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[一名因巴特综合征导致慢性肾衰竭的患者]

[A patients with chronic renal failure due to Bartter's syndrome].

作者信息

Sato K, Ogata M

机构信息

Department of Internal Medicine, Asahi Hospital, Koriyama, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 1995 Jul;37(7):404-9.

PMID:7637211
Abstract

A male patient with hyperplasia of the juxtaglomerular apparatus, hypokalemia, an associated increase in plasma renin activity and hyperaldosteronism without hypertension was diagnosed as having Bartter's syndrome at the age of three. He was treated with spironolactone, indomethacin and potassium supplements. However hypokalemia, increased plasma renin activity and hyperaldosteronism persisted. Proteinuria was observed at the age of fifteen. Because of the gradual reduction of his renal function, the patient was admitted to our hospital and underwent hemodialysis at the age of twenty-one. He was normotensive. Serum potassium was within the normal range. Plasma renin activity and aldosterone concentration levels were still elevated. Renal biopsies, which had been carried out four times throughout his treatment, revealed an increase in sclerosis of the glomerular mesangial region, and interstitial fibrosis in proportion to narrowing of the small arteries in association with the juxtaglomerular hyperplasia. These findings suggested that changes in the glomeruli and interstitium of the kidney were secondary to long-standing hypokalemia, stenosis of the small arteries and the effects of the prescribed drugs for Bartter's syndrome.

摘要

一名患有肾小球旁器增生、低钾血症、血浆肾素活性升高及醛固酮增多症但无高血压的男性患者,三岁时被诊断为巴特综合征。他接受了螺内酯、吲哚美辛和钾补充剂治疗。然而,低钾血症、血浆肾素活性升高及醛固酮增多症持续存在。15岁时出现蛋白尿。由于肾功能逐渐减退,该患者于21岁时入住我院并接受血液透析。他血压正常。血清钾在正常范围内。血浆肾素活性和醛固酮浓度水平仍升高。在其整个治疗过程中进行了四次肾活检,结果显示肾小球系膜区硬化增加,间质纤维化与小动脉狭窄成比例,同时伴有肾小球旁增生。这些发现提示,肾脏的肾小球和间质变化是长期低钾血症、小动脉狭窄以及用于治疗巴特综合征的药物作用的继发结果。

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