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巴特综合征

Bartter's syndrome.

作者信息

Bartter F C

出版信息

Urol Clin North Am. 1977 Jun;4(2):253-61.

PMID:331616
Abstract

The syndrome of juxtaglomerular hyperplasia with hypokalemic, hypochloremic alkalosis, aldosteronism, hyperreninemia, and normal blood pressure may be familial, and is probably inherited as a recessive trait. It can usually be distinguished from salt-losing chronic glomerulo-nephritis by the histologic appearance of the kidneys, and by the absence of sodium "leak" with a low-sodium intake. Urinary, and thus renal, prostaglandin E is increased in the untreated patient: when this is lowered with prostaglandin synthetase inhibitors, the plasma renin and aldosterone decrease, and the plasma potassium concentration rises. These and other results suggest a partial control of renin secretion by prostaglandin E, and also suggest that prostaglandin E is an essential feature of the syndrome; it may, indeed, be a "proximal" cause of all the essential features.

摘要

伴有低钾血症、低氯血症性碱中毒、醛固酮增多症、高肾素血症及血压正常的肾小球旁器增生综合征可能具有家族性,且可能作为隐性性状遗传。通常可通过肾脏的组织学表现以及低钠摄入时无钠“渗漏”情况,将其与失盐性慢性肾小球肾炎相鉴别。未经治疗的患者尿中,进而肾内的前列腺素E会增加:当用前列腺素合成酶抑制剂使其降低时,血浆肾素和醛固酮会减少,血浆钾浓度会升高。这些及其他结果提示前列腺素E对肾素分泌有部分调控作用,也提示前列腺素E是该综合征的一个基本特征;实际上,它可能是所有基本特征的“近端”原因。

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