title: Acute liver function decompensation in a patient with sickle cell disease managed with exchange transfusion and endoscopic retrograde cholangiography.
abstract: Sickle cell intrahepatic cholestasis is a relatively uncommon complication of homozygous sickle cell anemia, which may lead to acute hepatic failure and death. Treatment is mainly supportive, but exchange transfusion is used as salvage therapy in life threatening situations. We describe a case of a 16-year-old female with homozygous sickle cell anemia who presented to the emergency room with fatigue, malaise, dark urine, lower back pain, scleral icterus and jaundice. She was found to have marked hyperbilirubinemia, which persisted after exchange transfusion. Because of the concomitant presence of gallstones and choledocholithiasis, the patient underwent endoscopic ultrasound and laparoscopic cholecystectomy followed by endoscopic retrograde cholangiography and sphincterotomy.
authors.affiliation: Department of Gastroenterology, The Brooklyn Hospital Center, 121 DeKalb Ave, Brooklyn, NY 11201, USA.
authors.forename: Haris
authors.initials: H
authors.lastname: Papafragkakis
authors.affiliation: Department of Gastroenterology, The Brooklyn Hospital Center, Brooklyn, NY, USA.
authors.forename: Mel A
authors.initials: MA
authors.lastname: Ona
authors.affiliation: Department of Gastroenterology, The Brooklyn Hospital Center, Brooklyn, NY, USA.
authors.forename: Kinesh
authors.initials: K
authors.lastname: Changela
authors.affiliation: Department of Pediatric Hematology/Oncology, The Brooklyn Hospital Center, Brooklyn, NY, USA.
authors.forename: Swayamprabha
authors.initials: S
authors.lastname: Sadanandan
authors.affiliation: Department of Pediatric Gastroenterology, The Brooklyn Hospital Center, Brooklyn, NY, USA.
authors.forename: Abraham
authors.initials: A
authors.lastname: Jelin
authors.affiliation: Department of Gastroenterology, The Brooklyn Hospital Center, Brooklyn, NY, USA.
authors.forename: Sury
authors.initials: S
authors.lastname: Anand
authors.affiliation: Department of Gastroenterology, The Brooklyn Hospital Center, Brooklyn, NY, USA.
authors.forename: Sushil
authors.initials: S
authors.lastname: Duddempudi
keywords: cholecystitis; choledocholithiasis; cholelithiasis; hepatic crisis; intrahepatic cholestasis; sickle cell disease