Zhang Chunli, Ren Hong, Shen Pingyan, Xu Yaowen, Zhang Wen, Wang Weiming, Li Xiao, Ma Yuhuan, Chen Nan
Department of Nephrology, Shanghai Ruijin Hospital affiliated to Shanghai Jiaotong University, School of Medicine, China.
Intern Med. 2015;54(7):725-30. doi: 10.2169/internalmedicine.54.9421. Epub 2015 Apr 1.
Distal renal tubular acidosis (dRTA) is a hyperchloremic metabolic acidosis disorder characterized by a normal anion gap with abnormal urinary hydrogen (H(+)) excretion. At present, there are few available reports regarding the clinical status of primary dRTA. The primary objective of this study was to analyze the clinical features and outcomes of primary dRTA.
This was a retrospective study performed in patients with primary dRTA who were hospitalized at Ruijin Hospital between March 1996 and July 2009; the clinical features of these patients were analyzed.
This study included 95 consecutive inpatients: 40 men (42.11%) and 55 women (57.89%). Among them, 60 had hypokalemia (63.12%), 29 had complete dRTA and 66 had incomplete dRTA. The mean urine calcium levels of the patients with and without urinary lithiasis were 0.10±0.04 and 0.07±0.05 mmol/24 h・kg, respectively (p=0.04). The blood pH values of the patients with and those without bone disease were 7.37±0.06 and 7.32±0.06, respectively (p=0.01). A total of 8.33% (8/27) of the patients had tubular proteinuria.
Hypokalemia is the most common clinical manifestation of primary dRTA. Primary dRTA can also be accompanied by proximal tubular dysfunction. Controlling the urine calcium and citrate levels is crucial for the treatment of nephrocalcinosis and/or nephrolithiasis, while restoring the blood pH to the normal level is essential for controlling bone disease.
远端肾小管酸中毒（dRTA）是一种高氯性代谢性酸中毒疾病，其特征为阴离子间隙正常但尿氢（H⁺）排泄异常。目前，关于原发性dRTA临床状况的可用报告较少。本研究的主要目的是分析原发性dRTA的临床特征及预后。
这是一项对1996年3月至2009年7月在瑞金医院住院的原发性dRTA患者进行的回顾性研究；分析了这些患者的临床特征。
本研究纳入95例连续住院患者，其中男性40例（42.11%），女性55例（57.89%）。其中，60例有低钾血症（63.12%），29例为完全性dRTA，66例为不完全性dRTA。有和无尿路结石患者的平均尿钙水平分别为0.10±0.04和0.07±0.05 mmol/24 h・kg（p = 0.04）。有和无骨病患者的血pH值分别为7.37±0.06和7.32±0.06（p = 0.01）。共有8.33%（8/27）的患者有肾小管性蛋白尿。
低钾血症是原发性dRTA最常见的临床表现。原发性dRTA也可伴有近端肾小管功能障碍。控制尿钙和枸橼酸盐水平对治疗肾钙质沉着症和/或肾结石至关重要，而将血pH值恢复到正常水平对控制骨病至关重要。
