title: Recurrence of HUS due to CD46/MCP mutation after renal transplantation: a role for endothelial microchimerism.
abstract: Mutations in the gene of the membrane cofactor protein (MCP/CD46), a complement regulatory protein, were recently described as a cause of hemolytic uremic syndrome (HUS). MCP is a transmembrane glycoprotein expressed in kidneys; therefore, the transplantation of a normal kidney should not be complicated by HUS recurrence. However, we report the case of a 32-year-old woman with an MCP mutation who developed a recurrence of HUS after renal transplantation. We found that she had vascular microchimerism of endothelial cells. We suggest that recurrence may be favored by vascular microchimerism, in which the mutated protein is produced in the in the kidney graft by endothelial cells originating from recipient.
authors.affiliation: Immunology Department, Hôpital Européen Georges Pompidou, Paris, France.
authors.forename: V
authors.initials: V
authors.lastname: Frémeaux-Bacchi
authors.forename: N
authors.initials: N
authors.lastname: Arzouk
authors.forename: S
authors.initials: S
authors.lastname: Ferlicot
authors.forename: B
authors.initials: B
authors.lastname: Charpentier
authors.forename: R
authors.initials: R
authors.lastname: Snanoudj
authors.forename: A
authors.initials: A
authors.lastname: Dürrbach
mesh_terms: D058186:Acute Kidney Injury / Q000601:surgery; D000328:Adult; D001706:Biopsy; D046528:Chimerism; D004247:DNA / Q000235:genetics*; D042783:Endothelial Cells / Q000473:pathology*; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005500:Follow-Up Studies; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D016030:Kidney Transplantation; D051916:Membrane Cofactor Protein / Q000235:genetics* / Q000276:immunology / Q000378:metabolism; D009154:Mutation; D011183:Postoperative Complications; D012008:Recurrence; D020133:Reverse Transcriptase Polymerase Chain Reaction
chemical_list: C497857:CD46 protein, human; D051916:Membrane Cofactor Protein; D004247:DNA