[Distal renal tubular acidosis in patients with Wilson's disease]

Renal affection is part of the clinical picture of developed Wilson's disease. The most frequent sign of affection of the distal nephron is distal tubular acidosis (DRTA), more frequently its latent form with a normal systemic pH, but inability to reduce the urinary pH below 5.5 after an acid load. More detailed assessment of the type of this disorder was not made so far. To elucidate in greater detail the pathogenesis of DRTA in Wilson's disease the authors examined renal acidification in 13 patients with bioptically verified Wilson's disease, using three acidification tests: 1) the test involving oral administration of calcium chloride (CaCl2), 2) the test involving infusion of sodium sulphate (Na2SO4) and 3) the test using an infusion of NaHCO3. In none of the examined patients with Wilson's disease manifest (complete) DRTA was found with systemic acidosis, however, in five patients latent (incomplete) DRTA was revealed, i.e. after administration of CaCl2 the pH of urine did not decline below 5.5. Patients with Wilson's disease and DRTA differed from patients with Wilson's disease and normal renal acidification by the age at the time of assessment of the diagnosis (30.4 +/- 3.6 vs. 20.75 +/- 2.14 years, p less than 0.02) and the duration of the disease (i.e. the time from assessment of the diagnosis to the acidification examination: 4.6 +/- 4.86 vs. 15.38 +/- 2.40 years, p less than 0.01). In three of four patients with Wilson's disease and DRTA who were given Na2SO4, after its administration the pH of urine did not decline below 5.2.(ABSTRACT TRUNCATED AT 250 WORDS)