Membranous nephropathy in a patient with hereditary complete complement C4 deficiency
Open Access
- 18 March 2004
- journal article
- case report
- Published by Oxford University Press (OUP) in Nephrology Dialysis Transplantation
- Vol. 19 (4) , 990-993
- https://doi.org/10.1093/ndt/gfh008
Abstract
Hereditary complete deficiency of complement C4 is an extremely rare condition. Fewer than 30 cases have been reported so far. Because C4 exists in two isoforms, C4A and C4B, whose genes lie on chromosome 6 in the MHC class III region, complete deficiency requires a defect in all four C4 genes. In the majority of patients this is caused by homozygocity for a C4AQ0C4BQ0 haplotype (HLA A24 B38 Cw7 DR13 DQ6 in our patient).Keywords
This publication has 7 references indexed in Scilit:
- Complement C4 Is Protective for Lupus Disease Independent of C3The Journal of Immunology, 2002
- High-Dose Intravenous Immunoglobulin Treatment Activates Complement In VivoScandinavian Journal of Immunology, 1998
- Recurrent hematuria: A novel clinical presentation of hereditary complete complement C4 deficiencyAmerican Journal of Kidney Diseases, 1996
- Glomerulonephritis associated with complete deficiency of the fourth component of complement response to intravenous immunoglobulinArthritis & Rheumatism, 1995
- Urinary C3dg and C5b-9 indicate active immune disease in human membranous nephropathyKidney International, 1992
- Renal Disease in a Patient with Hereditary Complete Deficiency of the Fourth Component of ComplementNephron, 1990
- The Role of Complement and Its Receptor in the Elimination of Immune ComplexesNew England Journal of Medicine, 1986