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eMediNexus 28 September 2021
Liver involvement in sickle cell disease (SCD) is commonly termed sickle cell hepatopathy (SCH). SCH comes as a complication of SCD which may cause significant mortality. SCH includes all hepatobiliary dysfunction in the context of SCD.
Investigations for Liver Dysfunction in SCD includes-
In Asymptomatic cases:
In Signs of biliary obstruction (jaundice, abdominal pain, pale stool, fever):
In Signs of acute hepatomegaly, abdominal pain +/- jaundice
In Jaundice with/without symptoms and in signs of chronic liver disease
Management of other causes of liver disease- is similar to patients without SCD. For autoimmune liver disease remission is achievable with prednisolone with or without azathioprine or mycophenolate mofetil.
Thus with new and evolving treatments for SCD, the prevalence of SCH may decrease. Liver Transplants (LT) are restricted for very selected patients and specialized centres for managing LT and SCD. Haemopoietic stem cell transplantation (HSCT) may prove curative for SCD and avoid the risk of recurrence of SCH in the donors liver.
Source: Kyrana E, Rees D, Lacaille F, et al. Clinical management of sickle cell liver disease in children and young adults. Arch Dis Child. 2021;106(4):315-320. doi:10.1136/archdischild-2020-319778
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