Korean J Urol.  2014 Aug;55(8):515-519. 10.4111/kju.2014.55.8.515.

Multimodal Treatments of Cystine Stones: An Observational, Retrospective Single-Center Analysis of 14 Cases

• Shim M
• Park HK

• KMID: 1794585
• DOI: http://doi.org/10.4111/kju.2014.55.8.515

Abstract

PURPOSE
To document the experiences of a single institution in evaluating the clinical courses and treatment outcomes of patients with cystine stones.
MATERIALS AND METHODS
The clinical data of 14 patients with cystine stones who were treated at our institution from March 1994 to July 2012 were reviewed. These data included age at first visit, gender, family history, body mass index, presence of a single kidney, stone locations, stone burden, routine urinalysis, and culture. In addition, we also analyzed data on surgery, shock wave lithotripsy, medical treatment, stone recurrence or regrowth, and overall treatment success rates.
RESULTS
The mean age of our patients at their first visit was 19.6+/-5.0 years, and eight patients were males. The median stone burden and mean urine pH before each surgery were 6.5 cm2 and 6.5+/-0.9, respectively. Two patients had a family history of cystine stones. Patients underwent surgery an average of 2.7 times. The median interval between surgeries was 27.3 months, and 1 open surgery, 12 percutaneous nephrolithotomies, and 25 ureterorenoscopies were performed. Potassium citrate or sodium bicarbonate was used in nine cases. D-Penicillamine was continuously used in three patients. Patients had an average incidence of 3.2 recurrences or regrowth of stones during the median follow-up period of 60.5 months.
CONCLUSIONS
Patients with cystine stones have high recurrence or regrowth rates and relatively large stone burdens. Adequate treatment schedules must therefore be established in these cases to prevent possible deterioration of renal function.

Keyword

Calculi; Cystinuria; Nephrolithiasis; Urolithiasis

MeSH Terms

Adolescent
Adult
Child
Combined Modality Therapy
Cystine/*analysis
Cystinuria/complications
Female
Humans
Hydrogen-Ion Concentration
Kidney Calculi/chemistry/pathology/therapy
Lithotripsy/methods
Male
Nephrostomy, Percutaneous/methods
Recurrence
Reoperation
Retrospective Studies
Treatment Outcome
Ureteral Calculi/chemistry/pathology/therapy
Urinary Calculi/chemistry/etiology/pathology/*therapy
Young Adult
Cystine

Reference

1. Saravakos P, Kokkinou V, Giannatos E. Cystinuria: current diagnosis and management. Urology. 2014; 83:693–699. PMID: 24246330.

2. Claes DJ, Jackson E. Cystinuria: mechanisms and management. Pediatr Nephrol. 2012; 27:2031–2038. PMID: 22281707.

3. Knoll T, Zollner A, Wendt-Nordahl G, Michel MS, Alken P. Cystinuria in childhood and adolescence: recommendations for diagnosis, treatment, and follow-up. Pediatr Nephrol. 2005; 20:19–24. PMID: 15602663.

4. Leusmann DB, Blaschke R, Schmandt W. Results of 5,035 stone analyses: a contribution to epidemiology of urinary stone disease. Scand J Urol Nephrol. 1990; 24:205–210. PMID: 2237297.

5. Pahira JJ. Management of the patient with cystinuria. Urol Clin North Am. 1987; 14:339–346. PMID: 3576855.

6. Rogers A, Kalakish S, Desai RA, Assimos DG. Management of cystinuria. Urol Clin North Am. 2007; 34:347–362. PMID: 17678985.

7. Dello Strologo L, Pras E, Pontesilli C, Beccia E, Ricci-Barbini V, de Sanctis L, et al. Comparison between SLC3A1 and SLC7A9 cystinuria patients and carriers: a need for a new classification. J Am Soc Nephrol. 2002; 13:2547–2553. PMID: 12239244.

8. Ozalp I, Coskun T, Tokol S, Demircin G, Monch E. Inherited metabolic disorders in Turkey. J Inherit Metab Dis. 1990; 13:732–738. PMID: 2246857.

9. Tanzer F, Ozgur A, Bardakci F. Type I cystinuria and its genetic basis in a population of Turkish school children. Int J Urol. 2007; 14:914–917. PMID: 17880288.

10. Ito H, Murakami M, Miyauchi T, Mori I, Yamaguchi K, Usui T, et al. The incidence of cystinuria in Japan. J Urol. 1983; 129:1012–1014. PMID: 6406687.

11. Rosenberg LE, Downing S, Durant JL, Segal S. Cystinuria: biochemical evidence for three genetically distinct diseases. J Clin Invest. 1966; 45:365–371. PMID: 5904553.

12. Mattoo A, Goldfarb DS. Cystinuria. Semin Nephrol. 2008; 28:181–191. PMID: 18359399.

13. Ahmed K, Khan MS, Thomas K, Challacombe B, Bultitude M, Glass J, et al. Management of cystinuric patients: an observational, retrospective, single-centre analysis. Urol Int. 2008; 80:141–144. PMID: 18362482.

14. Onal B, Dogan C, Citgez S, Argun B, Onder AU, Sever L, et al. Percutaneous nephrolithotomy in children with cystine stone: long-term outcomes from a single institution. J Urol. 2013; 190:234–237. PMID: 23313363.

15. Ruggera L, Zanin M, Beltrami P, Zattoni F. Retrograde transureteral approach: a safe and efficient treatment for recurrent cystine renal stones. Urol Res. 2011; 39:411–415. PMID: 21193905.

16. Ahmed K, Dasgupta P, Khan MS. Cystine calculi: challenging group of stones. Postgrad Med J. 2006; 82:799–801. PMID: 17148700.