Medullary sponge kidney: abnormalities of renal tubular and glomerular function, and their relationship to clinical features.

This study confirms that medullary sponge kidney (MSK) has a good prognosis, but there is a considerable morbidity in patients with renal calcification; they suffer renal colic, ureteric obstruction, and frequently need operation. There is a high incidence of urinary infection in women. On follow-up, glomerular function is well maintained, although careful testing shows a mild depression of glomerular filtration rate in at least 40%. Proximal tubular function is normal, but abnormalities of distal tubular function are often seen: acidification defects occur in 24% and are associated with nephrocalcinosis, poor urine concentrating ability, and diminished glomerular function. Urine concentration defects occur in 73% and are probably secondary to nephrocalcinosis. Hypercalciuria was present in 19% and was not related to other defects.