Related Experiment Videos
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
Summary
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.