Renin-angiotensin axis blockade reduces proteinuria in presymptomatic patients with familial FSGS.
Familial and genetic forms of focal segmental glomerulosclerosis (FSGS) are associated with six different mutations in genes affecting the podocyte (NPHS2, ACTN4, CD2AP, WT1, TRPC6, and PLCE1). Immunosuppressive agents are often unsuccessful in treating this condition. Data regarding the efficacy of renoprotection through blockage of the renin-angiotensin axis is lacking. We describe three children from two different families with familial FSGS in whom partial to complete remission of proteinuria was attained through early blockade of the renin-angiotensin axis. In addition, there was no deterioration of renal function. We speculate that presymptomatic patients with normal renal function who have genetic or familial FSGS may benefit from early blockade of the renin-angiotensin axis and that this may also prevent progressive renal disease.
Copelovitch
Lawrence
L
Department of Pediatrics, Division of Nephrology, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Guttenberg
Marta
M
Pollak
Martin R
MR
Kaplan
Bernard S
BS
D000293:Adolescent; D002648:Child; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology*; D006417:Hematuria / Q000175:diagnosis; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D011507:Proteinuria / Q000175:diagnosis; D012084:Renin-Angiotensin System
D007166:Immunosuppressive Agents