Urinary acidification ability, acid-base status and urinary excretion of calcium and citrate were evaluated in 10 women with bilateral medullary sponge kidney (MSK) and in 10 healthy women. Patients with MSK had higher fasting urine pH compared to normal controls (p < 0.01). Four patients had incomplete renal tubular acidiosis (iRTA), 3 had hypercalciuria, and 5 patients had hypocitraturia. The 24-hour urinary excretion of calcium was increaed in the females with MSK (5.23 ± 0.78 mmol) compared to the healthy females (3.49 ± 0.29 mmol) (p < 0.02), and increased in MSK patients with iRTA (7.32 ± 1.45 mmol) compared to patients with normal urinary acidification (3.83 ± 0.12 mmol) (p < 0.01). The patients with iRTA had reduced levels of plasma standard bicarbonate (20.5 ± 1.0) after fasting compared to patients with normal urinary acidification (23.8 ± 0.8) and healthy women (22.7 ± 0.6) (p < 0.01), and reduced levels of 24-hour urinary excretion of citrate (0.93 ± 0.25 mmol) compared to patients with normal urinary acidification (3.58 ± 0.51) and healthy women (2.78 ± 0.49) (p < 0.005). A positive correlation was found between the degree of acidosis during ammonium chloride loading and urinary excretion of calcium (r = 0.71, p = 0.02), and a negative correlation between the degree of acidosis during ammonium chloride loading and urinary citrate excretion (r = 0.87, p = 0.001). The results suggest that defective urinary acidification might play an important role in the mechanism of hypercalciuria and hypocitraturia in patients with medullary sponge kidney. Furthermore, our data suggest that in the group of patients with bilateral MSK there might be two categories. In one category, iRTA is present. The main metabolic lithogenic factors in this group appear to be increased urinary excretion of calcium, decreased urinary excretion of citrate and increased urine pH. The other category does not have iRTA, and the metabolic abnormalities related to stone disease are much less pronounced.

Keywords:
Medullary sponge kidney, Women, Calcium, Citrate, Urine acidification
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© 1994 S. Karger AG, Basel
1994
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