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青少年先天性腎上腺皮質(zhì)增生癥的研究進(jìn)展

發(fā)布時(shí)間:2018-03-29 21:37

  本文選題:腎上腺皮質(zhì)疾病 切入點(diǎn):青少年 出處:《中國(guó)全科醫(yī)學(xué)》2017年18期


【摘要】:先天性腎上腺皮質(zhì)增生癥是由于皮質(zhì)激素合成過(guò)程中特定的酶缺乏引起的常染色體隱性遺傳病,臨床表現(xiàn)為糖皮質(zhì)激素、鹽皮質(zhì)激素缺乏和雄激素過(guò)多的癥狀。青少年患者的內(nèi)分泌紊亂、依從性差,加之治療不規(guī)范,可最終導(dǎo)致身材矮小以及出現(xiàn)生育能力降低、心血管疾病風(fēng)險(xiǎn)、骨密度降低等不良臨床特征。骨骺閉合前治療目標(biāo)主要是充分抑制雄激素、控制男性化癥狀、保證正常生長(zhǎng),骨骺閉合后,其治療目標(biāo)就轉(zhuǎn)變成抑制雄激素,預(yù)防長(zhǎng)期不良臨床特征。
[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.
【作者單位】: 山西醫(yī)科大學(xué)研究生院;山西省兒童醫(yī)院內(nèi)分泌科;
【分類號(hào)】:R725.8

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