Pediatric Nephrology

, Volume 22, Issue 11, pp 1869–1873 | Cite as

Cystinuria in children and young adults: success of monitoring free-cystine urine levels

  • Luca Dello Strologo
  • Chiara Laurenzi
  • Antonia Legato
  • Anna Pastore
Original Article


Medical treatment of cystinuria is often disappointing. Patients undergo frequent surgery, which is often followed by early relapse. The aim of our study was to evaluate the efficacy of medical treatment of cystinuria, to prevent formation or to reduce the numbers and dimensions of renal stones. Twenty cystinuric patients were treated with a combined approach, including cystine-binding drugs. Free and bound urine cystine levels were measured every 4 months. Drug dosage was adjusted to maintain free urine cystine level below 100 μmol/mmol creatinine. Eighteen patients completed the study; detection of new stones was reduced from 0.28 per year to 0.03 per year, and, in six patients, the numbers and dimensions of pre-existing renal stones were reduced. Surgery was required in one subject, and no relapse was observed 12 months afterwards. The dosage required to achieve target levels was closely correlated with patient body weight: older children required a lower dose. Medical management of cystinuria is feasible. The treatment must be personalised in children, as the amount of drug required is strictly dependent on body size.


Cystinuria Medical treatment Cystine-binding drugs Children Renal stones 



This research was supported by a European grant: project no. 502852, European Genomics Initiative on Disorders of Plasma Membrane Amino Acid Transporters; coordinator: Manuel Palacin.


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Copyright information

© IPNA 2007

Authors and Affiliations

  • Luca Dello Strologo
    • 1
  • Chiara Laurenzi
    • 1
  • Antonia Legato
    • 1
  • Anna Pastore
    • 2
  1. 1.Nephrology and Urology DepartmentBambino Gesù Children’s Hospital and Research InstituteRomeItaly
  2. 2.Clinical Biochemistry UnitBambino Gesù Children’s Research HospitalRomeItaly

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