Elsabbagh Ahmed M, Hawksworth Jason, Khan Khalid M, Yazigi Nada, Matsumoto Cal S, Fishbein Thomas M
MedStar Georgetown Transplant Institute, Georgetown University Hospital, Washington, DC, USA.
Pediatr Transplant. 2018 Feb;22(1). doi: 10.1111/petr.13082. Epub 2017 Nov 15.
We present a case of a 2-year-old child who underwent a combined en bloc liver and pancreas transplant following complications of WRS. WRS is characterized clinically through infantile insulin-dependent diabetes mellitus, neutropenia, recurrent infections, propensity for liver failure following viral infections, bone dysplasia, and developmental delay. Usually, death occurs from fulminant liver and concomitant kidney failure. Few cases with WRS are reported in the literature, mostly from consanguineous parents. To the best of our knowledge, combined en bloc liver and pancreas transplant has not been performed in small children.
我们报告一例2岁儿童，其在患WRS出现并发症后接受了整块肝脏和胰腺联合移植。WRS的临床特征为婴儿型胰岛素依赖型糖尿病、中性粒细胞减少、反复感染、病毒感染后易发生肝衰竭、骨发育异常和发育迟缓。通常，患者死于暴发性肝衰竭及随之而来的肾衰竭。文献中报道的WRS病例很少，大多来自近亲结婚的父母。据我们所知，整块肝脏和胰腺联合移植尚未在幼儿中进行过。
