The Long-Term Follow-Up of Patients with Cystine Stones: A Single-Center Experience for 13 Years

Abstract

Introduction: Cystine stone development is relatively uncommon among patients with urolithiasis, and most studies have reported only on small sample sizes and short follow-up periods. We evaluated clinical courses and treatment outcomes of patients with cystine stones with long-term follow-up at our center. Methods: We retrospectively analyzed 22 patients diagnosed with cystine stones between January 1989 and May 2019. Results: The median follow-up was 160 (range 6–340) months, and the median patient age at diagnosis was 46 (range 12–82) years. All patients underwent surgical interventions at the first visit (4 extracorporeal shockwave lithotripsy, 5 ureteroscopy, and 13 percutaneous nephrolithotripsy). The median number of stone events and surgical interventions per year was 0.45 (range 0–2.6) and 0.19 (range 0–1.3) after initial surgical intervention. The median time to stone events and surgical intervention was 2 years and 3.25 years, respectively. There was a significant difference in time to stone events and second surgical intervention when patients were divided at 50 years of age at diagnosis (p = 0.02, 0.04, respectively). Conclusions: Only age at a diagnosis under 50 was significantly associated with recurrent stone events and intervention. Adequate follow-up and treatment are needed to manage patients with cystine stones safely.

Pediatric cystine stone successfully treated by mini‐percutaneous nephrolithotripsy and antegrade ureteroscopy

“…Primary intervention was four SWL cases, five URS cases, and 13 PNL cases but the most patients were adults (median age: 46 years, range: 12-82 years). 5 Inoue et al reported about bilateral cystine stones of 2-year-old boy successfully treated by super ultra-mini endoscopic renal surgery (sheath 8.5/9.5Fr). 6 Currently, miniaturized nephroscopy and URS, improvement of laser technology and fine retrieval instruments have revolutionized minimally invasive surgery in children.…”

“…reported about 22 cases of long‐term follow‐up (median 160 months). Primary intervention was four SWL cases, five URS cases, and 13 PNL cases but the most patients were adults (median age: 46 years, range: 12–82 years) 5 . Inoue et al .…”

“…Primary intervention was four SWL cases, five URS cases, and 13 PNL cases but the most patients were adults (median age: 46 years, range: 12–82 years). 5 Inoue et al . reported about bilateral cystine stones of 2‐year‐old boy successfully treated by super ultra‐mini endoscopic renal surgery (sheath 8.5/9.5Fr).…”

Exploring the Contribution of the Transporter AGT1/rBAT in Cystinuria Progression: Insights from Mouse Models and a Retrospective Cohort Study

“…Thus, the number of new stone occurrences, spontaneous stone expulsions, renal colic, hematuria due to lithiasis, acute pyelonephritis, hospital admissions due to lithiasis, and surgical intervention to remove the lithiasis were grouped into a single numerical variable called lithiasis events. The number of lithiasis events for each patient was normalized by years of follow-up, as other authors have proceeded [31,32]. Urinary excretion of cystine, lysine, ornithine, and arginine, and where possible, aspartate and glutamate were also acquired.…”

Cystine Stones: Developments in Minimally Invasive Surgery and Their Impact on Morbidity and Stone Clearance

“…Cystine stones commonly occur bilaterally in 67-73% of cases. 29,30 Bilateral same-sitting ureteroscopy can be undertaken in the management of bilateral urolithiasis, and this is associated with an initial stone-free rate of 87%. 45 Complication rates reported were 50%, although three-quarters of these were Clavien 1 complications.…”