Medullary sponge kidney associated with distal renal tubular acidosis in a 5-year-old girl

Eur J Pediatr. 2006 Sep;165(9):648-51. doi: 10.1007/s00431-006-0125-0. Epub 2006 Apr 7.


Introduction: Medullary sponge kidney (MSK) is characterized by cystic dilatation of the inner medullary collecting ducts, which causes the kidneys to resemble a sponge.

Case report: Although distal renal tubular acidosis (dRTA) is commonly observed in patients with MSK, we report a 5-year-old girl with MSK who had features of both dRTA (nephrocalcinosis, hypercalciuria, hypocitraturia) and proximal tubular dysfunction (hyperuricosuria, impaired tubular phosphate reabsorption and proteinuria).

Discussion: Metabolic acidosis, hypercalciuria, hypocitraturia, tubular phosphate reabsorption and growth retardation in the patient improved with alkali therapy.

Publication types

  • Case Reports

MeSH terms

  • Acidosis, Renal Tubular / complications*
  • Acidosis, Renal Tubular / diagnostic imaging
  • Acidosis, Renal Tubular / pathology*
  • Biomarkers / blood
  • Biomarkers / urine
  • Child, Preschool
  • Female
  • Humans
  • Kidney Tubules, Distal / diagnostic imaging
  • Kidney Tubules, Distal / metabolism
  • Kidney Tubules, Distal / pathology*
  • Kidney Tubules, Proximal / pathology
  • Medullary Sponge Kidney / complications*
  • Medullary Sponge Kidney / diagnostic imaging
  • Medullary Sponge Kidney / pathology*
  • Nephrocalcinosis / etiology
  • Nephrocalcinosis / pathology
  • Ultrasonography, Interventional
  • Urography


  • Biomarkers