Familial membranoproliferative glomerulonephritis.
Four and two male sibs of two separate families who had biopsy-proven membranoproliferative glomerulonephritis (MPGN) are presented. In the first family four sibs of the first-degree consanguineous marriage showed the clinical picture of nephrotic syndrome without hypocomplementaemia at initial laboratory findings. In the second family two affected sibs showed nephrotic and nephritic syndromes on admission. Family investigations showed normal serum complement, immunoglobulins, T-cell subsets, urine analysis, and serum biochemistry. HLA typing in the two families revealed a common antigen HLA A2 in all affected sibs. Some other reports give suggestive evidence of MPGN in siblings but this is the first report that showed the occurrence of MPGN in four sibs. Our data strengthened the concept that genetic factors are involved in the development of MPGN but additional immunogenetic studies will shed light on the genetic aspects of the disease.
Bakkaloglu
A
A
Pediatric Nephrology Department, Hacettepe University, Children's Hospital, Ankara, Turkey.
Söylemezoglu
O
O
Tinaztepe
K
K
Saatçi
U
U
Söylemezoglu
F
F
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003165:Complement System Proteins / Q000276:immunology; D003241:Consanguinity; D015432:Glomerulonephritis, Membranoproliferative / Q000235:genetics* / Q000276:immunology / Q000473:pathology; D015789:HLA-A2 Antigen / Q000276:immunology; D006801:Humans; D007223:Infant; D007668:Kidney / Q000276:immunology; D007677:Kidney Function Tests; D008297:Male; D009404:Nephrotic Syndrome / Q000473:pathology; D010375:Pedigree; D016176:T-Lymphocyte Subsets / Q000276:immunology
D015789:HLA-A2 Antigen; D003165:Complement System Proteins