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Type 1 primary hyperoxaluria in pediatric patients: renal sonographic patterns

Ousséini Diallo1, Françoise Janssens, Michelle Hall

  • 1Department of Pediatric Imaging, Queen Fabiola Children's Hospital, Av. J.J. Crocq, Brussels 1020, Belgium.

AJR. American Journal of Roentgenology
|November 18, 2004
PubMed

Insights

Ultrasound can distinguish between two types of primary hyperoxaluria in children. Cortical nephrocalcinosis indicates a higher risk for end-stage renal disease (ESRD).

Area of Science:

  • Pediatric Nephrology
  • Medical Imaging
  • Genetics

Background:

  • Primary hyperoxaluria is a rare genetic disorder.
  • It leads to excessive oxalate production and deposition in the kidneys.
  • Type I primary hyperoxaluria is the most common subtype.

Purpose of the Study:

  • To review sonographic features of type I primary hyperoxaluria in children.
  • To correlate sonographic patterns with the development of end-stage renal disease (ESRD).

Main Methods:

  • Retrospective analysis of clinical and imaging files.
  • Involved 13 pediatric patients with type I primary hyperoxaluria.
  • Sonographic patterns and clinical follow-up were analyzed.

Main Results:

  • Two sonographic patterns were identified: medullary nephrocalcinosis (8 patients) and cortical nephrocalcinosis (5 patients).
  • Cortical nephrocalcinosis presented as a hyperechoic renal cortex with acoustic shadowing.
  • All patients with medullary nephrocalcinosis developed kidney stones; cortical nephrocalcinosis was associated with a higher risk of ESRD.

Conclusions:

  • Sonography can differentiate between medullary and cortical nephrocalcinosis in type I primary hyperoxaluria.
  • Cortical nephrocalcinosis is linked to a greater risk of developing ESRD.
Abstract

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