TY - JOUR
T1 - Angiotensin converting enzyme inhibitor therapy in children with Alport syndrome
T2 - Effect on urinary albumin, TGF-β, and nitrite excretion
AU - Adler, Liora
AU - Mathew, Roy
AU - Futterweit, Stephen
AU - Frank, Rachel
AU - Gauthier, Bernard G.
AU - Kashtan, Clifford E.
AU - Trachtman, Howard
PY - 2002/2/14
Y1 - 2002/2/14
N2 - 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-β, and nitrite excretion. Patients were treated with enalapril, ≃ 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-β, and nitrite excretion. Results: Prior to treatment, urinary excretion of transforming growth factor-β 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-β, or nitrite excretion. Conclusion: 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-β and, therefore, routine treatment with an angiotensin converting enzyme inhibitor may not be warranted.
AB - 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-β, and nitrite excretion. Patients were treated with enalapril, ≃ 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-β, and nitrite excretion. Results: Prior to treatment, urinary excretion of transforming growth factor-β 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-β, or nitrite excretion. Conclusion: 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-β and, therefore, routine treatment with an angiotensin converting enzyme inhibitor may not be warranted.
KW - Alport syndrome
KW - Angiotensin converting enzyme inhibitors
KW - Transforming growth factor-β
KW - Urinary nitrite excretion
UR - http://www.scopus.com/inward/record.url?scp=3342886011&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=3342886011&partnerID=8YFLogxK
U2 - 10.1186/1471-2369-3-2
DO - 10.1186/1471-2369-3-2
M3 - Article
C2 - 11869456
AN - SCOPUS:3342886011
SN - 1471-2369
VL - 3
SP - 1
EP - 5
JO - BMC Nephrology
JF - BMC Nephrology
M1 - 2
ER -