title: Angiotensin converting enzyme inhibitor therapy in children with Alport syndrome: effect on urinary albumin, TGF-beta, and nitrite excretion.
abstract: BACKGROUND
  Angiotensin converting enzyme inhibitors are routinely prescribed to patients with chronic kidney disease because of their known renoprotective effects. We evaluated the effect of short-term therapy with the angiotensin converting enzyme inhibitor, enalapril, in early Alport syndrome, defined as disease duration less than 10 years and a normal glomerular filtration rate.
  
  
  METHODS
  11 children with early Alport syndrome were investigated. Two consecutive early morning urine specimens were collected at the start of the study for measurement of urinary creatinine, total protein, albumin, TGF-beta, and nitrite excretion. Patients were treated with enalapril, congruent with 0.2 mg/kg/day, once a day for 14 days. Two early morning urine specimens were collected on days 13 and 14 of enalapril treatment and two weeks later for measurement of urinary creatinine, total protein, albumin, TGF-beta, and nitrite excretion.
  
  
  RESULTS
  Prior to treatment, urinary excretion of transforming growth factor-beta and nitrite, the major metabolite of nitric oxide, was within normal limits in all patients. Administration of enalapril for 2 weeks did not alter urinary albumin, transforming growth factor-beta, or nitrite excretion.
  
  
  CONCLUSIONS
  These findings suggest that early Alport syndrome represents a disease involving exclusively intrinsic glomerular barrier dysfunction. At this stage of the illness, there is no evidence of angiotensin II-mediated proteinuria or increased production of transforming growth factor-beta and, therefore, routine treatment with an angiotensin converting enzyme inhibitor may not be warranted.
authors.affiliation: Department of Pediatrics, Division of Nephrology, Schneider Children's Hospital of the North Shore-Long Island Jewish Health System, Long Island Campus for the Albert Einstein College of Medicine, New Hyde Park, NY, USA. lweinste@aecom.yu.edu
authors.forename: Liora
authors.initials: L
authors.lastname: Adler
authors.forename: Roy
authors.initials: R
authors.lastname: Mathew
authors.forename: Stephen
authors.initials: S
authors.lastname: Futterweit
authors.forename: Rachel
authors.initials: R
authors.lastname: Frank
authors.forename: Bernard G
authors.initials: BG
authors.lastname: Gauthier
authors.forename: Clifford E
authors.initials: CE
authors.lastname: Kashtan
authors.forename: Howard
authors.initials: H
authors.lastname: Trachtman
mesh_terms: D000419:Albuminuria / Q000175:diagnosis* / Q000188:drug therapy; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D015415:Biomarkers / Q000652:urine; D002648:Child; D003404:Creatinine / Q000652:urine*; D004656:Enalapril / Q000627:therapeutic use*; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D008297:Male; D009394:Nephritis, Hereditary / Q000188:drug therapy* / Q000652:urine; D009573:Nitrites / Q000652:urine*; D016212:Transforming Growth Factor beta / Q000652:urine*
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D015415:Biomarkers; D009573:Nitrites; D016212:Transforming Growth Factor beta; D004656:Enalapril; D003404:Creatinine