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[Medical treatment of cystine lithiasis in children]
Insights
Medical therapy using alpha-mercaptopropionylglycine (alpha-MPG) or D-penicillamine effectively dissolved bladder cystine stones in children. Preventive therapy showed success, though adherence is key to preventing stone recurrence.
Area of Science:
- Pediatric Nephrology
- Urology
- Medical Chemistry
Context:
- Cystine bladder stones pose a significant challenge in pediatric urology.
- Long-term management often requires medical intervention to prevent stone recurrence or aid dissolution.
Purpose:
- To evaluate the efficacy of alpha-mercaptopropionylglycine (alpha-MPG) and D-penicillamine in dissolving pediatric bladder cystine stones.
- To assess the effectiveness of prophylactic medical therapy in preventing cystine stone recurrence in children.
Summary:
- A 10-year study involved 14 children with bladder cystine stones treated with alpha-MPG or D-penicillamine.
- Lytic therapy achieved total dissolution in 6 of 10 patients, with partial dissolution in one.
- Preventive therapy was successful in 10 of 12 patients, with recurrences linked to medication non-adherence.
Impact:
- Medical therapy offers a satisfactory approach for dissolving bladder cystine stones in children.
- Challenges in long-term adherence to preventive therapy highlight the need for patient education and monitoring to prevent relapses.
Abstract:
Prophylactic measures or dissolution by medical therapy was used to treat bladder cystine stones in 14 children over about a 10-year period. All patients received alpha-MPG (alpha-mercaptopropionylglycine) except one child allergic to alpha-MPG who was treated with DNP (D-penicillamine). In 10 patients the treatment was administered 11 times to dissolve the stones, with resulting total dissolution in 6 cases, partial removal in one case. Two patients are still under treatment and 2 cases failed to respond. Among the 12 patients receiving preventive therapy, only 2 cases were reported as developing recurrences, due in fact to irregular taking of medication. Lytic treatment proceeded without any particular problems, and results were more than satisfactory, but for preventive therapy the difficulty resides in its application and duration when confronted with the serious and frequent danger of a relapse.