Chinese Journal of Contemporary Pediatrics 2006;8(4):311-314

McCune-Albright syndrome: a difficult and complicated case study.

Li-Yang LIANG 1 ; Zhe MENG ; Qiao-Hui ZENG ; Wen-Yi LI

Affiliations

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Country

China

Language

Chinese

Abstract

McCune-Albright syndrome is a rare G proteins alpha disorder. The disorder is characterized by polyostotic fibrous dysplasia, sexual precocity and hyperpigmented macules. It is caused due to mutations in the gene Gsalpha that incodes the alpha subunit of the trimeric guanosine triphate-binding protein. There is no specific treatment for this syndrome. Treatment is generally symptomatic. This paper reported three cases of McCune-Albright syndrome and reviewed the relevant literatures regarding to the pathogenesis, pathological features, diagnosis and treatment. All three cases presented with a characteristic triad: polyostotic fibrous dysplasia, sexual precocity and hyperpigmented macules and were thus definitely diagnosed with McCune-Albright syndrome.