Batlle D, Kurtzman N A
Am J Kidney Dis. 1982 May;1(6):328-44. doi: 10.1016/s0272-6386(82)80004-8.
Distal renal tubular acidosis results from ineffective addition of hydrogen ions to the lumen of the distal nephron. The syndrome is manifested by hyperchloremic metabolic acidosis often associated with hypokalemia. More recently, it has been recognized that hyperkalemia rather than hypokalemia can be a dominant feature of some cases of distal renal tubular acidosis. It has been generally accepted that all cases of this syndrome ultimately resulted from a similar mechanism. The prevailing view was that the abnormality underlying distal renal tubular acidosis was that of inability to either generate or maintain a steep pH gradient across the distal nephron. Recent advances in our understanding of the process of distal acidification have provided evidence that different mechanisms can alter distal hydrogen ion secretion. In this article, the significance of the various indices of urinary acidification and their use in the characterization of the mechanism underlying distal renal tubular acidosis are revised. A classification of distal renal tubular acidosis on the basis of mechanism is presented. The importance of plasma potassium and renal potassium excretion in the evaluation of patients with distal renal tubular acidosis is emphasized.
远端肾小管酸中毒是由于远端肾单位管腔中氢离子添加无效所致。该综合征表现为高氯性代谢性酸中毒，常伴有低钾血症。最近，人们认识到高钾血症而非低钾血症可能是某些远端肾小管酸中毒病例的主要特征。人们普遍认为该综合征的所有病例最终都由相似的机制引起。普遍观点是，远端肾小管酸中毒的潜在异常是无法在远端肾单位中产生或维持陡峭的pH梯度。我们对远端酸化过程理解的最新进展提供了证据，表明不同机制可改变远端氢离子分泌。本文对尿酸化各种指标的意义及其在远端肾小管酸中毒潜在机制特征描述中的应用进行了修订。提出了基于机制的远端肾小管酸中毒分类。强调了血浆钾和肾钾排泄在评估远端肾小管酸中毒患者中的重要性。
