对有严重胃肠运动障碍患儿实施的肠管和多器官移植
Intestinal and multivisceral transplantation in children with severe gastrointestinal dysmotility作者机构：Division of Pediatric Pathology Department of Pathology University of Miami/Jackson Memorial Medical Center Miami FL 33136 United States
出 版 物：《世界核心医学期刊文摘（儿科学分册）》 (Dkgest of the World Latest Medical Information)
年 卷 期：2006年第2卷第4期
页 面：29-30页
学科分类：1002[医学-临床医学] 100202[医学-儿科学] 10[医学]
主 题：多器官移植 胃肠运动 先天性巨结肠 选择性治疗 胃肠外营养 免疫组织化学 硬皮病 肠神经 脏器移植
摘 要：Background/Purpose: Severe gastrointestinal dysmotility (GID) impairs patients’ quality of life and is almost uniformly fatal after complications of parenteral nutrition. Intestinal and multivisceral transplants have been used as alternative treatment of these disorders. We studied patients with GID treated with transplantation in our center, and reviewed their outcome to determine the therapeutic efficacy of multivisceral transplants. Methods: The transplant database was searched for patients with GID from 1994 to 2001. We excluded patients with Hirschsprung disease, scleroderma, and diabetic enteropathy. We reviewed explanted organs, histochemistry, and immunohistochemistry and classified cases by etiology. Results: We selected 12 children with GID from 124 patients transplanted. Nine presented before 1 year and 3 started with symptoms between 2 and 8 years. By combined clinical and histopathological features, 6 were classified as megacystis microcolon intestinal hypoperistalsis syndrome, 4 as chronic idiopathic intestinal pseudoobstruction, and 2 as intestinal neuronal dysplasias. Six patients died during the follow-up from 21 to 546 days after transplant. The Kaplan-Meier actuarial survival rates were 66.7% at 1 year and 50% at 3 years. Conclusions: Multivisceral transplantation is a valuable therapeutic alternative for children with severe GID who cannot be adequately managed with parenteral nutrition.
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