Renal Acidification Defects in Medullary Sponge Kidney

Medullary Sponge Kidney with Distal Renal Tubular Acidosis: A Case Report and Review of the Literature

“…Although most commonly asymptomatic, 50% of patients with MSK have the propensity to develop renal calculi, most commonly comprised of calcium oxalate and calcium phosphate [4]. It has been known that hypercalciuria and distal tubular acidification defects play a role in the pathogenesis of kidney stones formation and nephrocalcinosis [4,15]. While there is no hypercalciuria associated with MSK, the dilated collecting tubules contribute to urinary stasis, which allows the urine to crystalize and subsequently pass the ductal stones into the renal pelvis where they may serve as foci for further stone formation [4,16].…”

Renal Cystic Disease

“…Renal tubular acidosis is reported to occur in 69% of patients with MSK (182). The impact of bicarbonate therapy on the pathogenesis of nephrolithiasis has not been examined critically in patients with renal tubular acidosis, yet bicarbonate therapy is believed to decrease the frequency of stone passage (191).…”

“…Although MSK is largely an asymptomatic disease, patients may present with hematuria, nephrolithiasis, hypercalciuria, urinary tract infections, and renal tubular acidosis (181,182). Nephrolithiasis, with renal colic, loin pain, and excretion of small stones, can be a prominent feature (183).…”