Medicine, Tampa, Florida, USA. mjwilsey@tampabay.rr.com
Rossbach
Hans Christof
HC
Ignacio
Joseph R A
JR
Winesett
Michele P
MP
Kaiser
Greg C
GC
Kumar
Mudra
M
Gilbert-Barness
Enid
E
Wilsey
Michael J
MJ
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications*; D001803:Blood Transfusion; D002648:Child; D002769:Cholelithiasis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male; D014812:Vitamin K / Q000627:therapeutic use
D014812:Vitamin K