Abstract：
Background: 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. Methods: 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. Results: 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. Conclusions: 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 of Evidence: Level IV (Epidemiological).
摘要：
背景：在资源受到挑战的国家，由于缺乏全国范围的监测系统,先天性手差异(CHD)的真实程度仍然未知.我们创建了一个基于医院的注册系统，旨在确定冠心病的患病率和分布，并比较Swanson/IFSSH（SI）和Oberg，Manske和Tonkin(OMT)分类。方法：从2018年1月至2021年12月，将0至18岁冠心病儿童的数据输入在线注册表，并根据SI和OMT分类进行分类。使用描述性分析总结了冠心病的患病率和分布以及家族史等危险因素。使用Fischer精确检验和比值比进一步分析了综合征和遗传关联。使用聚类列来比较两个分类系统。结果：研究期间共有307例冠心病患者，患病率为2.4/1,000。其中,164是单方面的，21人是近亲结婚（7.6％），10人（3.6％）有冠心病家族史。虽然没有统计学意义，与单方面参与相比,双边参与的遗传可能性是其两倍.在27例患者中，总共发现了10种不同的综合征，其中最常见的是Holt-Oram综合征。最常见的CHD是径向纵向缺乏（111）。共有82名（27％）儿童患有其他相关异常，无法归类为综合征。无法使用SI或OMT分类对两名患者进行分类。结论：在线注册建立了一种有效的方法来存储和分析与CHD相关的数据。它提供了有关其在印度南部流行的新信息，这与现有文献相似。大多数CHD可以分为SI和OMT分类。然而,仍然存在一些无法分类的条件。需要建立CDH的国家登记册，以进行有效管理，资金和研究。证据级别：IV级（流行病学）。
