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Familial aggregation of primary glomerulonephritis in an Italian population
isolate: Valtrompia study
#MMPMID16528253
Izzi C
; Sanna-Cherchi S
; Prati E
; Belleri R
; Remedio A
; Tardanico R
; Foramitti M
; Guerini S
; Viola BF
; Movilli E
; Beerman I
; Lifton R
; Leone L
; Gharavi A
; Scolari F
Kidney Int
2006[Mar]; 69
(6
): 1033-40
PMID16528253
show ga
Hereditary factors are suspected to contribute to the pathogenesis of sporadic
primary glomerulonephritis, but their contribution is difficult to delineate in
the general population. We studied the prevalence of primary glomerulonephritis
in an isolated population from the extreme northern Valtrompia valley, Northern
Italy. Investigation of medical records, community urinary screening program and
molecular characterization of the population's ancestry were performed;
genealogies of affected individuals were researched. Forty-three patients with
primary glomerulonephritis were identified: 25 had biopsy-proven disease (11
immunoglobulin A (IgA) nephropathy; eight mesangial proliferative
glomerulonephritis without IgA deposits; four focal segmental glomerular
sclerosis; two membranous nephropathy), and 18 had clinical glomerulonephritis.
All 43 patients originated from three mountain villages (Collio, San Colombano,
and Bovegno). In contrast, we found only four cases of primary glomerulonephritis
in two nearby villages (Pezzaze and Tavernole) that shared similar population
histories and lifestyles, demonstrating heterogeneity of risk factors for
glomerulonephritis (P=3 x 10(-5)). All 43 affected individuals could be traced
back to common ancestors (XVI-XVII centuries), enabling the construction of three
large pedigree including three parent-child affected pairs and five affected
siblings pairs. Molecular data showed lower genetic diversity and increased
inbreeding in the Valtrompia population compared to the control population.
Molecular and genealogical evidence of limited set of founders and the absence of
shared nephritogenic environmental factors suggest that our patients share a
common genetic susceptibility to the development of primary glomerulonephritis.
Further molecular study of our families will offer the possibility to shed light
on the genetic background underlying these glomerular disorders.
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