Table 1.

Treatment of Urolithiasis Associated with Lesch-Nyhan syndrome

Medical Management

Invasive Procedures

ᅟ■ High fluid intake ᅟ■ Maintain alkaline urine with potassium or sodium citrate to prevent uric acid stone formation (urinary pH: 6.5–7.0) ᅟ■ Gradually increase allopurinol dose from 2.5 mg/kg doses per day to 10 mg/kg per day and administer 2–3 doses per day ᅟ■ Monitor purine metabolites of allopurinol in blood and urine ᅟ■ Assess urinary uric acid, xanthine, hypoxanthine, and oxypurinal excretion to determine accurate allopurinol dosage ᅟ■ Avoid excess dietary purines, calcium, salts ᅟ■ Routine renal ultrasound to monitor for uric acid nephropathy

■ Percutaneous nephrolithotomy ■ Shock wave lithotripsy ■ Ureteroscopy ■ Open surgery