Adverse events associated with currently used medical treatments for cystinuria and treatment goals: results from a series of 442 patients in France.
Adolescent
Adult
Aged
Child
Child, Preschool
Cystinuria
/ drug therapy
Drug-Related Side Effects and Adverse Reactions
Female
France
Humans
Hydrogen-Ion Concentration
Infant
Male
Middle Aged
Penicillamine
/ adverse effects
Retrospective Studies
Sodium Bicarbonate
/ adverse effects
Tiopronin
/ adverse effects
Treatment Outcome
Urinalysis
Young Adult
D-penicillamine
cystinuria
potassium citrate
sodium bicarbonate
tiopronin
Journal
BJU international
ISSN: 1464-410X
Titre abrégé: BJU Int
Pays: England
ID NLM: 100886721
Informations de publication
Date de publication:
11 2019
11 2019
Historique:
pubmed:
26
2
2019
medline:
2
6
2020
entrez:
26
2
2019
Statut:
ppublish
Résumé
To evaluate medical treatments, in terms of adverse events (AEs) and therapeutic goals, in a large series of patients with cystinuria. 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. 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). 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.
Substances chimiques
Sodium Bicarbonate
8MDF5V39QO
Tiopronin
C5W04GO61S
Penicillamine
GNN1DV99GX
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
849-861Informations de copyright
© 2019 The Authors BJU International © 2019 BJU International Published by John Wiley & Sons Ltd.
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