Sickle cell disease, extreme hyperbilirubinemia, and pericardial tamponade: Case report and review of the literature

Khurshid, Imtiaz; Anderson, Laura; Downie, Gordon; Pape, Gregory S.

doi:10.1097/00003246-200210000-00029

Cited by 36 publications

(32 citation statements)

References 15 publications

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“…There are several case reports demonstrating that acute SCIC can be reversed with prompt EBT. 3,16,[21][22][23][24] With progressive disease, it is not clear when EBT should be commenced or what the aims of treatment should be. In addition to our patient, there are 2 examples in the literature of the use of a program of EBT to manage chronic SCIC in the nontransplant setting.…”

Section: -19mentioning

confidence: 99%

How we treat sickle hepatopathy and liver transplantation in adults

Gardner

Suddle²,

Kane

et al. 2014

Blood

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Sickle cell disease (SCD) has evolved into a debilitating disorder with emerging end-organ damage. One of the organs affected is the liver, causing “sickle hepatopathy,” an umbrella term for a variety of acute and chronic pathologies. Prevalence of liver dysfunction in SCD is unknown, with estimates of 10%. Dominant etiologies include gallstones, hepatic sequestration, viral hepatitis, and sickle cell intrahepatic cholestasis (SCIC). In addition, causes of liver disease outside SCD must be identified and managed. SCIC is an uncommon, severe subtype, with outcome of its acute form having vastly improved with exchange blood transfusion (EBT). In its chronic form, there is limited evidence for EBT programs as a therapeutic option. Liver transplantation may have a role in a subset of patients with minimal SCD-related other organ damage. In the transplantation setting, EBT is important to maintain a low hemoglobin S fraction peri- and posttransplantation. Liver dysfunction in SCD is likely to escalate as life span increases and patients incur incremental transfusional iron overload. Future work must concentrate on not only investigating the underlying pathogenesis, but also identifying in whom and when to intervene with the 2 treatment modalities available: EBT and liver transplantation.

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Section: -19mentioning

confidence: 99%

How we treat sickle hepatopathy and liver transplantation in adults

Gardner

Suddle²,

Kane

et al. 2014

Blood

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“…It has been proposed that the pathogenesis of SCIC is associated with obstruction of the hepatic sinusoids by sickled RBC and bile with resulting vascular stasis, ballooning of hepatocytes, and local hypoxia. Moreover, SCIC has been reported to have an overall mortality rate of up to 50% in adults and 31% in pediatric patients due to coagulopathy and fulminant liver failure [Khurshid et al 2002].…”

Section: Discussionmentioning

confidence: 99%

Acute liver function decompensation in a patient with sickle cell disease managed with exchange transfusion and endoscopic retrograde cholangiography

Papafragkakis

Ona

Changela

et al. 2014

Therap Adv Gastroenterol

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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.

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“…More than 25 cases of sickle cell, intra-hepatic cholestasis have been reported in the literature and overall mortality rate is about 37%. 39 The major cause of death in these patients is fulminant liver failure, and/or bleeding diathesis. The mortality rate in adult patients is 50% compared to 31% in pediatric patients.…”

Section: Sickle Cell Intra-hepatic Cholestasismentioning

confidence: 99%