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印度南部一家三级医院先天性手差异的流行病学研究-使用 Swanson/IFSSH 和 Oberg、Manske 和 Tonkin 分类法建立新的登记处和评估方法。

Epidemiology of Congenital Hand Differences at a Tertiary Hospital in Southern India - Establishment of a New Registry and Assessment Using Both the Swanson/IFSSH and the Oberg, Manske and Tonkin Classifications.

机构信息

Unit of Hand and Microsurgery, Department of Orthopaedics, Kasturba Medical College & Hospital, Manipal, Manipal Academy of Higher Education, Manipal, India.

出版信息

J Hand Surg Asian Pac Vol. 2022 Oct;27(5):801-809. doi: 10.1142/S2424835522500825.

DOI:10.1142/S2424835522500825
PMID:36285755
Abstract

In resource challenged nations, the true magnitude of the congenital hand differences (CHD) remain unknown due to a lack of nation-wide surveillance system. We created a hospital-based registry system with the aim to determine the prevalence and distribution of CHD and compare the Swanson/IFSSH (SI) and Oberg, Manske and Tonkin (OMT) classifications. Data of children aged 0 to 18 years with CHD was entered into the online registry and classified based on the SI and OMT classifications from January 2018 to December 2021. The prevalence and distribution of CHD and risk factors like family history were summarised using descriptive analysis. Syndromic and heredity associations were further analysed using Fischer exact test and odds ratio. Clustered columns were used to compare the two classification systems. A total of 307 patients with CHD presented during the study with a prevalence of 2.4/1,000 patients. Among them, 164 were unilateral, 21 were born of consanguineous marriages (7.6%) and 10 (3.6%) had a family history of CHD. Although not statistically significant, bilateral involvement was twice as likely to be hereditary compared to unilateral involvement. A total of 10 different syndromes were identified among 27 patients of which Holt-Oram syndrome was most common. The most common CHD was Radial longitudinal defificiency (111). A total of 82 (27%) children had other associated anomalies that could not be grouped as a syndrome. Two patients could not be classified using either SI or OMT classifications. The online registry established an efficient way to store and analyse data related to CHD. It provides new information on its prevalence in South India, which is similar to the existing literature. Most of the CHD can be grouped in both the SI and OMT classifications. However, there still remains some conditions that are unclassifiable. There is a need for a national registry of CDH for effective management, funding and research. Level IV (Epidemiological).

摘要

在资源有限的国家,由于缺乏全国性的监测系统,先天性手部差异 (CHD) 的真实规模仍不清楚。我们创建了一个基于医院的登记系统,旨在确定 CHD 的患病率和分布,并比较 Swanson/IFSSH (SI) 和 Oberg、Manske 和 Tonkin (OMT) 分类。2018 年 1 月至 2021 年 12 月,将年龄在 0 至 18 岁之间患有 CHD 的儿童的数据输入在线登记系统,并根据 SI 和 OMT 分类进行分类。使用描述性分析总结 CHD 的患病率和分布以及家族史等危险因素。使用 Fisher 精确检验和优势比进一步分析综合征和遗传关联。使用聚类列比较两种分类系统。在研究期间,共有 307 名患有 CHD 的患者就诊,患病率为每 1000 名患者中有 2.4 名。其中,164 例为单侧,21 例为近亲结婚(7.6%),10 例(3.6%)有 CHD 家族史。尽管没有统计学意义,但双侧受累比单侧受累更有可能是遗传性的。在 27 名患者中发现了 10 种不同的综合征,其中 Holt-Oram 综合征最常见。最常见的 CHD 是桡侧纵向缺乏(111)。共有 82 名(27%)儿童有其他无法归类为综合征的相关异常。有两名患者无法使用 SI 或 OMT 分类进行分类。在线登记系统建立了一种有效存储和分析与 CHD 相关数据的方法。它提供了关于其在印度南部流行率的新信息,与现有文献相似。大多数 CHD 可以归入 SI 和 OMT 分类。然而,仍然存在一些无法分类的情况。需要建立一个全国性的 CDH 登记系统,以进行有效的管理、资金和研究。四级(流行病学)。

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