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Crystalluria: a clinically useful investigation in children with primary hyperoxaluria post-transplantation
P Jouvet1, L Priqueler, M F Gagnadoux
1Département d'Anesthésie, Hôpital Necker-Enfants Malades, Paris, France. philippe.jouvet@nck.ap-hop-paris.fr
Insights
Primary hyperoxaluria type I (PH I) can cause kidney failure. Oxalate crystal volume (OCV) monitoring offers a rapid, non-invasive method to assess treatment effectiveness in children with PH I post-transplant.
Area of Science:
- Biochemistry
- Pediatric Nephrology
- Metabolic Disorders
Background:
- Primary hyperoxaluria type I (PH I) is a genetic metabolic disorder characterized by excessive oxalate production, leading to kidney damage and failure.
- Liver/kidney transplantation in PH I patients can still result in persistent oxalate excretion, risking systemic oxalosis and graft dysfunction.
- Crystalluria, the presence of crystals in urine, is hypothesized to predict the tendency for stone formation (nephrolithiasis) in these patients.
Purpose of the Study:
- To investigate the efficacy of an intensive therapeutic strategy aimed at preventing crystal formation in children with PH I.
- To evaluate the utility of Oxalate Crystal Volume (OCV) measurements as a non-invasive tool for monitoring treatment response.
- To compare the predictive value of crystalluria (OCV) against urine supersaturation for nephrolithogenic risk.
Main Methods:
- A cohort of 13 children with PH I underwent an intensive therapeutic intervention.
- Oxalate Crystal Volume (OCV) was measured at regular intervals over 36 months.
- OCV measurements were compared with urine supersaturation data.
Main Results:
- Oxalate crystal volume (OCV) measurement proved to be a non-invasive and easily performed method.
- OCV provided rapid feedback on the effectiveness of PH I therapy, within one hour.
- The study demonstrated the feasibility of using OCV for monitoring treatment efficacy.
Conclusions:
- Oxalate crystal volume (OCV) monitoring is a promising, rapid, and non-invasive tool for assessing PH I treatment efficacy.
- Further research is required to ascertain if OCV is a superior predictor of nephrocalcinosis compared to urine supersaturation alone.
- This method offers valuable insights into managing crystalluria and preventing complications in PH I patients post-transplant.
Abstract:
Primary hyperoxaluria type I (PH I) is a congenital error of metabolism that can be manifested by an increased oxalate production, and ultimately result in kidney failure. After a combined liver/kidney transplantation, children with PH I have persistent excretion of oxalate that causes crystal formation in the urinary tract, and could result in systemic oxalosis and eventual graft failure. We speculated that crystalluria may be predictive of this nephrolithogenic tendency and thus investigated the effect of an intensive therapeutic strategy to prevent crystal formation in 13 children at our hospital. Oxalate crystal volume (OCV) measurements were performed at regular intervals for 36 months, and compared with urine supersaturation measurements. We found that crystalluria with the OCV measurement is non-invasive, easily performed, and gives feedback on the efficacy of PH I therapy within one hour. Further study is needed to determine whether this method is a better predictor of nephrocalcinosis than is supersaturation alone.