巴特综合征(Bartter’s Syndrome,简称BS)的特点是肾小球旁器的肥大和增生,高肾素血症,高醛固酮血症,低血钾性碱中毒,对外源性血管紧张素反应低下,血压正常。早期临床表现为多尿、烦渴、便秘、厌食和呕吐。1962年由Bartter首次报告2例,此后陆续有类似报告。迄今,英美文献报告百余例,大多为小儿,其中5岁以下占一半。国内文献多为个例报告。可能为常染色体隐性遗传性疾病。病因和发病机制BS的病因和发病机制尚不完全清楚。由于BS临床类型的复杂多变,还没有一种理论能圆满解释发病机理,现简要做一回顾。1.末梢血管对血管紧张素Ⅱ反应低下说:无论是外源性或内源性的血管紧张素Ⅱ,末梢血管对 Bartter’s Syndrome (BS) is characterized by hypertrophy and hyperplasia of the glomerular periderm, hyperrenalmia, hyperaldosteronism, hypokalemic alkalosis, low response to exogenous angiotensin, Normal blood pressure. Early clinical manifestations of polyuria, polydipsia, constipation, anorexia and vomiting. Two cases were first reported by Bartter in 1962, followed by similar reports one after another. So far, more than 100 cases have been reported in the Anglo-American literature, most of them children, half of whom are under 5 years of age. Domestic literature is mostly a case report. May be autosomal recessive genetic disease. Etiology and pathogenesis The etiology and pathogenesis of BS is not fully understood. Due to the complex and changeable clinical types of BS, no theory can satisfactorily explain the pathogenesis, so we briefly review it. 1. Peripheral blood vessels of low response to angiotensin II said: either exogenous or endogenous angiotensin Ⅱ, peripheral blood vessel pairs