Background Angiotensin-converting enzyme inhibitors (ACEis) have evolved as a first-line therapy for delaying end-stage renal failure (ESRF) in Alport syndrome (AS). The present study tested the hypothesis of a superior nephroprotective potential of an early ACEi intervention, examining a cohort with the COL4A5 missense variant p.(Gly624Asp). Methods In this observational cohort study (NCT02378805), 114 individuals with the identical gene variant were explored for age at ESRF and life expectancy in correlation with treatment as endpoints. Results All 13 untreated hemizygous patients developed ESRF (mean age 48.9 +/- 13.7 years), as did 3 very late treated hemizygotes (51.7 +/- 4.2 years), with a mean life expectancy of 59.2 +/- 9.6 years. A...
Das Alport Syndrom ist eine hereditäre Kollagen-Typ-IV-Erkrankung, die in ihrem Vollbild ...
Background: A progressive trajectory toward renal failure is common in patients with Alport syndrome...
Purpose : Alport syndrome is clinically characterized by hereditary progressive nephritis causing ES...
Background Angiotensin-converting enzyme inhibitors (ACEis) have evolved as a first-line therapy for...
BACKGROUND: Angiotensin-converting enzyme inhibitors (ACEis) have evolved as a first-line therapy fo...
Alport syndrome inevitably leads to end-stage renal disease and there are no therapies known to impr...
Alport syndrome inevitably leads to end-stage renal disease and there are no therapies known to impr...
The Alport syndrome (AS) is a hereditary chronic kidney disease which is characterized by ultrastruc...
Alport syndrome (AS) is a clinically and genetically heterogeneous disorder with a wide phenotypic s...
Alport Syndrome (AS) is a hereditary disease that leads to a progressive loss of glomerular basement...
Alport syndrome is a hereditary kidney disease which typically has a progressive course (Alport 1927...
Alport syndrome (AS) is a hereditary glomerulopathy due to abnormal composition of the glomerular ba...
Background Alport syndrome (AS) is an inherited glomerular disease caused by mutations in COL4A3, CO...
BACKGROUND: Alport syndrome (AS) is an inherited glomerular disease caused by mutations in COL4A3, C...
Background. Alport syndrome (AS) is characterized by progressive kidney disease. There is increasing...
Das Alport Syndrom ist eine hereditäre Kollagen-Typ-IV-Erkrankung, die in ihrem Vollbild ...
Background: A progressive trajectory toward renal failure is common in patients with Alport syndrome...
Purpose : Alport syndrome is clinically characterized by hereditary progressive nephritis causing ES...
Background Angiotensin-converting enzyme inhibitors (ACEis) have evolved as a first-line therapy for...
BACKGROUND: Angiotensin-converting enzyme inhibitors (ACEis) have evolved as a first-line therapy fo...
Alport syndrome inevitably leads to end-stage renal disease and there are no therapies known to impr...
Alport syndrome inevitably leads to end-stage renal disease and there are no therapies known to impr...
The Alport syndrome (AS) is a hereditary chronic kidney disease which is characterized by ultrastruc...
Alport syndrome (AS) is a clinically and genetically heterogeneous disorder with a wide phenotypic s...
Alport Syndrome (AS) is a hereditary disease that leads to a progressive loss of glomerular basement...
Alport syndrome is a hereditary kidney disease which typically has a progressive course (Alport 1927...
Alport syndrome (AS) is a hereditary glomerulopathy due to abnormal composition of the glomerular ba...
Background Alport syndrome (AS) is an inherited glomerular disease caused by mutations in COL4A3, CO...
BACKGROUND: Alport syndrome (AS) is an inherited glomerular disease caused by mutations in COL4A3, C...
Background. Alport syndrome (AS) is characterized by progressive kidney disease. There is increasing...
Das Alport Syndrom ist eine hereditäre Kollagen-Typ-IV-Erkrankung, die in ihrem Vollbild ...
Background: A progressive trajectory toward renal failure is common in patients with Alport syndrome...
Purpose : Alport syndrome is clinically characterized by hereditary progressive nephritis causing ES...