Department of Pediatric Surgery, Hospital Universitario La Paz, Paseo de Castellana 261, 28046 Madrid, Spain.
J Pediatr Surg. 2010 Feb;45(2):330-6; discussion 336. doi: 10.1016/j.jpedsurg.2009.10.071.
Graft-vs-host disease (GVHD) is a rare complication of transplantation of organs rich in immunocompetent cells. The goal of this study was to report the features of GVHD after small bowel transplantation (SBTx) in children.
The study involved a retrospective review of patients undergoing SBTx between 1999 and 2009 who had GVHD.
Of 46 children receiving 52 intestinal grafts (2 liver-intestine and 3 multivisceral), 5 (10%) developed GVHD. Median age at transplant was 42 (19-204) months. Baseline immunosupression consisted of tacrolimus and steroids supplemented with thymoglobulin (n = 2) or basiliximab (n = 3) for induction. Median time between transplantation and GVHD was 47 (16-333) days. All patients had generalized rash, 2 had diarrhea, and 2 had respiratory symptoms. Other symptoms were glomerulonephritis (n = 1) and conjunctivitis (n = 1). Four developed severe hematologic disorders. The diagnosis was confirmed by skin biopsy in 4 patients and supported by chimerism studies in two. Colonoscopy and opthalmoscopic findings were also suggestive in one. Treatment consisted of steroids and decrease of tacrolimus, with partial response in four. Other immunosuppressants were used in refractory or recurrent cases. Three patients died within 4 months after diagnosis.
Graft-vs-host disease is a devastating complication of SBTx, with high mortality probably associated with severe immunologic dysregulation.
移植物抗宿主病（GVHD）是富含免疫活性细胞的器官移植的罕见并发症。本研究的目的是报告儿童小肠移植（SBTx）后 GVHD 的特征。
本研究回顾性分析了 1999 年至 2009 年间接受 SBTx 且发生 GVHD 的患者。
在接受 52 个肠移植物（2 个肝肠和 3 个多脏器）的 46 名儿童中，有 5 名（10%）发生了 GVHD。移植时的中位年龄为 42（19-204）个月。基线免疫抑制包括他克莫司和类固醇，并用胸腺球蛋白（n = 2）或巴利昔单抗（n = 3）进行诱导。GVHD 发生的中位时间为移植后 47（16-333）天。所有患者均有全身性皮疹，2 例有腹泻，2 例有呼吸道症状。其他症状包括肾小球肾炎（n = 1）和结膜炎（n = 1）。4 例出现严重血液系统疾病。4 例患者通过皮肤活检确诊，2 例患者通过嵌合体研究支持诊断。1 例结肠镜检查和眼底检查结果也有提示。治疗包括皮质类固醇和他克莫司减量，4 例有部分缓解。在难治性或复发性病例中使用了其他免疫抑制剂。3 例患者在诊断后 4 个月内死亡。
GVHD 是 SBTx 的一种破坏性并发症，死亡率高，可能与严重的免疫失调有关。
