Report of 285 patients with congenital adrenal hyperplasia and evaluation of approximate prevalence of the disease in Iran

Acta Medica Iranica Journal

Volume 37 - Number 2

Article Type: ---- Unspecified ----

In this study, 285 cases of congenital adrenal hyperplasia who were followed in the Tehran university hospitals and institute of endocrinology and metabolism are reported. Among these cases, 165 (57.9%) were female and 120 (42.1%), male. The most common type of congenital adrenal hyperplasia in these patients was the salt-losing type of 21-hydroxylase deficiency (57.9%); 11-hydroxylase deficiency was present in 13.68% of patients. There were only 3 case with 3-beta hydroxysteroid dehydrogenase deficiency, 2 cases with 17-alphahydroxylase deficiency and one with 20, 22-desmolase deficiency. Presenting complaints were in decreasing order of frequency: Ambiguous genitalia, vomiting and dehydration, precocious puberty, hypertension, failure to thrive, hirsutism and primary amenorrhea. The age of patients at the time of diagnosis was between 2 days to 17 years and the most common age was in the first two years of life especially in the neonatal period. A positive family history of the same disease was present in 17 siblings of our patients. (21-OHD=14 H-OHD=3). There were 27 cases of death among these patients (23 male and 4 female that 24 cases had 21-OHD and 2 cases had 3 beta HSD deficiency and one case had 20, 22-desmolase deficiency).

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