Dysplasia of bone fibers-McCune-Albright syndrome: A clinical analysis of 7 children cases

Objective To study the diagnosis and treatment of McCune-Albright syndrome（MAS）. Methods Clinical manifestations, laboratory and imaging data of 7 children with McCune-Albright syndrome （MAS） were retrospectively analyzed. Results All the 7 cases who suffered from MAS at the age of 3 months-5.2 years were female. Their symptoms were characterized by sexual precocity and remarkable pigmentation. Of the 7 cases, 1 was complicated by hyperthyroidism and 2 by mild rickets. Laboratory test showed significantly elevated E₂ level, normal FSH and LH level, normal blood calcium and phosphorus level, and elevated alkaline phosphatase level in all the 7 cases. Of the 7 cases, 1 had an elevated T₃ and T₄ level with a slightly Lower TSH level. Ultrasound examination showed ruterus and vary enlargement in 7 cases and unilateral oophoritic cyst in cases. Imaging demonstrated dysplasia of bone fibers in 5 cases and pathologic fracture line in 1 case, which could be symptomatically treated. Conclusion Although rarely encountered in clinical practice, MAS has its characteristic manifestations. A good knowledge about it helps its early diagnosis and treatment, prevents severe complications and improves the prognosis of such patients.