AB074. Registry of congenital adrenal hyperplasia at the north pediatric referral centre of Vietnam during 15 years

Abstract: The National Hospital of Pediatrics (NHP), Hanoi, Vietnam is a 1,200 bed tertiary referral centre servicing approximately 30 million people from northern provinces of Vietnam. This audit was undertaken to analyze anecdotal reports of increasing patient numbers. Retrospective review of all congenital adrenal hyperplasia (CAH) patients registered at NHP from 1999-2014. Ethical clearance was granted by the NHP Directorate. At the start of 1999 there were 90 children with CAH managed at NHP. By May 2014 this increased to 715 including 375 (52%) male patients and 340 (48%) female patients. Number of cases with 21α-hydroxylase deficiency (21-OHD), 11β-hydroxylase deficiency and 3β-hydroxysteroid dehydrogenase deficiency was 703 (98.3%); 9 (1.3%) and 3 (0.4%), respectively. Among cases with 21-OHD, 72% were salt wasting and 28% were simple virilisation). Total number of cases represents a more than seven folds increase over 14 years. Number of new cases doubled from 30 to 67 in 2013. Most children (85%) were diagnosed at less than 12 months of age (55% at less than 1 month of age); 70% of all children were younger than 10 years. Formal mortality figures were low (seven known deaths). The caseload of CAH at NHP has increased since 1999 and additional capacity is needed for patient care. Introduction of NBS would enable more accurate estimation of CAH incidence, reduce infant mortality and minimize trauma to affected infants and their families.

Keywords: Congenital adrenal hyperplasia (CAH); pediatrics; Vietnam