LONG-TERM FOLLOW-UP OF 2 CYSTINURIC BROTH ERS - CASE-REPORT AND LITERATURE-REVIEW

Long-term follow-up (20 respective 13 years) of two brothers with cyst inuria is reported. The two children presented 1970 respective 1979 wi th complete staghorn calculi. Both patients underwent a pyelolithotomy . In both patients, stone analysis indicated calculi composed of subst ances other than cystine. Although adequate prophylaxis with hydration , alkalization, vitamin C und alpha-Mercaptopropionylglycine was start ed in both patients, calculi formations were recurrent, treated by ext racorporeal shock wave lithotripsy. Stones composed of 100% cystine we re rare. Often calculi were composed of a heterogenous mixture of cyst ine and uric acid. Over the whole period in both patients uric acid co ncentration in serum was normal.