Adrenogenital syndrome in children

Objective To discuss the clinical features and therapeutic effects of adrenogenital syndrome in children. Methods Clinical and follow up data were summarized for 42 children with adrenogenital syndrome, including 30 cases of congenital adrenal hyperplasia (CAH) and 12 cases of adrenal cortex tumor. Results Endocrine function examination in 15 cases of CAH showed that 24h urine 17 KS was elevated in 10 cases and normal in 5.There was obvious clitorism in all 30 CAH children and urogenital sinus malformation in 28.Of the 12 cases of adrenal cortex tumor 7 had sexual abnormality and hypercortisolism simultaneously.Clitoris shortening or clitoridectomy was performed in CAH cases plus vaginal vestibule plasty simultaneously.Of the 12 cases of adrenal cortex tumor 10 underwent complete tumor resection;1 partial tumor resection and the remaining 1 declined treatment.During the follow up 8 case of cortex tumor,4 cases of adenoma survived tumor free with an average survival time of 7.5 years;2 cases of cortex carcinoma survived tumor free for 3 and 4 years;while the other 2 cases died soon after discharge. Conclusions The diagnosis can be established based on clitorism,labium confluence or male sexual precocity,increase in urine 24h 17 KS and blood testrone.Cortex hormone should be taken early,properly,life long in CAH patients,and appropriate perineoplasty should be performed.Early detection and resection of tumor is the key point for treating adrenogenital syndrome caused by cortex tumor.