Alport syndrome and thin glomerular basement membrane disease.
CE Kashtan - Journal of the American Society of Nephrology, 1998 - journals.lww.com
Alport syndrome(AS) is a generalized inherited disorder of basement membranes,
manifested by hematuria, progressive nephritis with proteinuria and declining renal function,
senso-rineural deafness, and ocular abnormalities. The natural history of AS is gender-
dependent: affected males typically have severe disease, while the course of AS in females
tends to be mild. The first description of familial hematuria was provided by Guthrie(1) in
1902. Follow-up studies of this family by Hurst (2) and Alport(3) described the progressive …
manifested by hematuria, progressive nephritis with proteinuria and declining renal function,
senso-rineural deafness, and ocular abnormalities. The natural history of AS is gender-
dependent: affected males typically have severe disease, while the course of AS in females
tends to be mild. The first description of familial hematuria was provided by Guthrie(1) in
1902. Follow-up studies of this family by Hurst (2) and Alport(3) described the progressive …