【摘要】 目的 探索远段肾小管性酸中毒(dRTA)的一些特殊临床表现，减少误诊和漏诊。方法 回顾性研究、分析了4例RTA临床表现、治疗及误诊原因。结果 4例误诊病例，均表现为高氯性代谢性酸中毒合并低钾血症，伴有不同程度肾功能损害。经积极治疗后，4例患者代谢性酸中毒、低钾血症均纠正；3例患者肾功能恢复正常。结论 多种因素能引起远段肾小管性酸中毒，其临床表现多样，临床上应全面判断，必要时行肾穿刺活检，争取早期诊断早期治疗，以防止肾功能进行性恶化。

    【关键词】 肾小管性酸中毒；误诊

    【Abstract】 Objective Questing for some special clinical appearance of dRTA，in order to reduce misdiagnosis.Methods Data of 4 cases with analyzed retrospectively，analysing the clinical appearance of dRTA and the cause of misdiagnosis. Results All 4 misdiagnosed cases responsed hypopotassemia，metabolic acidosis，and renal dysfunction.After positive treatment，their hypopotassemia and metabolic acidosis corrected，and kidey function improved.Conclusion Several factors cause dRTA，and the clinical characteristic is varied;we should think it over before perorating，make needling biopsy of kidney if necessarily，in order to diagnosis and therapy earlier，to prevent renal function worsening.

    【Key words】 renal tubular acidosis；misdiagnosis

    远端肾小管性酸中毒(RTA)是指一组由于肾小管远端分泌H+引起的慢性高氯性代谢性酸中毒为特点的临床综合征，是肾小管病变中较常见的疾病[1]。多数学者认为它是常染色体显性遗传病 ......