Open AccessClinical management of sickle cell liver disease in children and young adults
Author(s) -
Eirini Kyrana,
David C. Rees,
Florence Lacaille,
Emer Fitzpatrick,
Mark Davenport,
Nigel Heaton,
Sue Height,
Marianne Samyn,
Fulvio Mavilio,
Valentine Brousse,
Abid Suddle,
Subarna Chakravorty,
Anita Verma,
Girish Gupte,
Mark Velangi,
Baba Inusa,
Emma Drašar,
Nedim Hadžić,
Tassos Grammatikopoulos,
Jonathan Hind,
Maesha Deheragoda,
Maria E. Sellars,
Anil Dhawan
Publication year - 2020
Publication title -
archives of disease in childhood
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.037
H-Index - 146
eISSN - 1468-2044
pISSN - 0003-9888
DOI - 10.1136/archdischild-2020-319778
Subject(s) - medicine , liver transplantation , context (archaeology) , intensive care medicine , disease , complication , pediatrics , liver disease , transplantation , paleontology , biology
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.