Familial IgA Nephropathy

Abstract

The evaluation of familial glomerulonephritis in patients with IgA nephropathy who were from central and eastern Kentucky resulted in the discovery of potentially related pedigrees containing 14 patients. An additional 17 members of the pedigrees had clinical glomerulonephritis, and 6 had "chronic nephritis" noted on their death certificates. Six patients with IgA nephropathy had a common ancestor. In addition, both parents of six patients with the disease came from families with other cases of IgA nephropathy. No single HLA haplotype or antigen was found in all the patients with IgA nephropathy. Our data on these pedigrees strongly support an inherited mechanism in the pathogenesis of IgA nephropathy in some patients.

Dentatorubropallidoluysian Atrophy with Chronic Renal Failure in a Japanese Family

“…The proband suffered from renal failure and became dialysis-dependent at the age of 59, 4 years after the onset of chronic nephrotic syndrome [4]. Hereditary renal disorders include autosomal dominant polycystic kidney disease [6], familial IgA nephropathy [7], familial hematuria [8], and Alport’s syndrome [9]. The clinical symptoms and laboratory data showed that our patients had none of the above disorders, indicating that the renal failure in our DRPLA patients is not associated with known familial renal diseases.…”

IgA Nephropathy in Children

“…Immunologic studies in the families of patients have revealed abnormalities of the immune response similar to those found in affected patients. Significant associations between the incidence of IgA nephropathy and some class I (HLA-B12, B35) and II (HLA-DR1, DR4) major histocompatibilities have been reported [22, 23, 24, 25]. Higher frequencies of a homozygous null C4 phenotype [26]and a C3FF phenotype [27]have been described.…”

“…Familial clustering [22]and geographic variation in the prevalence of the disease are well recognized. Immunologic studies in the families of patients have revealed abnormalities of the immune response similar to those found in affected patients.…”

Molecular Genetics in IgA Nephropathy

“…Subsequently, Julian et al studied a pedigree encompassing seven generations including 14 patients with biopsy-proven IgAN, 17 with clinical hematuria or proteinuria, and six with Bright’s disease on their death certificates [10]; this has been followed by scattered reports of single families [11]. More recently, Schena et al [12]studied 269 relatives from 48 families of IgAN patients and found urinary abnormalities in about 23% of the relatives.…”