Pathophysiology Division, Pathology Department, School of Medicine, National University of Cuyo, Mendoza, Argentina; and Division of Nephrology, Department of Medicine, Northwestern University, Feinberg School of Medicine, Chicago, IL.
Pathophysiology Division, Pathology Department, School of Medicine, National University of Cuyo, Mendoza, Argentina; and Division of Nephrology, Department of Medicine, Northwestern University, Feinberg School of Medicine, Chicago, IL.
Adv Chronic Kidney Dis. 2018 Jul;25(4):303-320. doi: 10.1053/j.ackd.2018.05.003.
Distal renal tubular acidosis (DRTA) is defined as hyperchloremic, non-anion gap metabolic acidosis with impaired urinary acid excretion in the presence of a normal or moderately reduced glomerular filtration rate. Failure in urinary acid excretion results from reduced H secretion by intercalated cells in the distal nephron. This results in decreased excretion of NH and other acids collectively referred as titratable acids while urine pH is typically above 5.5 in the face of systemic acidosis. The clinical phenotype in patients with DRTA is characterized by stunted growth with bone abnormalities in children as well as nephrocalcinosis and nephrolithiasis that develop as the consequence of hypercalciuria, hypocitraturia, and relatively alkaline urine. Hypokalemia is a striking finding that accounts for muscle weakness and requires continued treatment together with alkali-based therapies. This review will focus on the mechanisms responsible for impaired acid excretion and urinary potassium wastage, the clinical features, and diagnostic approaches of hypokalemic DRTA, both inherited and acquired.
远端肾小管性酸中毒（DRTA）定义为在肾小球滤过率正常或中度降低的情况下，存在高氯、非阴离子间隙代谢性酸中毒和尿酸排泄受损。尿酸排泄的失败是由于闰细胞中 H+分泌减少所致。这导致 NH 和其他酸（统称为可滴定酸）的排泄减少，而在全身酸中毒的情况下，尿液 pH 值通常高于 5.5。DRTA 患者的临床表型特征为儿童生长迟缓伴骨骼异常，以及肾钙质沉着症和肾结石，这是高钙尿、低枸橼酸尿和相对碱性尿的后果。低钾血症是一个显著的发现，可导致肌肉无力，需要与碱化剂治疗一起持续治疗。这篇综述将重点介绍导致酸排泄和尿钾丢失受损的机制、低钾 DRTA 的临床特征和诊断方法，包括遗传性和获得性。
