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Title: Familial focal glomerulosclerosis: a genetic linkage to the HLA locus?
Authors: Goodman, D J;Clarke, B;Hope, R N;Miach, P J;Dawborn, J K
Affiliation: Department of Medicine, Austin Hospital, Heidelberg, Victoria, Australia.
Issue Date: 16-May-1995
Citation: American Journal of Nephrology; 15(5): 442-5
Abstract: The aetiology and pathogenesis of focal glomerulosclerosis is poorly understood and many conflicting reports suggest HLA locus associations in both familial and non-familial glomerulosclerosis. We report a family in which 4 of 5 sisters developed proteinuria, 2 with hypertension and 1 progressing to end-stage renal failure (index case). Three underwent renal biopsy which displayed characteristic features of focal glomerulosclerosis and all shared the HLA alleles HLA-A1, B8, DR3, DR7. The index case received two cadaveric renal transplants from HLA-A1, B8, DR3 donors and developed chronic rejection with no histological evidence of recurrent glomerulonephritis in either kidney. The frequency of this haplotype in the Australian dialysis and transplant population with focal glomerulosclerosis was compared to that seen in the general Australian Caucasian population and was not significantly different suggesting that the presence of the HLA alleles HLA-A1, B8, DR3, DR7 may increase the predisposition to familial glomerulosclerosis but additional factors are required for disease development and progression.
Internal ID Number: 7503147
Type: Journal Article
Subjects: Adolescent
Chromosome Mapping
Family Health
Genetic Linkage
Glomerulosclerosis, Focal Segmental.genetics.immunology
HLA Antigens.genetics
HLA-A1 Antigen.genetics
HLA-B8 Antigen.genetics
HLA-DR3 Antigen.genetics
HLA-DR7 Antigen.genetics
Appears in Collections:Journal articles

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