Traitement médical de la cystinurie: évaluation des résultats à long terme chez 30 patients [Medical treatment of cystinuria: evaluation of long-term results in 30 patients].

Details

Serval ID
serval:BIB_70320BF6A584
Type
Article: article from journal or magazin.
Collection
Publications
Title
Traitement médical de la cystinurie: évaluation des résultats à long terme chez 30 patients [Medical treatment of cystinuria: evaluation of long-term results in 30 patients].
Journal
Presse Médicale
Author(s)
Barbey F., Joly D., Rieu P., N'Guessau K., Daudon M., Jungers P.
ISSN
0755-4982 (Print)
ISSN-L
0755-4982
Publication state
Published
Issued date
2000
Peer-reviewed
Oui
Volume
29
Number
10
Pages
528-532
Language
french
Notes
Publication types: English Abstract ; Journal Article ; Research Support, Non-U.S. Gov't Publication Status: ppublish
Abstract
OBJECTIVE: Because urinary hyper-excretion of cystine is permanent in homozygous cystinuric patients, stone recurrence is frequent and may alter renal function. Identification of factors predictive of success of medical treatment (no further urological procedure required) is therefore needed to improve patient management.
PATIENTS AND METHODS: Thirty adult patients with homozygous cystinuria and urolithiasis were referred to the nephrology department of the Necker Hospital from 1963 to 1999, with a mean follow-up of medical therapy of 10.5 +/- 8.4 years. The basal treatment schedule was hyperdiuresis and alkalinization with thiol derivative (D-penicillamine or tiopronine) added when needed.
RESULTS: Overall incidence of urological procedures decreased from 0.33/pt-year in the pretherapeutic period to 0.15 on treatment (p < 0.01), a 55% reduction. Sixteen patients (53%) did not require any urological procedure during follow-up. The only significant difference between those patients and the other 14 in whom medical therapy failed was the daily urine volume (3.2 l/day in the former compared with 2.4 l/day in the latter, p < 0.001).
CONCLUSION: Regular medical therapy was able to stop stone disease activity in the long term in more than half of the patients. Sustained hyperdiuresis, with a daily urine volume > 3 liters, appears as a major factor of therapeutic success, even in patients treated with thiols.
Keywords
Adolescent, Adult, Age of Onset, Analysis of Variance, Biological Markers/blood, Case-Control Studies, Creatinine/blood, Cystinuria/complications, Cystinuria/physiopathology, Diuresis, Female, Follow-Up Studies, Humans, Kidney Calculi/etiology, Kidney Calculi/physiopathology, Male, Middle Aged, Penicillamine/therapeutic use, Sulfhydryl Reagents/therapeutic use
Pubmed
Web of science
Create date
09/02/2012 14:23
Last modification date
20/08/2019 14:28
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