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This study concerned a Tunisian family of 52 members. We have looked for distal tubular acidosis (D.T.A.) after we had discovered that 2 members of this family suffered from nephrocalcinosis with D.T.A. Twenty six of our patients (group I) suffered from D.T.A., 17 from confirmed metabolic acidosis with low urinary pH (5.22 +/- 0.25). Patients of latest groups may present a D.T.A. with intact capacity to lower urinary pH. Our literature review revealed 27 families composed of 228 patients.
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