Monitoring of menstrual cycles, ovulation, and adrenal suppression by saliva sampling in female patients with 21-hydroxylase deficiency.
OBJECTIVE
To investigate the correlation between menstrual cycles, ovulation, and adrenal suppression in congenital adrenal hyperplasia.


METHODS
Prospective observational study.


METHODS
An academic outpatient clinic.


METHODS
Five females with salt-wasting 21-hydroxylase deficiency, aged 15.5 to 22.9 years; one had amenorrhea, one had irregular bleeding, and three had regular bleeding.


METHODS
Daily morning saliva sampling for 40 to 280 days.


METHODS
Salivary levels of progesterone (P), 17-hydroxyprogesterone (17-OHP), and androstenedione.


RESULTS
In the amenorrheic patient, the elevated P and 17-OHP levels decreased when the glucocorticoid dose was increased, and subsequently menarche occurred. The androstenedione levels were normal. The correlations between P and 17-OHP levels before and after menarche suggest that adrenal progesterone had prevented menarche. The patient with irregular bleeding showed slightly elevated androstenedione levels and increased levels of 17-OHP and P in an irregular pattern, without correlation in time with vaginal bleeding. Three patients with regular cycles showed a biphasic pattern of P levels, indicating ovulation.


CONCLUSIONS
These longitudinal data support the hypothesis that menstrual cycling in females with 21-hydroxylase deficiency can be prevented or disturbed by elevated progesterone levels of adrenal origin, in the absence of androgen excess. Increasing glucocorticoid dose could suppress adrenal progesterone production, resulting in menarche.
Stikkelbroeck
Nike M M L
NM
Department of Pediatric Endocrinology, University Medical Center Nijmegen, Nijmegen, The Netherlands.
Sweep
C G J
CG
Braat
Didi D M
DD
Hermus
Ad R M M
AR
Otten
Barto J
BJ
D019326:17-alpha-Hydroxyprogesterone / Q000032:analysis; D000293:Adolescent; D000311:Adrenal Glands / Q000503:physiopathology*; D000312:Adrenal Hyperplasia, Congenital / Q000503:physiopathology*; D000735:Androstenedione / Q000032:analysis; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008597:Menstrual Cycle; D010060:Ovulation; D011159:Population Surveillance / Q000379:methods; D011446:Prospective Studies; D012463:Saliva / Q000737:chemistry*; D013255:Steroid 21-Hydroxylase / Q000032:analysis
D000735:Androstenedione; D019326:17-alpha-Hydroxyprogesterone; D013255:Steroid 21-Hydroxylase