• Chunli Zhang, Hong Ren, Pingyan Shen, Yaowen Xu, Wen Zhang, Weiming Wang, Xiao Li, Yuhuan Ma, and Nan Chen.
• Department of Nephrology, Shanghai Ruijin Hospital affiliated to Shanghai Jiaotong University, School of Medicine, China.
• Intern. Med. 2015 Jan 1; 54 (7): 725730725-30.

ObjectiveDistal 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.MethodsThis 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.ResultsThis 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.ConclusionHypokalemia 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.

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