Experience with 115 patients with congenital adrenal hyperplasma and evaluation of growth patterns in 24 patients with the salt-losing type

ABSTRACT

In this study the data on 115 cases of congenital adrenal hyperplasia [CAH] who were followed in the Pediatric Endocrine Clinic at Nemazee Hospital, Shiraz will be reported Among these cases 51 were male and 64 female. The most common type of CAH in these patients was the salt-losing type of 21-hydroxylase deficiency [85.2%]. 11-hydroxylase deficiency was present 13.04% of patients. There was only one case with 20,22-desmolase deficiency and one also with 3-beta-hydroxysteroid dehydrogenase deficiency. Presenting complaints were ambiguous genitalia, vomiting, failure to thrive, precocious puberty and hypertension. The analysis of data on 24patients with the salt-losing type of 21-hydroxylase deficiency who were followed for at least 2 years showed that these patients suffered from abnormal growth patterns. Growth failure was maximal during the first year of life