肾上腺紊乱

G. Butler, J. Kirk
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引用次数: 0

摘要

•胎儿肾上腺有一个额外的皮质,在出生后萎缩。•出生后,皮层有三个区域:◦肾小球产生矿化皮质激素;◦束状膜产生皮质醇;肾上腺素从6岁开始随着雄激素的增加而逐渐成熟。•急性肾上腺危机表现为:◦低钠◦高钾◦皮质醇低/检测不到。•ACTH在原发性原因中升高,在继发性原因中降低。•原发性功能不全可能是先天性发育不良(先天性肾上腺发育不全)和酶阻滞:先天性肾上腺增生(CAH)。•获得性原因包括自身免疫性肾上腺炎(艾迪森病)和结核病。继发性功能不全是由于垂体功能低下(先天性或后天)引起的促肾上腺皮质激素缺乏。•CAH,如21-羟化酶缺乏症(最常见),可表现为新生儿女性阳刚化、雄激素过多或耗盐肾上腺危机。医源性治疗是肾上腺类固醇过量的最常见原因。•其他原因是肾上腺腺瘤/癌(库欣综合征)或垂体腺瘤(库欣病)。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Adrenal gland disorders
• The fetal adrenal gland has an additional cortex which atrophies after birth. • Postnatally, there are three zones to the cortex: ◦ glomerulosa producing mineralocorticoids ◦ fasciculata producing cortisol ◦ reticularis producing androgens. • Adrenarche is a maturation process from age 6 years with an increase in androgens. • Acute adrenal crisis presents with: ◦ low sodium ◦ high potassium ◦ cortisol low/undetectable. • ACTH raised in primary causes, low in secondary. • Primary insufficiency may be congenital dysplasia (adrenal hypoplasia congenita) and enzyme blocks: congenital adrenal hyperplasia (CAH). • Acquired causes include autoimmune adrenalitis (Addison’s disease) and tuberculosis. • Secondary insufficiency arises due to adrenocorticotropic hormone deficiency from hypopituitarism (congenital or acquired). • CAH, e.g. 21-hydroxylase deficiency (commonest), can present with virilization of a newborn female, hyperandrogenism, or salt-wasting adrenal crisis. • Iatrogenic treatment is commonest cause of adrenal steroid excess. • Other causes are adrenal adenoma/carcinoma (Cushing syndrome) or pituitary adenoma (Cushing disease).
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