【先天性、醛固酮合成或功能不足引起的盐消耗综合征——病因、诊断和治疗】。

Aleksander Basiak, Beata Wikiera, Anna Noczyńska
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引用次数: 0

摘要

盐消耗综合征是由先天性或后天合成障碍或醛固酮功能障碍引起的。它表现为离子紊乱,钠和氯的水平降低,同时钾潴留。合成的醛固酮位于肾上腺皮质的肾小球区。电解质异常症的症状并不明显,它们在出生后的几天内就会出现。厌恶吸力,冷漠,缺乏生长或进展,体重下降是值得注意的。盐耗综合征最常见的病因是21-羟化酶缺陷引起的先天性皮质肾上腺增生症(CAH)。区分了有盐耗和无盐耗的经典形态和非经典形态。SW形式的治疗依赖于氢化可的松和柯蒂纳夫的管理。其他形式的盐消耗综合征不常发生,它们是:醛固酮合成缺陷,脱氢酶3 -羟基类固醇缺陷,脂质皮质增生,先天性肾上腺发育不全(AHC),肾上腺白质营养不良和假性醛固酮减少症。了解盐消耗综合征的症状和原因有助于适当的治疗管理,并有助于儿童正常的婴幼儿心理生理发育。
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[Salt wasting syndrome caused by congenital, insufficient synthesis or aldosterone function--etiology, diagnosis and management].

Salt wasting syndrome is caused by a congenital or acquired synthesis disorder or by the aldosterone function disorder. It manifests itself by ionic disorders where the sodium and chlorine level decrease with the simultaneous potassium retention. Synthesised aldosterone is in the glomerular zone of the adrenal cortex. Symptoms of dyselectrolitemia are not distinctive, they develop within a few first days of life. The suction aversion, apathy, lack of growth or progressing, body mass loss is being noticed. The most often cause of salt wasting syndrome is the congenital cortical adrenal hyperplasia (CAH) caused by 21-hydroxylase enzyme deficit. The classic form with and without salt wasting (SW), as well as non-classic form is distinguished. The therapy of SW form depends on Hydrocortisone and Cortineff administering. The other forms of salt wasting syndrome occur not so often and these are: aldosterone synthesis deficit, dehydrogenase 3beta-hydroxysteroid deficit, lipoid cortical hyperplasia, adrenal hypoplasia congenital (AHC), adrenoleukodystrophy and pseudohypoaldosteronism. The knowledge of the symptoms and causes of salt wasting syndrome allows for the proper therapeutic management and contributes to the regular psychophysical infantile development of the children.

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