J Korean Soc Pediatr Endocrinol.  1998 Nov;3(2):242-249.

A Case of Ketoconazole Treatment in McCune-Albright Syndrome

Abstract

The McCune-Albright Syndrome is characterized by polyostotic fibrous dysplasia, cafe-au-lait colored patches of the skin and endocrinological abnormalities, including precocious puberty. Affected patients progress from GnRH-independent puberty to GnRH-dependent puberty. GnRH analogues are ineffective in GnRH-independent precocious puberty. Three year and 2 month old girl with breast development(SMR B3) and irregular vaginal bleeding were seen & diagnosed as incomplete sexual precocity. Decapeptyl treatment was started for the purpose of regression of breast development & vaginal bleeding with no effect. After 10 months, cafe-au-lait skin lesion & polyostotic fibrous dysplasia were noted and diagnosed as McCune-Albright syndrome. Breast development regressed to SMR B2 and vaginal bleeding was controlled with ketoconazole. As our experience, ketoconazole treatment might be effective to delay the progression of sexual development in patients with precocious puberty in McCune-Albright Syndrome.

Keyword

McCune-Albright syndrome; Precocious puberty; Ketoconazole

MeSH Terms

Adolescent
Breast
Female
Fibrous Dysplasia, Polyostotic*
Gonadotropin-Releasing Hormone
Humans
Infant
Ketoconazole*
Puberty
Puberty, Precocious
Sexual Development
Skin
Triptorelin Pamoate
Uterine Hemorrhage
Gonadotropin-Releasing Hormone
Ketoconazole
Triptorelin Pamoate
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