Adverse events associated with currently used medical treatments for cystinuria and treatment goals: results from a series of 442 patients in France.
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Prot-Bertoye Caroline, Lebbah Saïd, Daudon Michel, Tostivint Isabelle, Jais Jean-Philippe, Lillo-Le Louët Agnés, Pontoizeau Clément, Cochat Pierre, Bataille Pierre, Bridoux Franck, Brignon Pierre, Choquenet Christian, Combe Christian, Conort Pierre, Decramer Stéphane, Doré Bertrand, Dussol Bertrand, Essig Marie, Frimat Marie, Gaunez Nicolas, Joly Dominique, Le Toquin-Bernard Sophie, Méjean Arnaud, Meria Paul, Morin Denis, N'Guyen Hung V, Normand Michel, Pietak Michel, Ronco Pierre, Saussine Christian, Tsimaratos Michel, Friedlander Gérard, Traxer Olivier, Knebelmann Bertrand, Courbebaisse Marie, French Cystinuria Group
Аннотация
PATIENTS AND METHODS: Data from 442 patients with cystinuria were recorded retrospectively. Crystalluria was studied in 89 patients. A mixed-effects logistic regression model was used to estimate how urine pH, specific gravity and cysteine-binding thiols (CBT) correlate with risk of cystine crystalluria.
RESULTS: Alkalizing agents and CBT agents were given to 88.8% (n = 381) and 55.3% (n = 238) of patients, respectively. Gastrointestinal AEs were reported in 12.3%, 10.4% and 2.6% of patients treated with potassium bicarbonate, potassium citrate and sodium bicarbonate, respectively (P = 0.008). The percentages of patients who experienced at least one AE with tiopronin (24.6%) and with D-penicillamine (29.5%) were similar (P = 0.45). Increasing urine pH and decreasing urine specific gravity significantly reduced the risk of cystine crystalluria, whereas D-penicillamine and tiopronin treatments did not reduce this risk (odds ratio [OR] 1 for pH ≤6.5; OR 0.52 [95% confidence interval {95% CI} 0.28-0.95] for 7.0 <pH ≤7.5, P = 0.03; OR 0.26 [95% CI 0.13-0.53] for 7.5 < pH ≤8.0, P <0.001; OR 1 for specific gravity ≤1.005 OR 5.76 [95% CI 1.45-22.85] for 1.006 ≤ specific gravity ≤1.010, P = 0.01; and OR 11.06 [95% CI 2.76-44.26] for 1.011 ≤ specific gravity ≤ 1.014, P < 0.001). Increased urine pH significantly increased the risk of calcium phosphate crystalluria (OR 1 for pH≤ 6.5; OR 6.09 [95% CI 2.15-17.25] for pH >8.0, P <0.001).
CONCLUSION: Adverse events were frequent with D-penicillamine and tiopronin. Alkaline hyperdiuresis was well tolerated and reduced cystine crystalluria. Urine specific gravity ≤1.005 and urine pH >7.5, while warning about calcium-phosphate crystallization, should be the goals of medical therapy.