TY - JOUR
T1 - Clinical Management of Sickle Cell Liver Disease in Children and Young Adults
AU - Kyrana, Serena
AU - Lacaille, Florence
AU - Rees, David
AU - Fitzpatrick, Emer
AU - Davenport, Mark
AU - Heaton, Nigel
AU - Height, Sue
AU - Samyn, Marianne
AU - Mavilio, Fulvio
AU - Brousse, Valentine
AU - Suddle, Abid
AU - Chakravorty, Subarna
AU - Verma, A
AU - Gupte, Girish
AU - Velangi, Mark
AU - Inusa, Baba
AU - Drasar, Emma
AU - Hadzic, Dino
AU - Grammatikopoulos, Tassos
AU - Hind, Jonathan
AU - Deheragoda, Maesha
AU - Sellars, M.
AU - Dhawan, Anil
PY - 2020/9/13
Y1 - 2020/9/13
N2 - 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.
AB - 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.
KW - sickle cell disease
KW - Liver
UR - http://www.scopus.com/inward/record.url?scp=85096074778&partnerID=8YFLogxK
U2 - 10.1136/archdischild-2020-319778
DO - 10.1136/archdischild-2020-319778
M3 - Review article
SN - 0003-9888
JO - Archives of Disease in Childhood
JF - Archives of Disease in Childhood
M1 - 319778
ER -