Document Detail

Menstrual bleeding in a female infant with congenital adrenal hyperplasia: altered maturation of the hypothalamic-pituitary-ovarian axis.
MedLine Citation:
PMID:  9329357     Owner:  NLM     Status:  MEDLINE    
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.
N Uli; D Chin; R David; N Geneiser; K Roche; F Marino; E Shapiro; K Prasad; S Oberfield
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Journal of clinical endocrinology and metabolism     Volume:  82     ISSN:  0021-972X     ISO Abbreviation:  J. Clin. Endocrinol. Metab.     Publication Date:  1997 Oct 
Date Detail:
Created Date:  1997-11-17     Completed Date:  1997-11-17     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0375362     Medline TA:  J Clin Endocrinol Metab     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  3298-302     Citation Subset:  AIM; IM    
Department of Pediatrics, New York University Medical Center, New York 10016, USA.
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MeSH Terms
Adrenal Hyperplasia, Congenital / physiopathology*
Genitalia, Female / pathology,  radiography,  ultrasonography
Hormones / blood
Hypothalamo-Hypophyseal System / growth & development*
Magnetic Resonance Imaging
Menstruation / physiology*
Ovarian Cysts / complications,  diagnosis
Ovary / growth & development*
Pelvis / ultrasonography
Tomography, X-Ray Computed
Reg. No./Substance:

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