Mekeel Kristin L, Langham Max R, Gonzalez-Peralta Regino, Fujita Shiro, Hemming Alan W
Division of Transplantation, Department of Surgery, University of Florida, Gainesville, FL 32610, USA.
Liver Transpl. 2007 Apr;13(4):505-8. doi: 10.1002/lt.20999.
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.
严重肝病是镰状细胞病（SCD）一种不常见但可能致命的并发症。肝移植因SCD持续存在和血栓形成而变得复杂。我们回顾了1990年至2005年在我们机构进行的214例儿科移植手术。3例患者因SCD并发症接受移植，包括肝内胆汁淤积和病毒性肝炎。患者和移植物总体生存率为66%。1例患者在6年后死于硬膜下血肿。没有移植物丢失、原发性无功能或血栓形成的情况发生。所有3例患者术后需要1至4次输血，以使血红蛋白（Hgb）>9 g/dL，S分数低于25%。1例患者术前因血红蛋白S（HbS）分数为30%而需要输血。平均随访时间为4.2年（范围2.6 - 5.4年）。所有3名儿童仍患有SCD的后遗症。1名儿童患有复发性镰状细胞性肝病和慢性移植物功能衰竭。总之，SCD患儿在极少数情况下会发生急性和慢性肝衰竭。这些儿童可以成功接受移植并取得良好的结果。必须密切关注HbS分数和血红蛋白水平，以防止镰状化和血管血栓形成。不幸的是，肝移植无法改变疾病的自然进程。
