CONGENITAL ADRENAL-HYPERPLASIA COMPLICATED BY CENTRAL PRECOCIOUS PUBERTY - LINEAR GROWTH DURING INFANCY AND TREATMENT WITH GONADOTROPIN-RELEASING-HORMONE ANALOG

Some children with congenital adrenal hyperplasia (CAH) develop true p recocious puberty with early maturation of the hypothalamic-pituitary- gonadal axis. We have seen six such children who had the diagnosis of CAH with late initiation of corticosteroid treatment and/or poor compl iance who developed central precocious puberty (CPP), These patients w ere treated with standard-dose hydrocortisone and fludrocortisone. Adm inistration of depot leuprorelin (3.75 mg subcutaneously every 28 days ) for 2 years or longer was effective in arresting the manifestations of puberty, decelerating the pretreatment growth velocity ([GV] 10.8 /- 1.5 v3.65 +/- 0.95 cm/yr), increasing the predicted adult height ([ PAHT] 147.5 +/- 7.8 v153.4 +/- 8.3 cm), and decreasing the bone age to statural age ratio (1.26 +/- 0.13 v1.16 +/- 0.09). Analysis of auxano logical data during the first 2 years of life showed that linear growt h was significantly accelerated and bone age was advanced in patients who developed CPP compared with 11 age-matched patients, It appears th at proper glucocorticoid replacement to achieve adequate control of hy perandrogenemia during early life might prevent development of CPP in these patients, Gonadotropin-releasing hormone agonist (GnRHa) therapy can improve the final adult height, bringing it closer to that expect ed from the genetic potential. Copyright (C) 1997 by W.B. Saunders Com pany.