| Liver transplantation in children with sickle-cell disease. | |
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MedLine Citation:
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PMID: 17394147 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Severe liver disease is an unusual but potentially fatal complication of sickle-cell disease (SCD). Liver transplantation has been complicated by ongoing SCD and thrombosis. We reviewed 214 pediatric transplants done at our institution from 1990 to 2005. Three patients were transplanted for complications of SCD, including intrahepatic cholestasis and viral hepatitis. Overall patient and graft survival was 66%. One patient died after 6 years from a subdural hematoma. There were not any incidences of graft loss, primary nonfunction, or thrombosis. All 3 patients required between 1 and 4 postoperative transfusions to keep hemoglobin (Hgb) >9 g/dL with an S fraction of less than 25%. One patient required a preoperative transfusion for a hemoglobin S (HbS) fraction of 30%. Mean follow-up has been 4.2 years (range, 2.6-5.4 years). All 3 children continued to suffer sequelae from their SCD. One child suffered from recurrent sickle-cell hepatopathy and chronic graft failure. In conclusion, children with SCD can in rare instances develop acute and chronic liver failure. These children can be successfully transplanted with good outcomes. Careful attention must be paid to HbS fraction and hemoglobin level to prevent sickling and vascular thrombosis. Unfortunately, liver transplant cannot alter the natural course of the disease. |
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Authors:
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Kristin L Mekeel; Max R Langham; Regino Gonzalez-Peralta; Shiro Fujita; Alan W Hemming |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society Volume: 13 ISSN: 1527-6465 ISO Abbreviation: Liver Transpl. Publication Date: 2007 Apr |
Date Detail:
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Created Date: 2007-04-16 Completed Date: 2007-06-13 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 100909185 Medline TA: Liver Transpl Country: United States |
Other Details:
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Languages: eng Pagination: 505-8 Citation Subset: IM |
Affiliation:
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Division of Transplantation, Department of Surgery, University of Florida, Gainesville, FL 32610, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Anemia, Sickle Cell / complications* Aspartate Aminotransferases / blood Bilirubin / blood Child Graft Survival Humans Liver Cirrhosis / blood, etiology*, surgery* Liver Transplantation* Treatment Outcome |
| Chemical | |
Reg. No./Substance:
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635-65-4/Bilirubin; EC 2.6.1.1/Aspartate Aminotransferases |
| Comments/Corrections | |
Comment In:
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Liver Transpl. 2007 Apr;13(4):483-5
[PMID:
17394145
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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