患儿，女，8岁，自幼多尿、烦渴、多饮，近1-2年常诉腿痛。血Na+132mmol/L,K+2.9mmol/L,Cl-116mmol/L,Ca2+1.8mmol/L。血气分析：pH7.22,HC03_11.8mmol/L,SBE-8mmol/L。尿pH6.5,镜检（-)。B超示双肾髓质区可见强回声团。哪一项是本病致病的关键

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