Liver involvement in sickle cell disease (SCD) is often referred to as sickle cell hepatopathy (SCH) and is a complication of SCD which may be associated with significant mortality. This review is based on a round-table workshop between paediatric and adult hepatologists and haematologists and review of the literature. The discussion was prompted by the lack of substantial data and guidance in managing these sometimes very challenging cases. This review provides a structured approach for the diagnosis and management of SCH in children and young adults. The term SCH describes any hepatobiliary dysfunction in the context of SCD. Diagnosis and management of biliary complications, acute hepatic crisis, acute hepatic sequestration and other manifestations of SCH are discussed, as well as the role of liver transplantation and haemopoietic stem cell transplantation in the management of SCH.
- adolescent health
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Contributors EK, DR, FL, EF, NHe, SH, MSa, FM, VB, AS, SC, ED and AD: participated in the round table discussions and presentations and contributed to the writing and editing of the manuscript. MDa, AV, MDe and MSe contributed in the writing and editing of the manuscript. GG, MV, BI, NHa, TG and JH participated in the round table discussions and presentations and reviewed the manuscript.
Funding The workshop was supported by grant 31966 from the British Heart Foundation.
Competing interests EK, FL, EF, MDa, NHe, SH, MS, FM, VB, AS, AV, MV, BI, ED, NHa, TG, JH, MDe, MS and AD have nothing to disclose. DR is on advisory boards of Novartis and DSMB of AstraZeneca outside the submitted work. GG reports personal fees from Alexion Pharmaceuticals outside the submitted work. SC reports personal fees from Novartis outside the submitted work. VB reports personal fees from Bluebirdbio and from Addmedica, outside the submitted work.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement No data are available. No data are available for this review.
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