Forma no clásica de hiperplasia adrenal congénita en la ni？ez y adolescencia

introduction: the congenital adrenal hyperplasia is an inherited disorder of suprarenal esteroidogenesis, transmitted by genetic mutations with a autosomal recessive character affecting the enzymes intervening in cortisol biosynthesis. in the 90 to 95% of cases, the cause is a deficiency of hydroxylase enzyme 21. objective: to show the current paper authors' experience in the non-classic way of this entity. methods: we made a characterization of 7 female patients diagnosed in the children endocrinology ward of the national institute of endocrinology located in the children hospital, cerro municipality during 1998-2008. results: symptoms started at a mean age of 8,8 years and more the a half of cases had an early pubarche. menarche appeared at a mean age of 19,7 years. we made a biochemical diagnosis achieving higher values of 17 hydroxyprogesterone (in basal conditions). different treatment modalities were used according to each patient and predominant symptoms in each case. conclusions: we verified the great frequency of this entity in female sex, as well as the study and follow-up significance in face of a patient presenting early pubarche.