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日本持续性泄殖腔和泄殖腔外翻的现状：2014年全国性调查结果及文献综述

The current profile of persistent cloaca and cloacal exstrophy in Japan: the results of a nationwide survey in 2014 and a review of the literature.

作者信息

Kubota Masayuki

机构信息

Department of Pediatric Surgery, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-Dori, Chuo-ku, Niigata, 951-8510, Japan.

出版信息

Pediatr Surg Int. 2017 Apr;33(4):505-512. doi: 10.1007/s00383-016-4053-4. Epub 2017 Jan 5.

DOI:10.1007/s00383-016-4053-4
PMID:28058485
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5348550/
Abstract

PURPOSE

The current profile of persistent cloaca (PC) and cloacal exstrophy (CE) in Japan was first examined in 2014.

MATERIALS AND METHODS

Information was obtained by sending a questionnaire to 244 university hospitals and children's hospitals.

RESULTS

Responses from 113 institutions reported 466 PC cases and 229 CE cases. The incidences of PC and CE from 1980 to 2012 were 0.97 and 0.49 per 100,000 live births, respectively. In the previous 5 years, antenatal abnormalities were found in 57.6% of PC and 72.7% of CE patients. Myelomeningocele was observed in 45.6% of CE patients. As a result of various surgical treatments used in the neonatal and infantile periods, the respective rates of bladder dysfunction, clean intermittent catheterization, and permanent enterostomy were 32.6, 22.5, and 7.3% in PC patients and 60.7, 28.4, and 73.8% in CE patients. Menstrual outflow obstruction was found in 22.5% of PC and 48.9% of CE patients with menstruation.

CONCLUSION

The clinical outcomes of PC and CE remain unsatisfactory. Therefore, the establishment of treatment guidelines might be a useful objective for improving the current status of PC and CE.

摘要

目的

2014年首次对日本持续性泄殖腔（PC）和泄殖腔外翻（CE）的当前概况进行了研究。

材料与方法

通过向244家大学医院和儿童医院发送调查问卷获取信息。

结果

113家机构的回复报告了466例PC病例和229例CE病例。1980年至2012年期间，PC和CE的发病率分别为每10万例活产0.97例和0.49例。在过去5年中，57.6%的PC患者和72.7%的CE患者在产前被发现存在异常。45.6%的CE患者观察到脊髓脊膜膨出。由于在新生儿期和婴儿期采用了各种手术治疗，PC患者膀胱功能障碍、间歇性清洁导尿和永久性肠造口术的发生率分别为32.6%、22.5%和7.3%，CE患者分别为60.7%、28.4%和73.8%。在有月经的PC患者和CE患者中，分别有22.5%和48.9%发现月经流出受阻。

结论

PC和CE的临床结果仍不尽人意。因此，制定治疗指南可能是改善PC和CE现状的一个有益目标。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7de4/5348550/c02421ba6450/383_2016_4053_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7de4/5348550/b1b840d0eb84/383_2016_4053_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7de4/5348550/e19b24893a2f/383_2016_4053_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7de4/5348550/b94115dd1f81/383_2016_4053_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7de4/5348550/c02421ba6450/383_2016_4053_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7de4/5348550/b1b840d0eb84/383_2016_4053_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7de4/5348550/e19b24893a2f/383_2016_4053_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7de4/5348550/b94115dd1f81/383_2016_4053_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7de4/5348550/c02421ba6450/383_2016_4053_Fig4_HTML.jpg

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