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CYSTINURIA IN A GROUP OF CHILDREN IN IRAQ | ||
| IRAQI JOURNAL OF MEDICAL SCIENCES | ||
| Article 1, Volume 7, Issue 2, 2009, Pages 26-34 | ||
| Author | ||
| Shatha Hussain Ali | ||
| Abstract | ||
| Background: Cystinuria is an autosomal recessive defect in reabsorptive transport of Cystine and dibasic amino acids. Increased urinary excretion of Cystine, the least soluble of all amino acids, results in formation of stones. Objectives: we report our experience with management of cystinuria in a group of Iraqi children. Patients and Methods: from 1999 to 2006, all children with cystinuria were evaluated, treated and followed in Al – Kadhimiya Teaching Hospital. Results: Twenty three patients with cystinuria having calculi (16 males, 7 females) were treated. Their age ranged from 10 months to 18 years. Associated hyperuricosuria was recorded in 30.5%, hypercalciuria in 13% and hyperoxaluria in 4.3%. Follow up period ranged from 1 – 88 months. Nine patients were treated with increased oral fluids and alkalis only. D–Penicillamine therapy was given to 13 patients. Side effect to penicillamine was noticed in 4 patients (22.2%). Captopril was given to 4 patients. Extracorporeal shock wave lithotripsy (ESWL) was performed in 8 patients, and 18 patients underwent open surgical procedures. The stone free rate was 55.6% with fluids and alkali alone, 58.3% with D–Penicillamine, 0% with Captopril and 50% with ESWL. Combined treatments were required in 45% of patients. Stone recurrence rate was 70%. Conclusion: Oral fluids and alkali was most successful when used in patients with mild disease. D–Penicillamine and ESWL had nearly equal rate of successful results. Keywords: cystinuria, chlidren, calculi, urolithiasis | ||
| Keywords | ||
| cystinuria; chlidren; calculi; urolithiasis | ||
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