title: Liver failure complicating non-alcoholic steatohepatitis following allogeneic bone marrow transplantation for Shwachman-Diamond syndrome.
abstract: Bone marrow transplantation is potentially curative therapy for the hematologic complications associated with Shwachman-Diamond syndrome (SDS). This syndrome is, however, also associated with significant pancreatic and hepatic dysfunction, which may complicate BMT. We report a case of liver failure due to non-alcoholic steatohepatitis (NASH) following BMT for SDS. This case illustrates the need for assessing liver dysfunction pre-BMT in these patients, in addition to highlighting the potential risk posed by pre-existing steatosis for the development of rapidly progressive hepatic failure following transplantation.
authors.affiliation: Bone Marrow Transplant Service, Department of Clinical Haematology and Medical Oncology, Royal Melbourne Hospital, Melbourne, Australia.
authors.forename: D S
authors.initials: DS
authors.lastname: Ritchie
authors.forename: P W
authors.initials: PW
authors.lastname: Angus
authors.forename: P S
authors.initials: PS
authors.lastname: Bhathal
authors.forename: A P
authors.initials: AP
authors.lastname: Grigg
mesh_terms: D000328:Adult; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D000075202:Contraindications; D010188:Exocrine Pancreatic Insufficiency / Q000150:complications / Q000628:therapy*; D017809:Fatal Outcome; D005234:Fatty Liver / Q000150:complications / Q000209:etiology / Q000473:pathology; D005260:Female; D006505:Hepatitis / Q000150:complications / Q000209:etiology* / Q000473:pathology; D006801:Humans; D017093:Liver Failure / Q000209:etiology* / Q000473:pathology; D009190:Myelodysplastic Syndromes / Q000150:complications / Q000628:therapy; D014184:Transplantation, Homologous / Q000009:adverse effects