Congenital Adrenal Hyperplasia: Experience from Dhaka Shishu (Children) Hospital, Bangladesh.

Abstract

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.