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A six-year-old boy with multiple bone lesions, repeated fractures, and sexual precocity

Authors :
Majzoub, Joseph A.
Scully, Robert E.
Source :
The New England Journal of Medicine. Feb 18, 1993, Vol. v328 Issue n7, p496, 7 p.
Publication Year :
1993

Abstract

A six-year-old boy with sexual precocity, multiple skeletal deformities and light brown spots was diagnosed with McCune-Albright syndrome. Sexual precocity is the onset of sexual maturity before age nine and can be caused by a gonadotropin hormone dependent or independent mechanism. McCune-Albright syndrome is a form of gonadotropin-independent sexual precocity associated with multi-system defects. The boy had a 10-month history of facial acne, apocrine sweat, pubic hair growth, genital enlargement, mood swings and accelerated growth. He had suffered three fractures to his right leg following minor accidents at ages three, four and five. He had a testosterone level and genital size typical of mid-puberty and bone age typical of a nine-year-old. X-rays revealed polyostotic fibrous dysplasia, which is a bone disease. The boy's lack of gonadotropin pulsations indicated his sexual precocity was gonadotropin independent. McCune-Albright syndrome's association polyostotic fibrous dysplasia and cafe-au-lait spots explains his other defects.

Details

ISSN :
00284793
Volume :
v328
Issue :
n7
Database :
Gale General OneFile
Journal :
The New England Journal of Medicine
Publication Type :
Academic Journal
Accession number :
edsgcl.13669103