首例欧洲小儿同时肝胰移植治疗沃-奥二氏综合征
First European Case of Simultaneous Liver and Pancreas Transplantation as Treatment of Wolcott-Rallison Syndrome in a Small Child.
机构信息
Division of Transplantation Surgery, CLINTEC, Karolinska Institutet and Department of Transplantation Surgery, Karolinska University Hospital, Stockholm, Sweden.
Department of Clinical Sciences, Clinical Research Centre, Lund University, Malmö, Sweden.
出版信息
Transplantation. 2020 Mar;104(3):522-525. doi: 10.1097/TP.0000000000002869.
BACKGROUND
The concept of organ transplantation as treatment for complex genetic conditions, including Wolcott-Rallison syndrome (WRS), continues to show promise. Liver transplantation is essential for survival of patients with WRS, and pancreas transplantation cures their type I diabetes mellitus.
METHODS
The recipient, a 3-year-old girl weighing 14 kg at the time of transplantation, suffered from major complications of WRS, including repetitive liver failure episodes and poorly controlled diabetes. The patient underwent a nonacute, combined, simultaneous liver and pancreas transplantation from a pediatric donor without using the en bloc technique.
RESULTS
Well-preserved graft functions at 2-year follow-up with normal liver and pancreas function.
CONCLUSIONS
This is the first case report of simultaneous liver and pancreas transplantation as treatment of WRS in a small child in Europe. Two-year follow-up demonstrates that organ transplantation can halt life-threating recurrent liver failure episodes and cure type 1 diabetes.
背景
器官移植作为治疗复杂遗传疾病的概念，包括沃科特-罗利森综合征（WRS），继续显示出前景。对于 WRS 患者，肝移植是生存的关键，而胰腺移植则可治愈他们的 1 型糖尿病。
方法
受者为一名 3 岁女孩，移植时体重 14 公斤，患有 WRS 的严重并发症，包括反复肝功能衰竭和糖尿病控制不佳。患者接受了来自小儿供体的非急性、联合、同时肝胰腺移植，未使用整块技术。
结果
2 年随访时移植器官功能良好，肝、胰腺功能正常。
结论
这是欧洲首例小儿 WRS 患者接受同时肝胰腺移植治疗的病例报告。2 年随访表明，器官移植可以阻止危及生命的复发性肝功能衰竭发作，并治愈 1 型糖尿病。
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