학술논문
The liver in sickle cell disease.
Document Type
Academic Journal
Author
Duvoux C; Department of Hepatology and Medical Liver Transplant Unit, Henri Mondor Hospital-APHP, University Paris Est Créteil, France. Electronic address: christophe.duvoux@aphp.fr.; Blaise L; Department of Hepatology and Liver Oncology, Avicenne Hospital-APHP, Bobigny, France.; Matimbo JJ; Department of Hepatology and Medical Liver Transplant Unit, Henri Mondor Hospital-APHP, University Paris Est Créteil, France; Department of Hepatology and Liver Oncology, Avicenne Hospital-APHP, Bobigny, France; Department of Hepatology and Gastroenterology, Clinique Universitaire Kinshasa, Democratic Republic of Congo.; Mubenga F; Agence Régionale de Santé de Guyane, Cayenne, France.; Ngongang N; Department of Hepatology and Medical Liver Transplant Unit, Henri Mondor Hospital-APHP, University Paris Est Créteil, France.; Hurtova M; Department of Hepatology and Medical Liver Transplant Unit, Henri Mondor Hospital-APHP, University Paris Est Créteil, France.; Laurent A; Department of Digestive & HPB Surgery, Henri Mondor Hospital-APHP, University Paris Est Créteil, France.; Augustin J; Department of Pathology, Henri Mondor Hospital-APHP, University Paris Est Créteil, France.; Calderaro J; Department of Pathology, Henri Mondor Hospital-APHP, University Paris Est Créteil, France.; Reizine E; Department of Radiology, Henri Mondor Hospital-APHP, University Paris Est Créteil, France.; Luciani A; Department of Radiology, Henri Mondor Hospital-APHP, University Paris Est Créteil, France.; Habibi A; UMGGR, Department of Internal Medicine, Sickle Cell National Referral Center, Henri Mondor-Hospital APHP, University Paris Est Créteil, France; IMRB, UPEC, INSERM, EFS, Team Pirenne. University Paris Est Créteil, France.; Bachir D; UMGGR, Department of Internal Medicine, Sickle Cell National Referral Center, Henri Mondor-Hospital APHP, University Paris Est Créteil, France.; Vole G; UMGGR, Department of Internal Medicine, Sickle Cell National Referral Center, Henri Mondor-Hospital APHP, University Paris Est Créteil, France; IMRB, UPEC, INSERM, EFS, Team Pirenne. University Paris Est Créteil, France.; Gellen-Dautremer J; Department of Internal Medicine, La Miletrie University Hospital, Poitiers, France.; Leroy V; Department of Hepatology and Medical Liver Transplant Unit, Henri Mondor Hospital-APHP, University Paris Est Créteil, France.; Levesque E; Department of Anesthesia and Surgical Intensive Care-liver ICU, Henri Mondor Hospital-APHP, University Paris Est Créteil, France.; Bartolucci P; UMGGR, Department of Internal Medicine, Sickle Cell National Referral Center, Henri Mondor-Hospital APHP, University Paris Est Créteil, France; IMRB, UPEC, INSERM, EFS, Team Pirenne. University Paris Est Créteil, France.
Source
Publisher: Elsevier Masson Country of Publication: France NLM ID: 8302490 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2213-0276 (Electronic) Linking ISSN: 07554982 NLM ISO Abbreviation: Presse Med Subsets: MEDLINE
Subject
Language
English
Abstract
Liver involvement in SCD patients is frequent but often misdiagnosed or underestimated, except in case of advanced liver diseases. Because of so far poorly recognized forms of chronic SCD-related vascular injury that can silently evolved towards end stages or facilitate ACLF, any persisting liver function tests abnormalities should be carefully investigated, following the above proposed algorithm. Work up and management must be considered multidisciplinary in relationship with a Hepatologist. Early SCD hepatopathy should prompt revision of SCD management to prevent further liver injury and decompensation, discussing transfusion exchanges and hydro urea when not yet initiated, and control for any cofactor of liver injury. The role of HSCT in early SCD hepatopathies also deserves evaluation. In advanced SCD hepatopathies, liver transplantation, which has been rarely performed so far, is the only therapeutic option associated with improved survival. It should definitely be discussed- either electively in case of decompensation in SCD cirrhosis or jaundice/recurrent cholangitis in cholestatic diseases, with excellent outcome, - or emergently in case of ALF or ACLF with more mitigate results. To improve knowledge and management of SCD liver diseases, creation of national and international registries, as well as longitudinal observational cohorts are encouraged.
Competing Interests: Disclosure of Interest The authors declare that they have no competing interest.
(Copyright © 2023. Published by Elsevier Masson SAS.)
Competing Interests: Disclosure of Interest The authors declare that they have no competing interest.
(Copyright © 2023. Published by Elsevier Masson SAS.)