Liver Transplant for Primary Hyperoxaluria Type 1: Results of Sequential, Combined Liver and Kidney, and Preemptive Liver Transplant
Liver Transplant for Primary Hyperoxaluria Type 1: Results of Sequential, Combined Liver and Kidney, and Preemptive Liver Transplant
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DOI：
10.6002/ect.2019.0150
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发表时间：
2021-05-01
影响因子：
0.9
通讯作者：
Malek-Hosseini, Seyed Ali
中科院分区：
文献类型：
--
作者：
Horoub, Rafat;Shamsaeefar, Alireza;Malek-Hosseini, Seyed Ali
目的：原发性高草酸尿症1型是一种常染色体隐性遗传疾病，可引起草酸的过量产生和尿排泄。肝移植已被建议作为治疗原发性高尿酸1型，因为有缺陷的酶在肝脏中表达。本研究旨在探讨肝肾联合、序贯和先行肝移植治疗1型原发性高尿症患者的结果。材料和方法：在本队列研究中，我们随访了在伊朗设拉子中心接受肝移植的1型原发性高尿症患者。收集患者的临床和实验室数据，并记录主要结局，包括肝移植后肾衰竭、排斥反应和死亡率。采用Kaplan-Meier法分析患者的生存情况。男性16例（66.6%），女性8例（33.33%）。13例患者属于儿科年龄组（年龄< 18岁），11例患者为成人（年龄>= 18岁）。13例患者接受了序贯移植，8例患者接受了肝肾联合移植，3例患者接受了抢先移植。所有患者都接受了已故捐赠者的器官。序贯移植与肝肾联合移植在移植后死亡率、排斥反应和血液透析方面无统计学差异（P > 0.05）。结论：肝移植可作为1型原发性高尿酸血症患者的治疗方法。肝肾联合移植和先行肝移植可能是这些患者的适当选择。
Objectives: Primary hyperoxaluria type 1 is an autosomal recessive disorder that causes overproduction and urinary excretion of oxalate. Liver transplant has been suggested as a treatment for primary hyperoxaluria type 1 since the defective enzyme is expressed in the liver. This study aimed to investigate results of combined liver and kidney, sequential, and preemptive liver transplant in patients with primary hyperoxaluria type 1.Materials and Methods: In this cohort study, we followed patients with primary hyperoxaluria type 1 who underwent liver transplant at our center in Shiraz, Iran. Clinical and laboratory data of patients were gathered, and major outcomes, including renal failure after liver transplant, rejection, and mortality were recorded. Survival of patients was analyzed by the Kaplan-Meier method.Results: Our study included 24 patients. There were 16 male (66.6%) and 8 female (33.33%) patients. Thirteen patients were in the pediatric age group (age < 18 y), and 11 patients were adults (age >= 18 y). Thirteen patients underwent sequential transplant, 8 patients underwent combined liver and kidney transplant, and 3 patients underwent preemptive transplant. All patients received organs from deceased donors. There were no statistically significant differences in mortality, rejection, and hemodialysis after transplant between those with sequential transplant and those with combined liver and kidney transplant (P > .05).Conclusions: Liver transplant can be considered a treatment for patients with primary hyperoxaluria type 1. Combined liver and kidney transplant and preemptive liver transplant could be proper options for these patients.