Unilateral and segmental medullary sponge kidney: renal function and calcium excretion

The renal acidification defect and renal hypercalciuria have been reported in patients with pan-renal and bilateral medullary sponge kidney. However, little is known about patients with unilateral or segmentally affected medullary sponge kidney. We report 2 cases of partially affected medullary sponge kidney with normal acidification ability and normal urinary calcium excretion, although the ability to concentrate urine was diminished. These findings suggest that in patients with partially affected medullary sponge kidney nephrolithiasis is not the consequence of renal hypercalciuria induced by systemic acidosis but is owing to the urinary stasis caused by cystic dilatation of the terminal collecting duct.