OBJECTIVE: To investigate the effect of prophylactic treatment with Shohl's solution on the rates of stone recurrence in paediatric patients with cystinuria. PATIENTS AND METHODS: Between June 2007 and October 2011, 185 patients aged 16 years and younger whose stones had been completely removed by percutaneous nephrolithotomy (PCNL) were assessed for metabolic risk factors. Seventeen (9%) patients with positive cyanide-nitroprusside tests (CNT) and cystine stones enrolled in this study, and a Shohl's solution was used for alkalinisation. The patients were followed up for a mean period of two years for stone recurrence. RESULTS: Of the patients, 10 (59%) were male, and 7 (41%) were female (p = 0.13). Twelve patients (70.5%) continued to receive medical prophylaxis regularly, whereas 5 (29.5%) patients did not. The mean pre-treatment and post-treatment urinary pH values were 5.8 ± 0.5 (5-7) and 7.5 ± 0.4 (6.5-8), respectively (p < 0.001). The pre-treatment and post-treatment specific gravities of the urine were 1021.5 ± 5.4 (1010-1030) and 1006 ± 2.3 (1004-1015), respectively (p < 0.001). The rates of recurrence were 16.6% among those who continued prophylaxis and 100% among those who did not receive prophylaxis (p = 0.001). The most common combination of metabolic anomalies was cystinuria and hypocitraturia (p < 0.001). CONCLUSIONS: This study demonstrated that detailed clinical and laboratory evaluations should be performed for all children with cystine stone disease, and, appropriate prophylactic treatment should be recommended to prevent the reformation of stones.
OBJECTIVE: To investigate the effect of prophylactic treatment with Shohl's solution on the rates of stone recurrence in paediatric patients with cystinuria. PATIENTS AND METHODS: Between June 2007 and October 2011, 185 patients aged 16 years and younger whose stones had been completely removed by percutaneous nephrolithotomy (PCNL) were assessed for metabolic risk factors. Seventeen (9%) patients with positive cyanide-nitroprusside tests (CNT) and cystine stones enrolled in this study, and a Shohl's solution was used for alkalinisation. The patients were followed up for a mean period of two years for stone recurrence. RESULTS: Of the patients, 10 (59%) were male, and 7 (41%) were female (p = 0.13). Twelve patients (70.5%) continued to receive medical prophylaxis regularly, whereas 5 (29.5%) patients did not. The mean pre-treatment and post-treatment urinary pH values were 5.8 ± 0.5 (5-7) and 7.5 ± 0.4 (6.5-8), respectively (p < 0.001). The pre-treatment and post-treatment specific gravities of the urine were 1021.5 ± 5.4 (1010-1030) and 1006 ± 2.3 (1004-1015), respectively (p < 0.001). The rates of recurrence were 16.6% among those who continued prophylaxis and 100% among those who did not receive prophylaxis (p = 0.001). The most common combination of metabolic anomalies was cystinuria and hypocitraturia (p < 0.001). CONCLUSIONS: This study demonstrated that detailed clinical and laboratory evaluations should be performed for all children with cystine stone disease, and, appropriate prophylactic treatment should be recommended to prevent the reformation of stones.