J Korean Pediatr Soc.  2001 Jan;44(1):75-82.

Factors Affecting the Presence of Menarche and Final Adult Height in Adult Female Patients with Congenital Adrenal Hyperplasia of 21-Hydroxylase Deficiency

• Lee SY
• Song CK
• Jung MH
• Hwang JS
• Yang SW

Abstract

PURPOSE: Amenorrhea and short stature are serious complications in patients with congenital adrenal hyperplasia of 21-hydroxylase deficiency(CAH). We analysed several factors influencing the presence of menarche and final adult height in these patients.
METHODS
We retrospectively reviewed medical records of 24 female patients with CAH. We analysed possible factors affecting the menarche and final adult height, such as clinical type, predicted adult height, age at treatment, age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP.
RESULTS
The occurrence rate of menarche were significantly higher in patients with the simple virilizing form rather than the salt losing form(P<0.05). No significant differences were found in age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP between menarche group and amenorrhea group. The age at treatment was younger in amenorrhea group(P<0.05). There were no significant differences in the proportion of patients who attained predicted adult height between two clinical types. No significant differences were found in age at treatment, age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP between the group of patients who attained predicted adult height and the group of patients who didn't. Final adult height did not show any correlation with age at treatment, age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP but showed significant correlation with mid-parental height(r=0.426, P=0.01).
CONCLUSION
Our data suggest that the presence of menarche in patients with CAH depends on the degree of prenatal exposure to adrenal androgen regardless of the degree of postnatal control and age at onset of puberty. Additionally, it seems likely that mid-parental height determines the final adult height.

Keyword

21-Hydroxylase deficiency; Amenorrhea; Final adult height

MeSH Terms

Adolescent
Adrenal Hyperplasia, Congenital*
Adult*
Amenorrhea
Dehydroepiandrosterone
Female
Female*
Humans
Medical Records
Menarche*
Plasma
Puberty
Retrospective Studies
Steroid 21-Hydroxylase*
Dehydroepiandrosterone
Steroid 21-Hydroxylase