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The effect of prophylactic treatment with Shohl's solution in children with cystinuria
Volkan Izol1, Ibrahim Atilla Aridogan, Onur Karsli
1Department of Urology, Faculty of Medicine, University of Çukurova, Balcali, 01330 Adana, Turkey.
Insights
Prophylactic treatment with Shohl's solution significantly reduced cystine stone recurrence in children. Patients adhering to treatment had a 16.6% recurrence rate, while non-adherent patients experienced 100% recurrence.
Area of Science:
- Pediatric Nephrology
- Urology
- Medical Chemistry
Background:
- Cystinuria is a rare genetic disorder characterized by cystine stones.
- Recurrent stone formation poses significant challenges in pediatric patients.
- Effective prophylactic strategies are crucial for managing cystine stone disease.
Purpose of the Study:
- To evaluate the efficacy of Shohl's solution in preventing cystine stone recurrence in children.
- To assess the impact of adherence to prophylactic treatment on stone recurrence rates.
- To identify associated metabolic risk factors in pediatric cystinuria patients.
Main Methods:
- A cohort of 17 pediatric patients with cystine stones, post-percutaneous nephrolithotomy (PCNL), received Shohl's solution for alkalinization.
- Patients were monitored for a mean of two years for stone recurrence.
- Urinary pH and specific gravity were measured pre- and post-treatment.
Main Results:
- Adherence to Shohl's solution prophylaxis was associated with a significantly lower recurrence rate (16.6%) compared to non-adherence (100%).
- Treatment with Shohl's solution effectively increased urinary pH and decreased urine specific gravity.
- Cystinuria combined with hypocitraturia was the most frequent metabolic anomaly observed.
Conclusions:
- Comprehensive clinical and laboratory evaluation is essential for children with cystine stone disease.
- Appropriate prophylactic treatment, including alkalinization with Shohl's solution, is vital for preventing stone recurrence.
- Patient adherence to medical prophylaxis is a critical factor in managing pediatric cystinuria.
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.
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