Chronic intrahepatic cholestasis in sickle cell disease requiring exchange transfusion.

O’Callaghan, Adrian; O’Brien, Susan; Ninković, M.; Butcher, G. P.; Foster, Chris; Walters, J. R. F.; Roberts, Irene

doi:10.1136/gut.37.1.144

Cited by 45 publications

(22 citation statements)

References 7 publications

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“…These cases are characterized by right upper quadrant pain, extreme elevation of bilirubin, striking elevation of alkaline phosphatase and variable elevation of transaminases, histological features: intracanalicular cholestasis, sinusoidal dilatation, kupffer cell hyperplasia, and erythrophacocytosis [17]. This clinical and histological picture of chronic intrahepatic cholestasis was reported in one case in the studied group.…”

Section: Discussionmentioning

confidence: 96%

Study of Chronic Hepatopathy in Patients With Sickle Cell Disease

Maher

Mansour

2009

Gastroenterol Res

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BackgroundHepatic lesions in sickle cell disease were studied essentially in autopsy specimens. We investigated chronic hepatopathy in living adults with sickle cell disease and report the clinical, biochemical, and hepatic histological findings in these patients.MethodsA total of 170 adult patients with sickle cell syndrome were prospectively investigated. Clinical and laboratory investigation including liver function tests, serological tests for viral hepatitis, autoimmune hepatitis, and abdominal ultrasonography were performed in all of the patients. Liver biopsies were studied from 27 patients.ResultsThere was clinical evidence of jaundice in 123 (72.4%) patients, 118 (69.4%) patients had palpable liver, and 69% percent of the patients had elevated enzymes. Serological tests demonstrated the presence of hepatitis B infection in 18 (10.6%) patients and hepatitis C infection in 39 (23%) patients, serological markers for autoimmune hepatitis were positive in two female patients, one diagnosed chronic intrahepatic cholestasis. All 27 biopsies presented some degree of sickling hepatopathy. Moderate or marked liver siderosis was associated with the number of transfusions.ConclusionsThe clinical spectrum of sickle cell disease ranges from mild liver function test abnormalities to significant hepatic abnormalities with marked hyperbilirubinemia. Multiple factors may contribute to the etiology of the liver disease, including ischemia, transfusion related viral hepatitis, iron overload, and gallstones.

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Section: Discussionmentioning

confidence: 96%

Study of Chronic Hepatopathy in Patients With Sickle Cell Disease

Maher

Mansour

2009

Gastroenterol Res

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“…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. 19,25 Maintaining HbS levels at ,20% to 30% has been proposed, but again this is based on pragmatism rather than evidence. Future work must explore the natural history of SCIC in order to identify which patients are at risk of disease progression and which would benefit from regular EBT.…”

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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“…Additionally, a case of chronic recurrent intra-hepatic cholestasis responding to exchange transfusion has been described. 42 Hepatic iron overload Patients with SCD are at higher risk of iron overload and damage to the liver from chronic transfusion. Serum ferritin levels in these patients correlate with the number of units of blood transfused.…”

Section: Sickle Cell Intra-hepatic Cholestasismentioning

confidence: 99%