Final Adult Height and Its Influencing Factors in Patients with Congenital Adrenal Hyperplasia Caused by 21-Hydroxylase Deficiency / 대한소아내분비학회지

ABSTRACT

PURPOSE: We evaluated adult final height and factors influencing adult height outcome in patients with congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency. METHOD: We retrospectively reviewed medical records of 20 male and 22 female patients with 21- hydroxylase deficiency who reached adult final height from 1980 to 2004. We obtained final adult height and analysed possible factors influencing final adult height such as urine 17-ketosteroid (17- KS), plasma 17-OH-progesterone (17-OHP), plasma renin activity, pubertal initiaton age, glucocorticoid dose, age at diagnosis and effect of growth hormone or gonadotropin releasing hormone analogue. RESULT: Mean FH (final height) SDS-MPH (midparental height) SDS were -1.89+/-1.0 in male simple virilization (SV) and -0.83+/-0.8 in female SV patients, -2.27+/-1.3 in male salt wasting (SW) and -1.12+/-1.1 in female SW patients. Mean final adult height and mean FH SDS-MPH SDS were not different between SV and SW patients, but mean FH SDS-MPH SDS in males was significantly lower than that of females (-2.12+/-1.2 vs 0.95+/-0.9, P<0.05). In SW mean FH SDS-MPH SDS were correlated positively with pubertal height gain (r=0.484. P<0.05) and correlated negatively with pubertal initiation age (r=-0.334, P<0.05). but mean FH SDS-MPH SDS were not correlated with age at diagnosis, glucocorticoid dose, urine 17-KS level, plasma 17-OHP level, plasma renin activity, treatment with growth hormone or gonadotropin releasing hormone analogue in both SW and SV. CONCLUSION: Mean final height and mean FH SDS-MPH SDS in 21-hydroxylase deficiency were below the mean for the general population. In SW, adult final height was correlated with pubertal initiation age and pubertal height gain. Therefore it should be important to control early onset of puberty and make improvement pubertal height gain during puberty.