Growth and pubertal characteristics in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
This retrospective study examined the pubertal characteristics and growth in patients with 21-hydroxylase deficiency (21-OHD). There were 18 males and 31 females with salt wasting (SW), simple virilizing (SV), or non-classical (NC) 21-OHD. Mean ages at onset of puberty (AOP) in SW, SV, and NC males were 9.2 +/- 1.9, 10.3 +/- 1.1, and 10.7 +/- 0.8 years, respectively; while mean AOP in SW, SV, and NC females were 9.3 +/- 1.7, 8.6 +/- 1.6, and 8.5 +/- 1.3 years, respectively. Mean final height (FH) in SW males (159.6 +/- 7.8 cm) was less than in SV (166.8 +/- 7.5 cm, p = 0.06) and NC (173.4 +/- 6.4 cm, p = 0.005) males. Mean FH in SW females (157.1 +/- 5.5 cm) was similar to SV (156.0 +/- 8.4 cm) but less than NC (161.3 +/- 5.4 cm, p = 0.01) females. In conclusion, while the patients as a group entered puberty earlier than the general population, SW males entered puberty the earliest and had the most compromised FH outcome.
Trinh
Lien
L
Adrenal Steroid Disorders Program, Division of Pediatric Endocrinology, The Mount Sinai School of Medicine, New York, NY, USA.
Nimkarn
Saroj
S
New
Maria I
MI
Lin-Su
Karen
K
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000503:physiopathology*; D001827:Body Height / Q000502:physiology*; D002648:Child; D002657:Child Development / Q000502:physiology*; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D011627:Puberty / Q000502:physiology*; D012189:Retrospective Studies; D012737:Sex Factors; D012741:Sexual Maturation / Q000502:physiology*; D013255:Steroid 21-Hydroxylase / Q000378:metabolism
D013255:Steroid 21-Hydroxylase