D. Castelo, E. Silva, P. Moreira, P. Simões, H. Dinis, A. Figueiredo, A. Mota
Introduction : Patients with cystinuria require multiple and varied treatments due to disease persistence and recurrence. In this study we analyzed therapeutic modalities and outcomes in the treatment of cystine calculi at a single academic center over the last 7 years. Materials and methods : We retrospectively analyzed patients undergoing invasive treatment of cystine calculi at our institution in the period between January 2006 and March 2013. Medical and surgical interventions and patient outcomes regarding stone-free status and renal function were considered. Results : A total of 7 cystinuric patients were invasively treated in our institution over the period of 7 years from January 2006 to March 2013. Of these 4 were male and 3 female with an average age at last follow-up of 39 years (range 19-59). Treatment modalities included medical therapy with high fluid intake, sodium restriction and urine alkalinization (all 7 patients), captopril (6 patients), and penicillamine (1 patient). These patients underwent a total of 156 procedures, with an average of 22.3 procedures per patient. All patients underwent extracorporeal shockwave lithotripsy (SWL), with a mean of 20 sessions per patient, range 8-35). Other treatment modalities included ureterorenoscopy and intracorporeal lithotripsy, (9 procedures in 4 patients, mean = 2, range 1-4), percutaneous nephrolithotomy (PCNL), (3 procedures in 3 patients), pyelolithotomy (2 procedures in 2 patients, one laparoscopic and one open) and one open nephrectomy (sepsis due to an atrophic kidney at presentation). Three patients have been stone free for the last 12 months, all have undergone PCNL or pyelolithotomy. Three patients have had serial renal scintigraphy, all maintained global and differential renal function over a period of 6 years (2 patients) and 2 years (1 patient) despite continued invasive procedures. Conclusions : In our series, more invasive procedures (PCNL or pyelolithotmy) were required for debulking disease burden or achieving a stone-free status. Medical therapy and repeated minimally invasive procedures, especially SWL, were required for preventing recurrence and treating residual stones. Despite the frequent use of procedures with potential deleterious effect for the kidney, in our series renal function was not affected either by the disease or its aggressive treatment over a period of several years. © 2014 Associação Portuguesa de Urologia. Published by Elsevier España, S.L.U. All rights reserved.
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Published on 11/04/17
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