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Membranoproliferative glomerulonephritis in two siblings: report and literature review
R M Bogdanović1, J Z Dimitrijević, V N Nikolić
1Institute of Mother and Child Health of Serbia, Belgrade, Yugoslavia. maloun@eunet.yu
Abstract:
There is evidence of a genetic basis in some cases of idiopathic membranoproliferative glomerulonephritis (MPGN) types I and III, particularly those occurring in families. The clinical and morphological features and disease course in two siblings with MPGN are described. In the male sibling, both clinical and morphological features as well as serum complement profile suggested type I MPGN; electron microscopy appearance in the female sibling was consistent with type III MPGN. Both patients had treatment-resistant nephrotic syndrome which evolved into renal insufficiency in the girl. No hereditary complement deficiencies were found in siblings or their parents. Both children exhibited HLA-A24; -B27, w4; -DR11, 52; -DQ3 antigens. Between 1981 and 1996, 18 patients from eight families with unequivocal diagnosis of MPGN I or III had been described. The mode of inheritance appeared to be autosomal dominant or X-linked in four of these families. In 11 patients, including our 2, in whom HLA typing was performed, eight had the HLA-A2 antigen. Similarities and discrepancies regarding clinical and morphological features and outcomes were evident in these intrafamilial cases, suggesting either a similar genetic background or a multigenic origin of MPGN. The familial occurrence of the MPGN, highlighted by our report, supports the concept that genetically determined factors may be involved in the pathogenesis of the disease.
Insights
Familial membranoproliferative glomerulonephritis (MPGN) suggests a genetic basis for MPGN types I and III. Genetic factors likely contribute to the varied clinical and morphological features observed in these kidney diseases.
Area of Science:
- Nephrology
- Genetics
- Immunology
Background:
- Idiopathic membranoproliferative glomerulonephritis (MPGN) can have a genetic component, especially in familial cases.
- Understanding the genetic underpinnings of MPGN types I and III is crucial for diagnosis and treatment.
Observation:
- Two siblings presented with MPGN, with the male exhibiting features of type I and the female of type III.
- Both siblings had treatment-resistant nephrotic syndrome, progressing to renal insufficiency in the female.
- No hereditary complement deficiencies were identified in the family.
Findings:
- Familial MPGN cases suggest a genetic predisposition, potentially with autosomal dominant or X-linked inheritance patterns.
- Human Leukocyte Antigen (HLA) typing revealed shared antigens (HLA-A24, -B27, -DR11, -DQ3) in the siblings.
- A significant proportion of familial MPGN patients, including these siblings, carry the HLA-A2 antigen.
Implications:
- The familial occurrence of MPGN supports the role of genetically determined factors in its pathogenesis.
- Variations in clinical presentation and outcomes within families may indicate a shared genetic background or multigenic origins.
- Further research into the genetic basis of MPGN is warranted to improve understanding and therapeutic strategies.