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先天性巨结肠症患者接受根治性手术后的预后。

Outcomes in patients with Hirschsprung disease following definitive surgery.

作者信息

Karina Stefani Melisa, Dwihantoro Andi

机构信息

Pediatric Surgery Division, Department of Surgery, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr, Sardjito Hospital, Jl. Kesehatan No. 1, Yogyakarta, 55281, Indonesia.

出版信息

BMC Res Notes. 2018 Sep 4;11(1):644. doi: 10.1186/s13104-018-3751-5.

DOI:10.1186/s13104-018-3751-5
PMID:30180876
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6123951/
Abstract

OBJECTIVE

Several pull-through procedures have been described for Hirschsprung disease (HSCR) with varying outcomes. We aimed to describe the outcomes in HSCR patients < 18 year of age who underwent surgical procedures at Dr. Sardjito Hospital, Yogyakarta, Indonesia from January 2013 to December 2014.

RESULTS

We utilized 67 HSCR patients, of whom 49 (73%) were males and 18 (27%) females. Neonatal presentation was seen in 57 cases (85%) and most patients (98.5%) had short-segment HSCR. The clinical manifestations were mainly abdominal distension (94%) and delayed passage of meconium (45%). The most common definitive treatment performed was transanal endorectal pull-through (TEPT) (54%), followed by Soave (18%) and Duhamel (13%) procedures. Enterocolitis occurred in 13% of the HSCR patients after endorectal pull-through, but did not reach a significant level (p-value = 0.65), while the constipation rate was significantly higher in HSCR patients who underwent posterior neurectomy compared with those other procedures (OR = 15.5, 95% CI = 1.8-132.5; p-value = 0.019). In conclusions, most HSCR patients in Indonesia were diagnosed in the neonatal period and underwent the TEPT procedure. Furthermore, the risk of constipation is increased in HSCR patients following posterior neurectomy compared with other definitive surgical techniques.

摘要

目的

针对先天性巨结肠(HSCR)已描述了多种拖出术式,其结果各异。我们旨在描述2013年1月至2014年12月期间在印度尼西亚日惹市萨迪托博士医院接受手术治疗的18岁以下HSCR患者的治疗结果。

结果

我们纳入了67例HSCR患者,其中49例(73%)为男性,18例(27%)为女性。57例(85%)患者为新生儿期发病,大多数患者(98.5%)为短段型HSCR。临床表现主要为腹胀(94%)和胎粪排出延迟(45%)。最常用的确定性治疗方法是经肛门直肠拖出术(TEPT)(54%),其次是Soave术(18%)和Duhamel术(13%)。直肠拖出术后13%的HSCR患者发生小肠结肠炎,但未达到显著水平(p值 = 0.65),而行后根神经切断术的HSCR患者便秘发生率显著高于其他手术方式(OR = 15.5,95%CI = 1.8 - 132.5;p值 = 0.019)。结论是,印度尼西亚的大多数HSCR患者在新生儿期被诊断出来并接受了TEPT手术。此外,与其他确定性手术技术相比,行后根神经切断术的HSCR患者便秘风险增加。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/441d/6123951/6bf2eb40ba9a/13104_2018_3751_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/441d/6123951/6bf2eb40ba9a/13104_2018_3751_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/441d/6123951/6bf2eb40ba9a/13104_2018_3751_Fig1_HTML.jpg

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Effects of RET, NRG1 and NRG3 Polymorphisms in a Chinese Population with Hirschsprung Disease.
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