This review concentrates on the treatment for some of the more common causes of hypergonadotropic hypogonadism. Females with hypergonadotropic hypogonadism have constituted much of this data because those with chromosomally competent ovarian failure (CCOF) and chromosomally incompetent ovarian failure (CIOF) have the potential to conceive, and women with CIOF have been treated hormonally in attempts to increase growth. Males with chromosomally incompetent gonad failure (CIGF), most commonly 47,XXY, are not short, so this is not an issue. Pure 47,XXY males rarely are able to impregnate women unless they are mosaics. More data is needed in CIOF women before the final consensus of recombinant growth hormone (rGH) and oxandrolone can be reached. It is important to note that treatment with rGH has been restricted to study protocols, and only the treatment of growth hormone deficiency is FDA approved. Currently it appears prudent to discuss treatment options with patients having hypergonadotropic hypogonadism with respect to sexual development, reproductive potential, growth, and the prevention of complications such as osteoporosis and heart disease. It is the opinion of this author that if patients do desire to use rGH and oxandrolone, that they be treated by physicians directly involved in research protocols or after discussion with investigators who have experience in these treatment protocols. The induction of secondary sexual characteristics may be started after the completion of treatment for growth, which may be up to 3-5 years. If the woman does not desire therapy for growth, it appears reasonable to begin hormone replacement at ages 9-11, or at the time of diagnosis, if the individual is older. Likewise, males may begin testosterone treatment beginning at about ages 10-12. Support, discussion about potential complications of the disease and the replacement medication, and psychologic considerations must be considered for the complete management in individuals with hypogonadism.
- Hypergonadotropic hypogonadism
- Ovarian failure
- Sexual development
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Obstetrics and Gynecology