Nephrocalcinosis in a patient with primary hyperoxaluria type 2

Pediatr Nephrol. 1996 Aug;10(4):442-4. doi: 10.1007/s004670050135.

Abstract

Although nephrocalcinosis is a classical finding in primary hyperoxaluria type 1 (PH 1) associated with a poor renal survival it is exceptional in patients with PH type 2 (PH 2), characterized by a more favorable outcome. We describe an 8-month-old girl who suffered from recurrent urinary tract infections. Imaging studies revealed a profound corticomedullary nephrocalcinosis with no evidence of calculi. Urinary oxalate and D-glycerate excretion were massively elevated, while urinary glycolate or glyoxylate could not be detected, confirming the diagnosis of PH 2. Although the nephrocalcinosis progressed radiologically, renal function remained stable for over 2 years. Only further follow-up will show whether the associated nephrocalcinosis worsens the prognosis of our patient and of PH 2 in general.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Female
  • Glyceric Acids / urine
  • Glycolates / urine
  • Glyoxylates / urine
  • Humans
  • Hydrogen-Ion Concentration
  • Hyperoxaluria, Primary / complications*
  • Hyperoxaluria, Primary / diagnostic imaging
  • Kidney / diagnostic imaging
  • Nephrocalcinosis / diagnostic imaging
  • Nephrocalcinosis / urine*
  • Oxalates / urine
  • Radiography
  • Ultrasonography
  • Urinary Calculi / diagnostic imaging
  • Urinary Calculi / urine

Substances

  • Glyceric Acids
  • Glycolates
  • Glyoxylates
  • Oxalates