title: A case of atypical hemolytic uremic syndrome in a second renal transplant.
abstract: Atypical hemolytic uremic syndrome (aHUS) has gained increased visibility over several years as an important cause of renal failure. Unfortunately, diagnosis is often difficult because individual courses can be highly variable depending the causative genetic mutations. Here we present the case of a patient with a failed renal allograft and acute failure of a second allograft who was ultimately diagnosed with aHUS. Interestingly, he developed early de novo donor specific antibodies (DSA) after the second renal transplant in context of likely recurrent aHUS. Terminal complement inhibition with eculizumab resulted in prompt improvement of renal allograft function.
authors.affiliation: Comprehensive Transplant Center, Northwestern University Feinberg School of Medicine, Chicago, USA. nzwang1@uic.edu.|McGaw Medical Center of Northwestern University, Chicago, USA. nzwang1@uic.edu.|Northwestern Memorial Hospital/Arkes Family Pavilion Suite 1900, 676N. St. Clair, Chicago, IL, 60611, USA. nzwang1@uic.edu.
authors.forename: Nicholas A
authors.identifier: http://orcid.org/0000-0003-3675-5264
authors.initials: NA
authors.lastname: Zwang
authors.affiliation: Comprehensive Transplant Center, Northwestern University Feinberg School of Medicine, Chicago, USA.
authors.forename: Bing
authors.initials: B
authors.lastname: Ho
authors.affiliation: Department of Pathology, Northwestern Memorial Hospital, Chicago, USA.
authors.forename: Yashpal S
authors.initials: YS
authors.lastname: Kanwar
authors.affiliation: Machaon Diagnostics, Oakland, CA, USA.
authors.forename: Brad
authors.initials: B
authors.lastname: Lewis
authors.affiliation: Comprehensive Transplant Center, Northwestern University Feinberg School of Medicine, Chicago, USA.
authors.forename: Matthew
authors.initials: M
authors.lastname: Cusick
authors.affiliation: Comprehensive Transplant Center, Northwestern University Feinberg School of Medicine, Chicago, USA.|McGaw Medical Center of Northwestern University, Chicago, USA.|Department of Pathology, Northwestern Memorial Hospital, Chicago, USA.|Machaon Diagnostics, Oakland, CA, USA.|Northwestern Memorial Hospital/Arkes Family Pavilion Suite 1900, 676N. St. Clair, Chicago, IL, 60611, USA.
authors.forename: John J
authors.initials: JJ
authors.lastname: Friedewald
authors.affiliation: Comprehensive Transplant Center, Northwestern University Feinberg School of Medicine, Chicago, USA.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Gallon
mesh_terms: D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D065766:Atypical Hemolytic Uremic Syndrome / Q000175:diagnosis / Q000235:genetics / Q000276:immunology* / Q000628:therapy; D001706:Biopsy; D003167:Complement Activation / Q000187:drug effects / Q000235:genetics; D017244:Complement Factor I / Q000235:genetics; D051056:Complement Inactivating Agents / Q000627:therapeutic use; D004252:DNA Mutational Analysis; D020022:Genetic Predisposition to Disease; D006801:Humans; D007668:Kidney / Q000187:drug effects / Q000276:immunology* / Q000473:pathology / Q000601:surgery; D016030:Kidney Transplantation / Q000009:adverse effects*; D008297:Male; D009154:Mutation; D010641:Phenotype; D012008:Recurrence; D012086:Reoperation; D017211:Treatment Failure
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D051056:Complement Inactivating Agents; C481642:eculizumab; C493611:CFI protein, human; D017244:Complement Factor I
keywords: Atypical hemolytic uremic syndrome; Complement biology.; Graft failure; Histocompatibility; Kidney transplantation