Successful treatment of homozygous cystinuria with captopril

Cystinuria is an autosomal recessive hereditary disorder associated with nephrolithiasis and its attendant complications. Traditional management using oral alkali, D-penicillamine, or mercaptopropionyglycine in an attempt to increase urinary cystine solubility is often unsuccessful due to intolerable side-effects. The aim of this study was to determine, if captopril could reduce urninary cystine excretion in homozygous cystinuric patients. Three cystinuric patients with a history of multiple cystine stones despite previous traditional therapy were treated with 150 mg captopril daily for 3 years after determination of their baseline 24-hour urine cystine excretion. Cystine excretion studies were repeated subsequently at 6-month intervals. The baseline 24-hour urine cystine excretion was within the expected limits for homozygous cystinuria in all patients [1072, 962 and 959 mg cystine per gm creatinine per 24 hours]. After institution of captopril treatment, all patients had a significant decrease in urinary cystine levels [374,313 and 451 mg cystine per gm creatinine per 24 hours]. No patient experienced recurrent nephrolithiasis or adverse drug effects. We conclude that captopril can significantly decrease urinary cystine excretion in patients with homozygous cystinuria. Captopril should be considered an alternative to traditional drug management of cystinuria

[ artificial urinary sphicter in the male - a study of 23 cases]

Objectives: To evaluate our experience with the placement of artificial urinary sphincters and to review the literature about the indications, additional measures required and prognosis of the device

Patients and Methods: This retrospective study was based on 23 male patients who received artificial urinary sphincters of the AMS 800 type at the University Hospital of Dupuytren, Limoges, between April 1996 and April 2005. The mean age of our patients was 70.3 years [range 47 to 77 years]. Eight patients had been treated previously by pelvic radiotherapy for prostate cancer. Sphincter insufficiency occurred following radical prostatectomy in 10, endoscopic resection of a prostatic adenoma in 5, transvesical adenectomy in 4 and endoscopic sphincterotomy with bladder augmentation in 2 patients, as well as following external radiotherapy for the treatment of a localized prostatic adenocarcinoma stage T2a in one and a cystoprostatectomy with Camey II type bladder replacement in the remaining case. Routine investigations included urine analysis, assessment of a possible concomitant inflammation or infection, urethroscopy and a urodynamic work-up. Pelvic floor training was done in all cases, while macroplastique was administered in 15 cases only. Finally, an artificial sphincter was placed in periurethral position in all cases

Results: The functional results of the artificial sphincter were assessed 6 months after activation of the sphincter and were based on the clinical results as well as on the patient's satisfaction. Eighteen out of our 23 patients are completely satisfied, while the 5 remaining patients report minimal urinary leakage. One of them reported a reduction of urinary flow. In 9 cases late complications were noted; three of them could be easily repaired [a technical problem caused by high pressure in the balloon, one minimal vesico-parietal fistula and migration of the pump needing reposition]. The 6 remaining cases suffered from urethral erosion caused by the cuff of the device which incurred within a mean time of 5 years following the placement of the artificial sphincter

Conclusion: The high rate of satisfaction reported by the patients has proved the efficacy of the AMS 800 artificial sphincter in the treatment of urinary incontinence caused by sphincteric insufficiency. Nevertheless, a number of failures is still reported. They may cause severe complications eventually leading to the removal of the device, especially in cases of erosion. For this reason, it is mandatory to respect the indications, to carefully select the patients and to ensure an adequate follow-up to avoid any late complications

[Treatment of ureteral stricture due to tuberculosis by a "Double J"; catheter retrospective study of 12 observations]

Objective: To precise the clinical features and evaluate the accuracy of treatment by JJ endo-prsthesis associated with antituberculous medications and more or less corticoid treatment

Patients and Methods: From January 1992 to December 2001, 12 patients presenting with ureteral strictures due to tuberculosis were treated by JJ catheter for a mean of 12 months in association with antituberculous medication for 8 months and corticoid treatment in 2 cases. The population included 6 males and 6 females aged 20 - 73 years [mean age: 40 years]

Results: The most frequent clinical expressions were pain [66.66%] and haematuria [41.6%]. The stricture was unilateral and unifocal in 4 casers, unilateral and bifocal in 3 cases, multifocal in 3 cases. The controlateral kidney was normal in 9 cases and mastic in 3 cases. The stricture was bilateral in 2 cases, located on the right ureter in 6 cases and on the left side in 4 cases. The outcome was favourable in 9 cases with complete disappearance of the stricture in 4 cases, a partial regression of the stricture and the pyelo-calyceal dilatation in 5 cases, while the condition of the remaining 3 cases deteriorated 6 months after removal of the JJ catheter leading to reconstructive surgery using a Boari flap [1 case], a psoas hitch [1 case] and uretero-pelvic junction plastic surgery [1 case]

Conclusion: JJ endoprosthesis associated with antituberculous medication is proposed as an alternative treatment to classic surgery. A corticoid treatment used early can be helpful to reduce the stricture