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Liver Update: Clinical Management of Sickle Cell Liver Disease in Children and Young Adults

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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:

  • 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

  • Chronic liver disease workup
  • Liver ultrasound (+/- MRCP)
  • Immunoglobulins, anti-nuclear antibodies (ANA), smooth muscle antibody (SMA) and liver-kidney microsomal antibody (LMK1) [+/- fecal calprotectin, anti-neutrophil cytoplasm antibody(ANCA)]
  • 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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