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Reproductive dysfunction in women with Albright's hereditary osteodystrophy
A B Namnoum1, G R Merriam, A M Moses
1Department of Gynecology and Obstetrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.
The Journal of Clinical Endocrinology and Metabolism
|March 20, 1998
Summary
Reproductive dysfunction is common in women with Albright's hereditary osteodystrophy (AHO). This is likely due to partial resistance to gonadotropins, impacting ovarian function in affected individuals.
Area of Science:
- Endocrinology
- Reproductive Medicine
- Genetics
Background:
- Albright's hereditary osteodystrophy (AHO) is characterized by G(s alpha) deficiency and hormone resistance.
- Hormonal resistance in AHO affects parathyroid hormone (PTH) and other hormones stimulating adenylyl cyclase.
Purpose of the Study:
- To investigate the incidence and causes of ovarian dysfunction in women with AHO.
- To examine the reproductive history and hypothalamic-pituitary-ovarian axis in AHO patients.
Main Methods:
- Studied 17 women (aged 17-43) with AHO, assessing reproductive history and hormonal profiles.
- Measured erythrocyte G(s alpha) activity and analyzed 24-hour luteinizing hormone (LH) secretion.
- Administered gonadotropin-releasing hormone (GnRH) to assess follicle-stimulating hormone (FSH) and LH responses.
Main Results:
- 76% of AHO patients experienced oligomenorrhea or amenorrhea.
- Most women showed delayed sexual development and mild hypoestrogenism with normal/elevated gonadotropins.
- LH pulse frequency was variable but not significantly different from controls; GnRH challenge yielded normal FSH/LH responses.
Conclusions:
- Reproductive dysfunction is a frequent clinical manifestation in women with AHO.
- The findings suggest partial gonadotropin resistance contributes to ovarian dysfunction in AHO.
- Further research into the specific mechanisms of gonadotropin resistance in AHO is warranted.