先天性肾上腺皮质增生症合并中枢性性早熟临床分析

Clinical analysis on the congenital adrenal hyperplasia complicated central precocious puberty

目的分析先天性肾上腺皮质增生症(CAH)患儿合并中枢性性早熟的临床表现。方法通过回顾性分析和临床随访,在12例21羟化酶缺乏患儿中发现20例合并中枢性性早熟。根据治疗和非治疗情况分为A组(9例)和B组(11例),分析其发生的年龄、骨龄以及与激素替代治疗的关系。结果A组中发生中枢性性早熟的实际年龄平均为(5.6±2.1)岁,骨龄平均为(12.0±3.2)岁;B组中诊断中枢性性早熟平均年龄在(6.8±1.1)岁;骨龄平均值在(11.7±2.0)岁,两组在统计学上差异无显著性。B组应用氢化可的松治疗后平均2.3年出现中枢性性早熟。结论CAH患儿骨龄发育提前是发生性早熟的主要原因,早诊断和早治疗可改善预后。

Objective To study the clinical manifestation of patients with congenital adrenal hyperplasia （CAH） and central precocious puberty （CPP）, Methods Clinical data of 120 patients with 21-hydroxylase deficiency was studied retrospectively. Among them. twenty patients with 21-hydroxylase deficiency and CPP. These 20 patients were divided into 2 groups according to the treatment status and the relationship between the initial age of CPP presentation, bone age and steroid hormone replaced treatment was analyzed. Results The initial age of CPP presentation in group A was 5.6±2, 1 years, the bone age was 12.0±3,2 years, The initial age of CPP in group B were 6.8 ±1.1 years, the bone age was 11.7±2,0 years, There were no statistical differences between the two groups. The CPP occurred at 2.3 years after treatment with hydrocortisone in group B, Conclusions CPP were closely related to the advanced bone age, earlier nosis. Patients with congenital adrenal hyperplasia and diagnosis and treatment could improve the prognosis.