Ⅳ型肾小管性酸中毒(RTA)为远端RTA的一种特殊类型,以高氯型代谢性酸中毒伴高钾血症为临床特征,又称高血钾型酸中毒。该病相对少见,国内外常见为个案报道。因高钾血症导致误诊、漏诊甚至死亡,临床上应予以警惕。我科曾收治1例原发性肾上腺功能减退症(Addison病)合并Ⅳ型RTA的患者,现报道如下。 Type IV renal tubular acidosis (RTA) is a special type of distal RTA, high chloride metabolic acidosis with hyperkalemia as a clinical feature, also known as hyperkalemic acidosis. The disease is relatively rare, common case reports at home and abroad. Due to hyperkalemia lead to misdiagnosis, missed diagnosis and even death, should be vigilant clinically. My department has admitted a case of primary adrenal hypothyroidism (Addison’s disease) with type IV RTA, are reported below.