A Report Of McCune-Albright Syndrom (Two Cases) And Review

Objective:To investigate the pathogenesis, clinical manifestation, investigations and management of McCune-Albright syndrome.Methods:Collect two cases of McCune-Albright syndrome, analyze the pathogenesis, clinical manifestation, investigations and management, review the literatures about this disease.Results:McCune-Albright syndrome is characterized by polyostotic fibrous dysplasia; hyperpigmented macules; one or more endocrine hyperplasia or auto-overactivity by adenoma.The investigations contain evaluation of hormone, gene diagnosis, radiological examination of bone, radioisotope scanning, ultrasonic examination.Clnclusion:McCune-Albright syndrome is a kind of polyostotic fibrous dysplasia, one uncommon disease caused by congenital mutations in the cAMP regulating protein, Gsa. Widespread skeletal involvement is often associated with varying combinations of cafe'-au-lait skin spots, and/or endocrine dysfunction (precocious puberty, renal phosphate wasting, hyperthyroidism, and/or growth hormone excess). The investigations contain evaluation of hormone, gene diagnosis, radiological examination of bone, radioisotope scanning, ultrasonic examination.To delay progression of disease, elevate life quality, ease psychological burden on treatment; To understand McCune-Albright syndrome deeply and decrease misdiagnosis rate by learning the case and reviewing the literatures.