J Korean Soc Pediatr Endocrinol.  2005 Jun;10(1):42-49.

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

Affiliations
  • 1Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea. growth@snu.ac.kr
  • 2Department of Pediatrics, Bundang Jesaeng General Hospital, Seognam, Korea.
  • 3Department of Pediatrics, Kwang Myung Sung Ae General Hospital, Seoul, Korea.

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.

Keyword

Congenital adrenal hyperplasia; Final adult height

MeSH Terms

Adolescent
Adrenal Hyperplasia, Congenital*
Adult*
Diagnosis
Female
Gonadotropin-Releasing Hormone
Growth Hormone
Humans
Male
Medical Records
Plasma
Puberty
Renin
Retrospective Studies
Steroid 21-Hydroxylase*
Virilism
Gonadotropin-Releasing Hormone
Growth Hormone
Renin
Steroid 21-Hydroxylase
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