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Liver transplantation in children with sickle-cell disease
Kristin L Mekeel1, Max R Langham, Regino Gonzalez-Peralta
1Division of Transplantation, Department of Surgery, University of Florida, Gainesville, FL 32610, USA.
Insights
Liver transplantation is a viable option for children with severe liver disease due to sickle-cell disease (SCD). Careful management of hemoglobin S (HbS) levels is crucial for successful outcomes in these complex pediatric cases.
Area of Science:
- Pediatric Hepatology
- Hematology
- Transplantation Immunology
Background:
- Severe liver disease is a rare but serious complication of sickle-cell disease (SCD).
- Liver transplantation in SCD patients is challenging due to disease complications like thrombosis.
Purpose of the Study:
- To evaluate the outcomes of pediatric liver transplantation in patients with sickle-cell disease.
- To identify critical factors for successful transplantation in this patient population.
Main Methods:
- Retrospective review of 214 pediatric liver transplants performed between 1990 and 2005.
- Analysis of three patients transplanted for SCD-related liver complications (intrahepatic cholestasis, viral hepatitis).
- Monitoring of patient and graft survival, complications, and transfusion requirements.
Main Results:
- Overall patient and graft survival was 66% among the three SCD patients.
- No instances of graft loss, primary nonfunction, or thrombosis were observed.
- Postoperative transfusions were required to maintain hemoglobin >9 g/dL and HbS fraction <25%.
Conclusions:
- Children with SCD can develop acute and chronic liver failure, amenable to transplantation.
- Successful transplantation is achievable with careful attention to hemoglobin S fraction and levels to prevent sickling.
- Liver transplantation does not alter the underlying progression of sickle-cell disease.
Abstract:
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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