Menstrual Bleeding in a Female Infant with Congenital Adrenal Hyperplasia: Altered Maturation of the Hypothalamic-Pituitary-Ovarian Axis

Vaginal bleeding during the neonatal period is commonly related to the withdrawal of maternal estrogens. Vaginal bleeding has also been reported in female infants with congenital adrenal hyperplasia and has been proposed to be due to a treatment-induced activation of the hypothalamic-pituitary-ovari...

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Bibliographic Details
Published in:The journal of clinical endocrinology and metabolism 1997-10, Vol.82 (10), p.3298-3302
Main Authors: Uli, Naveen, Chin, Daisy, David, Raphael, Geneiser, Nancy, Roche, Kevin, Marino, Flavia, Shapiro, Ellen, Prasad, Kris, Oberfield, Sharon
Format: Article
Language:English
Subjects:
Adrenal Hyperplasia, Congenital - physiopathology
Adrenals. Adrenal axis. Renin-angiotensin system (diseases)
Biological and medical sciences
Endocrinopathies
Female
Genitalia, Female - diagnostic imaging
Genitalia, Female - pathology
Hormones - blood
Humans
Hypothalamo-Hypophyseal System - growth & development
Infant
Magnetic Resonance Imaging
Medical sciences
Menstruation - physiology
Non tumoral diseases. Target tissue resistance. Benign neoplasms
Ovarian Cysts - complications
Ovarian Cysts - diagnosis
Ovary - growth & development
Pelvis - diagnostic imaging
Tomography, X-Ray Computed
Ultrasonography
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Summary:Vaginal bleeding during the neonatal period is commonly related to the withdrawal of maternal estrogens. Vaginal bleeding has also been reported in female infants with congenital adrenal hyperplasia and has been proposed to be due to a treatment-induced activation of the hypothalamic-pituitary-ovarian axis. We report a female infant with the salt-losing form of congenital adrenal hyperplasia due to 21-hydroxylase deficiency, who had the onset of vaginal bleeding at 3 months of life. Adrenal steroid suppression had been achieved by 2.5 weeks of age. At the time of bleeding, imaging studies revealed an enlarged right ovary with a dominant 3-cm cyst and additional small cysts that had not been seen on the newborn sonogram. The uterus was enlarged and stimulated. Three weeks later (1 week after the cessation of bleeding), repeat ultrasound demonstrated a marked decrease in the size of the right ovary, and the dominant cyst was no longer seen. The patient had a heightened FSH response to GnRH and elevated levels of estradiol for age. At 5 months of age, no further episodes of sustained vaginal bleeding were observed. Repeat hormonal levels were prepubertal, and pelvic sonogram demonstrated no evidence of stimulation. The findings in our patient suggest that a decline in adrenal androgens after glucocorticoid treatment resulted in an increase in gonadotropin levels, which then triggered a transient and augmented end-organ response (menses). Further, we suggest that our infant’s hormonal findings may reflect a delay in the timely development of the negative restraint by sex steroids on gonadotropins that is normally observed in infancy.
ISSN:0021-972X
1945-7197
DOI:10.1210/jcem.82.10.4277