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:Liver function tests annually or moreLiver ultrasound annually after >5 years of ageFerritin annually if transfused, or monthly if chronically transfusedliver ultrasound, if conjug...
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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:
Liver function tests annually or more
Liver ultrasound annually after >5 years of age
Ferritin annually if transfused, or monthly if chronically transfused
liver ultrasound, if conjugated bilirubin is raised
If ALT +/- γ-GT is raised, investigate every 3 months
If ALT +/- γ-GT is raised twice, then chronic liver disease workup needed
MRI liver, if ferritin is >1000
In Signs of biliary obstruction (jaundice, abdominal pain, pale stool, fever):
Liver ultrasound and Liver function tests
magnetic resonance cholangiopancreatography (MRCP)/ Endoscopic Retrograde cholangio-pancreaticography (ERCP) depending on the pathology
In Signs of acute hepatomegaly, abdominal pain +/- jaundice
Liver ultrasound, Liver function tests, Hb
In Jaundice with/without symptoms and in signs of chronic liver disease
Caeruloplasmin and 24-hour urine copper [ophthalmology review for Kayser-Fleischer (KF) rings]
Hepatitis B, C and E serology
Alpha-1 antitrypsin phenotype
If acute presentation, hepatitis A and other viruses like EBV
Depending on the case endocrine and metabolic tests, cholestasis gene panel, sweat test
Liver biopsy (by hepatology centre)
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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