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Review
. 2022 May 17;15(Suppl 1):i23-i28.
doi: 10.1093/ckj/sfab231. eCollection 2022 May.

Primary hyperoxaluria: the pediatric nephrologist's point of view

Efrat Ben-Shalom  1 , Sander F Garrelfs  2 , Jaap W Groothoff  2
Affiliations
Review

Primary hyperoxaluria: the pediatric nephrologist's point of view

Efrat Ben-Shalom et al. Clin Kidney J. .

Abstract

The clinical presentation of primary hyperoxaluria in children ranges from mildly symptomatic nephrocalcinosis to very early onset end-stage kidney failure with systemic oxalosis, a devastating complication. We review the various manifestations of pediatric hyperoxaluria, treatment options for children with preserved kidney function and appropriate dialysis regimens. Liver or combined liver/kidney transplantation is currently the only definitive treatment for primary hyperoxaluria type 1, but novel RNA interference treatments offer hope for the future. Finally, we address the medical and ethical dilemmas facing pediatricians treating children with hyperoxaluria.

Keywords: dialysis; hyperoxaluria; kidney transplantation; liver transplantation; pediatrics; systemic oxalosis.

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Figures

FIGURE 1:
FIGURE 1:
Manifestations of systemic oxalosis.
FIGURE 2:
FIGURE 2:
Diagnostic approach to a child suspected of having primary hyperoxaluria. PTH, parathyroid hormone; Cr, creatinine; IBD, inflammatory bowel disease.
See this image and copyright information in PMC

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