一家肠道移植中心的瓦登堡综合征肠道功能衰竭患儿的预后:病例系列。

IF 0.7 4区 医学 Q4 TRANSPLANTATION
Experimental and Clinical Transplantation Pub Date : 2025-07-01 Epub Date: 2020-09-17 DOI:10.6002/ect.2020.0166
Yeliz Cagan Appak, Tuba Tinastepe, Betul Aksoy, Soysal Turhan, Cem Tugmen, Ismail Sert, Masallah Baran
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引用次数: 0

摘要

瓦登堡综合征是一种以听力损失和色素异常为特征的遗传病。华登堡综合征 4 型非常罕见,患有华登堡综合征 4 型的儿童会出现肠套叠。华登堡综合征 4 型的相关结果和严重程度在不同病例之间也会有很大差异。肠功能不全很可能会给治疗带来困难,可能需要进行肠移植。在本病例报告中,我们介绍了 4 例有肠道问题的瓦登堡综合征患者,其中 2 例接受了小肠移植手术。对于有胃肠道表现的瓦登堡四型综合征患者,应采取适当的手术和营养治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Outcome of Children With Intestinal Failure Due to Waardenburg Syndrome From an Intestinal Transplant Center: A Case Series.

Waardenburg syndrome is a genetic disease char-acterized by hearing loss and pigmentation abnor-malities. Waardenburg syndrome type 4 is very rare, and children with Waardenburg syndrome type 4 present with intestinal aganglionosis. The associated findings and severity of Waardenburg syndrome type 4 may also differ significantly between cases. Intestinal insufficiency is probable and creates difficulties in terms of treatment; intestinal transplant may be required. In this case report, we present 4 cases of patients with Waardenburg syndrome who have intestinal issues, 2 of whom underwent small bowel transplant. Appropriate surgical and nutritional management should be provided for patients with Waardenburg syndrome type 4 who have gastro-intestinal manifestations.

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来源期刊
CiteScore
1.40
自引率
11.10%
发文量
258
审稿时长
6-12 weeks
期刊介绍: The scope of the journal includes the following: Surgical techniques, innovations, and novelties; Immunobiology and immunosuppression; Clinical results; Complications; Infection; Malignancies; Organ donation; Organ and tissue procurement and preservation; Sociological and ethical issues; Xenotransplantation.
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