肾小管性酸中毒(Renal Tubular A-cidosis,RTA)是一种少见的肾小管功能障碍性疾病。自1936年Lighfwood氏首次报告以来,国内外有关本病报告逐渐增多,国内迄今已逾50余例。最近我们收治2例原发性肾小管性酸中毒,现报告如下。例一汪姓,男,二岁六个月,住院号84574。因多饮多尿年余,四肢乏力二十余天,于1681年5月17日入院。一年来厌食,呕吐,口渴多饮,日饮水量达3000毫升,尿量约1500毫升,生长发育迟缓,曾诊断为“尿崩症”,治疗无效。近一月来发现乏力,日渐消瘦。无家族史。检查:身高76 Renal Tubular A-cidosis (RTA) is a rare renal tubular dysfunction. Since the first report of Lighfwood in 1936, there has been a steady increase in the reports of this disease at home and abroad, with over 50 cases so far in China. Recently, we admitted 2 cases of primary renal tubular acidosis, are as follows. A case of Wang surname, male, two years six months, hospital number 84574. Due to drink more urine more than a year, limb fatigue more than twenty days, on May 17, 1681 admission. One year of anorexia, vomiting, thirsty drink, daily drinking water volume of 3000 ml, urine output of about 1500 ml, growth retardation, had been diagnosed as “diabetes insipidus”, treatment ineffective. Nearly a month to find fatigue, getting thinner. No family history. Check: height 76