Pediatric cystine calculi in west of Iran a study of 22 cases

Cystinuria is an autosomal recessive disorder which clinically presents as cystine calculi. In this study, we reviewed cystine calculi cases in the west of Iran to determine their common presentations and response to different therapeutic modalities. Between 1999 and 2005, we had 22 pediatric patients [11 boys and 11 girls] with cystine calculi. The demographic characteristics and clinical data of the patients as well as the treatment results were reviewed. The mean age of the patients was 34.20 ' 42.99 months [range, 4 to 156 months]. They were followed for a mean duration of 23 months [range, 3 to 70 months]. Thirteen patients [59.1%] had bilateral and 9 [41%] had unilateral kidney calculi. The sizes of the calculi were between 2 mm and 20 mm. Nine patients [41%] had renal atrophic changes and 1 [4.5%] had obstructive acute renal failure. Hydration and urinary alkalinization were administrated to all of the patients which yielded an excellent result in 54.5% and a poor response in 27.2%. Captopril started for 5 patients was effective only in 1. D-penicillamine had no favorable response. Extracorporeal shockwave lithotripsy was successful in 5 attempts and failed in 4. Surgical interventions were performed for 13 patients [59.1%] and 6 [27.2%] required more than 1 surgical operation. We recommend metabolic workup of childhood urolithiasis and appropriate medical management of its underlying disease. We also recommend minimally invasive urologic techniques including shockwave lithotripsy only when there are clear indications for nonmedical procedures

Urethroplasty for long anterior urethral strictures report of long term results

We reviewed the long-term outcome of substitution urethroplasty with skin flaps for anterior urethral strictures, comparing 1-stage with 2-stage repairs. A total of 45 patients with urethral strictures, 2.5 cm or longer, had undergone skin flap urethroplasty. A 1-stage surgical operation had been done in 10 patients [Orandi and bilateral pedicle island of penile skin] and a 2-stage surgical repair had been performed in 35 using the Johanson technique. They were followed up by retrograde urethrography for 1 to 10 years. The mean age of the patients was 46.3 +/- 17.1 years. Etiology of the stricture was instrumentation in 33.3% of the patients, urethritis in 28.9%, trauma in 13.3%, and unknown in 24.5%. At the first postoperative year, the success rate was 75.6%. This rate was 71.4% and 90% for the 1-stage and 2-stage operations, respectively [P = .23]. There were 1 [10%] and 10 [28.6%] cases of recurrent structure and 1 [10%] and 3 [8.6%] cases of fistula in the patients with the 1-stage and 2-stage operations within the first year, respectively. During the 5 postoperative years, 70% of the patients with the 1-stage repair, 57.1% with the 2-stage repair, and 60% in total had no complications. Based on the previous studies and our experience, we recommend skin flap urethroplasty. However, some measures such as proper tailoring of the flap and the extension of the incision onto the intact segments should be considered for achieving a better outcome