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Clinical management of sickle cell liver disease in children and young adults
- Source :
- Arch Dis Child
- Publication Year :
- 2020
- Publisher :
- BMJ, 2020.
-
Abstract
- 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.
- Subjects :
- Pediatrics
medicine.medical_specialty
Iron Overload
Adolescent
Digestive System Diseases
medicine.medical_treatment
gastroenterology
Context (language use)
Anemia, Sickle Cell
Disease
Liver transplantation
adolescent health
jaundice
Article
Young Adult
03 medical and health sciences
Liver disease
0302 clinical medicine
polycyclic compounds
medicine
Humans
heterocyclic compounds
Young adult
Child
Monitoring, Physiologic
business.industry
organic chemicals
Incidence
Liver Diseases
Hematopoietic Stem Cell Transplantation
Jaundice
medicine.disease
United Kingdom
Liver Transplantation
carbohydrates (lipids)
Transplantation
030220 oncology & carcinogenesis
Pediatrics, Perinatology and Child Health
Interdisciplinary Communication
medicine.symptom
Complication
business
030215 immunology
Subjects
Details
- ISSN :
- 14682044 and 00039888
- Volume :
- 106
- Database :
- OpenAIRE
- Journal :
- Archives of Disease in Childhood
- Accession number :
- edsair.doi.dedup.....2665e284a21093e74c1e7254604ed9d9