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Bartter综合征系一种常染色体隐性遗传的失钾性肾小管病。1962年Bartter首先报告2例低钾性碱中毒后,国外报告100余例,国内未见报告。发病机理未明,主要有4种推测:1.原发性高肾素血症;2.肾小管近端钠重吸收障碍;3.小动脉壁对AT-Ⅱ缺乏反应;4.肾脏前列腺素合成过多,致失钠过多,而继发肾素、醛固酮增多及失钾。治疗主要是纠正脱水,补充钠、钾、镁。螺旋内酯可帮助保钾;阿斯匹林、消炎痛因有抑制前列腺素合成酶的作用;心得安、甲基多巴因有阻滞肾素分泌的作用,故均有一定疗效,现将我科所见一例报告如下:
Bartter syndrome is an autosomal recessive K-tubule disease. Bartter first reported in 1962, two cases of hypokalemic alkalosis, more than 100 foreign reports, no domestic report. Pathogenesis is unknown, there are four kinds of speculation: 1. Primary hyperreninmia; 2. Renal proximal renal reabsorption disorders; 3. Arterial wall of AT-Ⅱ lack of response; 4. Kidney prostaglandin synthesis Too much sodium causes too much, and secondary to renin, aldosterone increased and loss of potassium. Treatment is mainly to correct dehydration, add sodium, potassium, magnesium. Spirolactone can help protect potassium; aspirin, indomethacin inhibited by prostaglandin synthase; propranolol, methyidopa due to block the role of renin secretion, it has a certain effect, will now A case study of our department is as follows: