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Primary hyperoxaluria type 1: urologic and therapeutic management
Harjivan Kohli1, Michael P Kurtz1
1Boston Children's Hospital, Department of Urology, Boston, MA, USA.
Clinical Kidney Journal
|May 20, 2022
Summary
Surgical interventions for primary hyperoxaluria (PH) urolithiasis require careful consideration due to unique toxicities. This review highlights disease-specific factors influencing treatment choices for PH patients.
Area of Science:
- Nephrology
- Urology
- Rare Diseases
Background:
- Primary hyperoxaluria (PH) presents unique challenges in urolithiasis management.
- Treatment indications and toxicities in PH differ significantly from general urolithiasis.
- Management decisions are often guided by limited case series due to the rarity of PH.
Purpose of the Study:
- To review and summarize existing literature on surgical interventions for PH.
- To highlight disease-specific considerations for treating urolithiasis in PH patients.
- To inform surgical decision-making by outlining the risks and benefits of various procedures.
Main Methods:
- Literature review of available studies on surgical approaches for PH urolithiasis.
- Analysis of disease-specific factors influencing treatment outcomes.
- Evaluation of common urological interventions including SWL, PCNL, and ureteroscopy.
Main Results:
- Shockwave lithotripsy (SWL) shows limited efficacy and potential renal function decline in pediatric PH patients.
- Percutaneous nephrostolithotomy (PCNL) is associated with rapid chronic kidney disease (CKD) progression postoperatively.
- Ureteroscopy is often the safest option but faces limitations like stent encrustation and residual stone growth.
Conclusions:
- Surgical management of PH urolithiasis necessitates balancing procedure-specific risks with the patient's CKD status.
- Each surgical modality (SWL, PCNL, ureteroscopy) presents distinct challenges in the context of PH.
- Careful patient selection and monitoring are crucial for optimizing outcomes in PH patients undergoing urolithiasis treatment.
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