青少年先天性肾上腺皮质增生症的研究进展
发布时间:2018-03-29 21:37
本文选题:肾上腺皮质疾病 切入点:青少年 出处:《中国全科医学》2017年18期
【摘要】:先天性肾上腺皮质增生症是由于皮质激素合成过程中特定的酶缺乏引起的常染色体隐性遗传病,临床表现为糖皮质激素、盐皮质激素缺乏和雄激素过多的症状。青少年患者的内分泌紊乱、依从性差,加之治疗不规范,可最终导致身材矮小以及出现生育能力降低、心血管疾病风险、骨密度降低等不良临床特征。骨骺闭合前治疗目标主要是充分抑制雄激素、控制男性化症状、保证正常生长,骨骺闭合后,其治疗目标就转变成抑制雄激素,预防长期不良临床特征。
[Abstract]:Congenital adrenocortical hyperplasia is an autosomal recessive disorder caused by a specific enzyme deficiency in the process of corticosteroid synthesis, with clinical manifestations of glucocorticoid. Symptoms of salt corticosteroid deficiency and androgen overload. Endocrine disorders, poor compliance, and nonstandard treatment can ultimately lead to short stature, reduced fertility, and a risk of cardiovascular disease in adolescent patients. Reduction of bone mineral density and other adverse clinical characteristics. Before epiphyseal closure, the main treatment goals are to fully inhibit androgen, to control masculine symptoms and to ensure normal growth. After epiphyseal closure, the treatment goal changes to inhibit androgen. Prevention of long-term adverse clinical characteristics.
【作者单位】: 山西医科大学研究生院;山西省儿童医院内分泌科;
【分类号】:R725.8
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