In:
BJU International, Wiley, Vol. 124, No. 5 ( 2019-11), p. 849-861
Abstract:
To evaluate medical treatments, in terms of adverse events (AEs) and therapeutic goals, in a large series of patients with cystinuria. 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.
Type of Medium:
Online Resource
ISSN:
1464-4096
,
1464-410X
Language:
English
Publisher:
Wiley
Publication Date:
2019
detail.hit.zdb_id:
2019983-1
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