先天性肾上腺皮质增生症:孟加拉国达卡 Shishu(儿童)医院的经验。

Mymensingh medical journal : MMJ Pub Date : 2024-10-01
R Biswas, A B Kamrul-Hasan, S N Rahman
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引用次数: 0

摘要

孟加拉国有关先天性肾上腺皮质增生症(CAH)的数据十分匮乏。本研究旨在收集基线信息并确定该疾病的相关具体问题。我们对 2008 年 12 月至 2020 年 12 月期间在达卡 Shishu(儿童)医院接受血清 17-羟孕酮(17-OHP)测定并被记录为患有 CAH 的儿科患者的病历进行了回顾性分析。诊断得到了生化检查结果的支持,并通过血清 17-OHP 检测和核型分析得到了证实。对相关临床数据进行了描述性分析。共纳入了六十(60)名确诊为 CAH 的患者。其中,40 例(66.7%)、15 例(25.0%)和 5 例(8.3%)患者分别患有盐耗竭型(SW)、单纯男性化型(SV)和非典型(NC)CAH。从核型上看,45(75.0%)名患者为女孩,15(25.0%)名患者为男孩。就诊时,30 例(50.0%)最初被分配为女性,24 例(40.0%)为男性,6 例(10.0%)未分配性别。所有 6 例未分配性别的病例均通过核型证实为女性,而 9 例被分配为男性的病例则被证实为女性;总体而言,15 例(25.0%)患者在初次就诊时被错误地分配了性别。SW型患者的发病年龄(中位年龄为1.0个月)早于SV型患者(中位年龄为12.0个月)。男孩的确诊时间晚于女孩。无论患儿性别如何,CAH 都应尽早确诊,以防止患儿死于失盐危机,并进行适当的性别鉴定。在孟加拉国这样一个资源匮乏的国家,我们应重视提高普通民众和护理人员的认识,以便在临床上及早发现病例并进行适当的转诊。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Congenital Adrenal Hyperplasia: Experience from Dhaka Shishu (Children) Hospital, Bangladesh.

There is a scarcity of data on congenital adrenal hyperplasia (CAH) in Bangladesh. This study aimed to collect baseline information and identify relevant issues specific to the disease. We analyzed the retrospective analysis of medical records of pediatric patients attending Dhaka Shishu (Children) Hospital, Dhaka, for serum 17-Hydroxyprogesterone (17-OHP) measurement and documented to have CAH from December 2008 to December 2020. The diagnosis was supported by biochemical findings and confirmed by serum 17-OHP assay and karyotyping. The relevant clinical data were descriptively analyzed. A total of sixty (60) patients with the diagnosis of CAH were enrolled. Among them, 40(66.7%), 15(25.0%) and 5(8.3%) patients had salt-wasting (SW), simple virilizing (SV) and non-classical (NC) CAH, respectively. Karyotypically, 45(75.0%) were girls and 15(25.0%) were boys. At presentation, 30(50.0%) were initially assigned as female and 24(40.0%) were male and in 6(10.0%) cases, the sex was not assigned. All six cases of unassigned sex were proven to be female by karyotype, while ninecases assigned as males were proven to be females; overall, 15(25.0%) patients were incorrectly assigned sex at the initial presentation. Patients with SW form of disease presented at an earlier age (median age 1.0 months) than those with SV form (median age 12.0 months). Boys were diagnosed later than girls. CAH should be diagnosed earlier, irrespective of the sex of the child, to prevent death from the salt-losing crisis and proper gender assignment. In a resource-poor country like Bangladesh, we should emphasize building awareness among the general population and caregivers for early clinical identification of the cases and proper referral.

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