【摘要】 Bartter综合征是一种极罕见的疾病。本文收集本院 1例及全国其他 3 4例 ,进行综合分析。发现儿童期发病与成人基本相等 ,儿童以男性为多 (66 7% ) ,成人则以女性为多 (82 3 % )。特点有 :(1)水、电解质及酸碱失衡 ,包括低钾、钠血症、低氯性碱中毒及脱水等 ;(2 )血肾素、血管紧张素Ⅱ及醛固酮等均明显升高 ,而血压正常 ;(3 )生长发育迟滞在儿童最为突出 (84 6% ) ,其中 4例有智力低下 ;(4) 14例肾活检肾脏病变除肾小球旁器增生 ,还有肾小管间质及肾小球损害 ;(5 )以吲哚美辛、螺内酯及补钾等综合治疗效果显著。结合病理、伴发疾病、症状及实验室检查等分析发现 ,成人以后天获得性为多 ,而儿童大多为特发性。更多还原

【Abstract】 Bartter’s syndrome is a rare disease.This study analysed 35 cases of the disease for the first time in our country,in which one cases was reported from our hospital and 34 cases reported throughout the country.We found the incidence was almost the same between children and adults.The majority of the patients in childhood were male(66 7%)and that of the adult was female(82.3%).The hallmarks of Bartter’s syndrome were mentioned below:(1)Water electrolytes and acid base disturbances including hypokalemia,hyponatremia,hypochloremic alkalosis and dehydration;(2)Obviously increase of renin,angiotension Ⅱ and aldosterone,but the blood pressure almost normal;(3)Failure to thrive was prominent feature of the patient in childhood,among them 4 cases had mental retardation;(4)Rneal histology was characterized by the damage to renal tubules,interstitial tissue,glomeruli neighboring the juxataglomerular cell hyperplasia and(5)Comprehensive therapy with indomethacin,spirolactone and potassium had remarkable effect.By analysis combined with clinical manifestations,laboratory examination and renal pathology,we found that the patients in adult were mostly secondary and these in childhood were mainly specific.更多还原