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• W2069236722 abstract "No AccessJournal of UrologyCLINICAL UROLOGY: Original Articles1 Feb 2002METABOLIC RISK FACTORS FOR STONE FORMATION IN PATIENTS WITH CYSTIC FIBROSIS MARCOS R. PEREZ-BRAYFIELD, DANIEL CAPLAN, JOHN M. GATTI, EDWIN A. SMITH, and ANDREW J. KIRSCH MARCOS R. PEREZ-BRAYFIELDMARCOS R. PEREZ-BRAYFIELD More articles by this author , DANIEL CAPLANDANIEL CAPLAN More articles by this author , JOHN M. GATTIJOHN M. GATTI More articles by this author , EDWIN A. SMITHEDWIN A. SMITH More articles by this author , and ANDREW J. KIRSCHANDREW J. KIRSCH More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(01)69068-2AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract Purpose: Cystic fibrosis is characterized by chronic pulmonary disease, insufficient pancreatic and digestive function, and abnormal sweat concentrations. Patients with cystic fibrosis also have an increased incidence of nephrolithiasis. We compared the results of metabolic evaluation in patients with cystic fibrosis with and without nephrolithiasis. Materials and Methods: A total of 496 patients were evaluated at our center, including 98 with a mean age of 25 years who had cystic fibrosis and complete metabolic evaluation available between 1996 and 2000. Of these patients 13 (13%) had a history of nephrolithiasis. The records were reviewed for clinical characteristics and all patients underwent metabolic evaluation, including serum electrolyte measurement and 24-hour urine collection. Statistical analysis was done to compare the stone versus nonstone groups. Results: The incidence of nephrolithiasis in our study was 3%. We identified 13 patients 16 to 41 years old (mean age 27) with nephrolithiasis, of whom 62% had had multiple episodes. Flank pain was the presenting symptom in 9 of the 13 cases (69%). Renal ultrasound and computerized tomography were the most common imaging modalities. In 9 cases stones were passed without intervention, extracorporeal shock wave lithotripsy was required in 2 and ureteroscopy with stone extraction was done in 2. Calcium oxalate was the dominant stone composition in the 9 patients in whom stone analysis was performed. Metabolic evaluation of the stone versus no stone groups showed elevated urinary oxalate (45.5 versus 42.5 mg./24 hours), relative calcium oxalate supersaturation (5.3 versus 7.2) and decreased urinary citrate in the 2 groups. There was a statistical difference in citrate excretion with lower levels in stone formers (102 versus 218 mg./24 hours, p = 0.0007). Conclusions: Patients with cystic fibrosis have an increased incidence of nephrolithiasis and are at high risk for recurrence. Metabolic evaluation is indicated in all patients with cystic fibrosis and urolithiasis since most have hyperoxaluria, urinary calcium oxalate supersaturation and decreased urinary citrate. Correcting detected stone risk factors in these cases may decrease stone recurrence. References 1 : Treatment of cystic fibrosis in adults. Am Fam Physician1996; 54: 1291. Google Scholar 2 : Carrier screening for cystic fibrosis: a perinatal perspective. J Perinatal Nurs1999; 13: 14. Google Scholar 3 : Cystic fibrosis: current survival and population estimates to the year 2000. Thorax1991; 46: 881. Google Scholar 4 : Urolithiasis and cystic fibrosis. J Urol1996; 155: 1563. Link, Google Scholar 5 Clinical Practice Guidelines for Cystic Fibrosis. Bethesda, Maryland: Cystic Fibrosis Foundation1997. Google Scholar 6 : Congenital absence of the vas deferens. The fertilizing capacity of human epididymal sperm. N Engl J Med1990; 323: 1788. Google Scholar 7 : Generation of camp-activated chloride currents by expression of CFTR. Science1991; 251: 679. Google Scholar 8 : Microscopic nephrocalcinosis in cystic fibrosis. N Engl J Med1988; 219: 263. Google Scholar 9 : Pathogenesis of nephrolithiasis post-partial ileal bypass surgery: case control study. Kidney Int1991; 39: 1249. Google Scholar 10 : Urinary excretion substances in patients with cystic fibrosis: risk of urolithiasis?. Pediatr Nephrol1998; 12: 275. Google Scholar 11 : Absence of Oxalobacter formigenes in cystic fibrosis patients: a risk factor for hyperoxaluria. Lancet1998; 352: 1026. Google Scholar 12 : Is there a risk for kidney stone formation in cystic fibrosis?. Helv Paediatr Acta1982; 37: 267. Google Scholar 13 : Hypocitraturia in patients with gastrointestinal malabsorption. N Engl Med1980; 303: 657. Google Scholar From the Division of Pediatric Urology, Department of Urology and Cystic Fibrosis Center, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia© 2002 by American Urological Association, Inc.FiguresReferencesRelatedDetailsCited byAssimos D (2018) Re: Loss of Cystic Fibrosis Transmembrane Regulator Impairs Intestinal Oxalate SecretionJournal of Urology, VOL. 196, NO. 5, (1586-1587), Online publication date: 1-Nov-2016. Volume 167Issue 2 Part 1February 2002Page: 480-484 Advertisement Copyright & Permissions© 2002 by American Urological Association, Inc.Keywordscystic fibrosiskidneykidney calculicitric acidMetricsAuthor Information MARCOS R. PEREZ-BRAYFIELD More articles by this author DANIEL CAPLAN More articles by this author JOHN M. GATTI More articles by this author EDWIN A. SMITH More articles by this author ANDREW J. KIRSCH More articles by this author Expand All Advertisement PDF downloadLoading ..." @default.
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