CKD and Its Risk Factors among Patients with Cystinuria

Caroline Prot-Bertoye 1 Saïd Lebbah 2, 3 Michel Daudon 4 Isabelle Tostivint 5 Pierre Bataille 6 Franck Bridoux 7 Pierre Brignon 8 Christian Choquenet 9 Pierre Cochat Christian Combe 10 Pierre Conort 11 Stéphane Decramer 12 Bertrand Doré 13 Bertrand Dussol 14 Marie Essig 15 Nicolas Gaunez 13 Dominique Joly 16, 17 Sophie Le Toquin-Bernard 18 Arnaud Méjean 19 Paul Meria 20 Denis Morin 21, 22 Hung Viet N'Guyen 20 Christian Noël 23 Michel Normand Michel Pietak 4 Pierre Ronco 24 Christian Saussine 25 Michel Tsimaratos 26 Gérard Friedlander 27, 2, 3 Olivier Traxer 28 Bertrand Knebelmann 29 Marie Courbebaisse 19
Abstract : BACKGROUND AND OBJECTIVES: Cystinuria is an autosomal recessive disorder affecting renal cystine reabsorption; it causes 1% and 8% of stones in adults and children, respectively. This study aimed to determine epidemiologic and clinical characteristics as well as comorbidities among cystinuric patients, focusing on CKD and high BP. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This retrospective study was conducted in France, and involved 47 adult and pediatric nephrology and urology centers from April 2010 to January 2012. Data were collected from 442 cystinuric patients. RESULTS: Median age at onset of symptoms was 16.7 (minimum to maximum, 0.3-72.1) years and median diagnosis delay was 1.3 (0-45.7) years. Urinary alkalinization and cystine-binding thiol were prescribed for 88.8% and 52.2% of patients, respectively, and 81.8% had at least one urological procedure. Five patients (1.1%, n=4 men) had to be treated by dialysis at a median age of 35.0 years (11.8-70.7). Among the 314 patients aged ≥16 years, using the last available plasma creatinine, 22.5% had an eGFR≥90 ml/min per 1.73 m(2) (calculated by the Modification of Diet in Renal Disease equation), whereas 50.6%, 15.6%, 7.6%, 2.9%, and 0.6% had an eGFR of 60-89, 45-59, 30-44, 15-29, and <15, respectively. Among these 314 patients, 28.6% had high BP. In multivariate analysis, CKD was associated with age (odds ratio, 1.05 [95% confidence interval, 1.03 to 1.07]; P<0.001), hypertension (3.30 [1.54 to 7.10]; P=0.002), and severe damage of renal parenchyma defined as a past history of partial or total nephrectomy, a solitary congenital kidney, or at least one kidney with a size <10 cm in patients aged ≥16 years (4.39 [2.00 to 9.62]; P<0.001), whereas hypertension was associated with age (1.06 [1.04 to 1.08]; P<0.001), male sex (2.3 [1.3 to 4.1]; P=0.003), and an eGFR<60 ml/min per 1.73 m(2) (2.7 [1.5 to 5.1]; P=0.001). CONCLUSIONS: CKD and high BP occur frequently in patients with cystinuria and should be routinely screened.
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Soumis le : mercredi 27 mars 2019 - 13:27:27
Dernière modification le : mardi 28 mai 2019 - 14:12:02

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Caroline Prot-Bertoye, Saïd Lebbah, Michel Daudon, Isabelle Tostivint, Pierre Bataille, et al.. CKD and Its Risk Factors among Patients with Cystinuria. Clinical Journal of the American Society of Nephrology, American Society of Nephrology, 2015, 10 (5), pp.842-851. ⟨10.2215/CJN.06680714⟩. ⟨hal-02081201⟩

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