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先天性巨结肠症(HSCR)的流行病学特征:来自孟加拉国的50例病例系列研究结果

Epidemiological characteristics of Hirschsprung's disease (HSCR): Results of a case series of fifty patients from Bangladesh.

作者信息

Karim Anwarul, Akter Mastura, Aziz Tasmiah T, Hoque Mozammel, Chowdhury Tanvir K, Imam Md Sharif, Walid Adnan, Kabir Mahfuzul, So Manting, Lam Wai Yee, Tang Clara Sm, Wong Kenneth K, Tam Paul K, Garcia-Barcelo Merce, Banu Tahmina

机构信息

Chittagong Research Institute for Children Surgery (CRICS), Chittagong, Bangladesh; Department of Surgery, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong.

Chittagong Research Institute for Children Surgery (CRICS), Chittagong, Bangladesh; Department of Pediatric Surgery, Chittagong Medical College and Hospital (CMCH), Bangladesh.

出版信息

J Pediatr Surg. 2018 Oct;53(10):1955-1959. doi: 10.1016/j.jpedsurg.2017.12.029. Epub 2018 Jan 31.

DOI:10.1016/j.jpedsurg.2017.12.029
PMID:29429767
Abstract

BACKGROUND

The epidemiology of Hirschsprung's disease (HSCR) in Bangladesh has never been studied. The aim of this study was to determine the epidemiological characteristics of HSCR in Bangladesh.

METHODS

Data from fifty patients were collected prospectively from two hospitals in Chittagong, Bangladesh.

RESULTS

The rate of consanguinity (16%) among parents of HSCR patients was higher than that of the general population (10%). Maternal age at the time of birth of the affected child was ≤30years in all cases except one. No association was found between parents' occupation and HSCR. No patient was born preterm and only three patients (6%) had low birth weight. Nine patients (18%) had associated anomalies. We found coexistence of bilateral accessory tragi and ankyloglossia in one patient, and coexistence of rectal duplication cyst in another. Neither anomaly had been previously reported in HSCR patients.

CONCLUSIONS

Our study suggests that consanguinity might increase the risk of HSCR whereas advanced maternal age does not. HSCR patients were found more likely to born at term and with normal birth weight. The coexistence of HSCR with previously unreported anomalies highlights the diversity of conditions that can co-occur with HSCR.

LEVELS OF EVIDENCE

IV.

摘要

背景

孟加拉国先天性巨结肠症(HSCR)的流行病学从未被研究过。本研究的目的是确定孟加拉国HSCR的流行病学特征。

方法

前瞻性收集了来自孟加拉国吉大港两家医院的50例患者的数据。

结果

HSCR患者父母的近亲结婚率(16%)高于普通人群(10%)。除1例病例外,所有患病儿童出生时母亲年龄均≤30岁。未发现父母职业与HSCR之间存在关联。没有患者早产,只有3例患者(6%)出生体重低。9例患者(18%)伴有其他异常。我们发现1例患者同时存在双侧副耳和舌系带过短,另1例患者同时存在直肠重复囊肿。此前在HSCR患者中均未报告过这两种异常。

结论

我们的研究表明,近亲结婚可能会增加HSCR的风险,而母亲年龄较大则不会。HSCR患者足月出生且出生体重正常的可能性更大。HSCR与此前未报告过的异常同时存在,凸显了可与HSCR同时出现的病症的多样性。

证据级别

IV级。

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