Advance in the diagnosis and treatment of McCune-Albright syndrome in children / 国际儿科学杂志

ABSTRACT

McCune-Albright syndrome is a clinical syndrome characterized by the triad of polyostotic fibrous dysplasia, precocious puberty, and café-au-lait skin macules.McCune-Albright syndrome is a rare guanine nucleotide-binding protein disease(G-protein disease), and was firstly described by Donovan McCune and Fuller Albright in 1936 and 1937.With the deepening of the understanding of this disease, more and more children have been clearly diagnosed.Early diagnosis and appropriate treatment are especially important in improving the final adult height and the quality of life.The application of bisphosphonates in the treatment of polyostotic fibrous dysplasia is currently advocated, but the long-term safety and effectiveness are not clear.There are some therapeutic strategies for precocious puberty, but the optimal one is not clear.This review elaborates on the pathogenesis, clinical manifestations, diagnosis and treatment of McCune-Albright syndrome, especially the treatment of polyostotic fibrous dysplasia and precocious puberty.