Recurrent hematuria: a novel clinical presentation of hereditary complete complement C4 deficiency

American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation
K LhottaP König

Abstract

A 10-year-old boy suffered from recurrent attacks of fever, vomiting, and hematuria. During disease flares, circulating immune complexes were detected in the serum. Elevated levels of Bb, Ba, and C3a indicated complement activation through the alternative pathway. Complement C4 was undetectable. C4 phenotyping by agarose gel electrophoresis showed complete C4 deficiency. Restriction fragment length polymorphism (RFLP) studies showed a homozygous deletion of the C4B and 21-hydroxylase A genes. A mild mesangioproliferative glomerulonephritis with mesangial deposits of immunoglobulin (1g) G, IgM, IgA, Clq, C3, properdin, and terminal complement complex was probably caused by immune complex deposition and alternative complement pathway activation. Treatment with low-dose prednisolone substantially reduced the frequency of further episodes.

Citations

Mar 20, 2004·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Karl LhottaGert Mayer
Jul 28, 2018·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Mieke F van EssenUNKNOWN COMBAT consortium
Jun 2, 2007·Internal Medicine·Tokutaro TsudaKaoru Tabei
Jul 23, 2002·The Journal of Immunology : Official Journal of the American Association of Immunologists·Kristi L RupertC Yung Yu
Aug 6, 2004·The Journal of Immunology : Official Journal of the American Association of Immunologists·Yan YangC Yung Yu
Apr 2, 2003·Journal of Immunological Methods·Markus WahrmannGeorg A Böhmig
Jun 29, 2010·Clinical Immunology : the Official Journal of the Clinical Immunology Society·K SotoC Y Yu

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