title: Acute renal failure associated to paroxysmal nocturnal haemoglobinuria leads to intratubular haemosiderin accumulation and CD163 expression.
abstract: Decreased renal function has been observed in diseases with intravascular haemolysis, including paroxysmal nocturnal haemoglobinuria (PNH). However, the mechanisms via which haemoglobin enhances renal damage in this pathology are not fully known. We report a case of acute renal failure associated to PNH and extensive haemosiderin deposits in tubular cells. Renal biopsy also revealed a strong immunostaining of CD163 (a haemoglobin scavenger receptor expressed in macrophages) and oxidative stress markers (NADPH-p22 phox and haeme oxigenase-1) in areas with deposits of iron. This fact provides evidence for a pathogenic role for free haemoglobin in tubulointerstitial renal injury in human PNH disease.
authors.affiliation: Department of Nephrology, Fundació Puigvert, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: José
authors.initials: J
authors.lastname: Ballarín
authors.forename: Yolanda
authors.initials: Y
authors.lastname: Arce
authors.forename: Roser
authors.initials: R
authors.lastname: Torra Balcells
authors.forename: Montserrat
authors.initials: M
authors.lastname: Diaz Encarnación
authors.forename: Felix
authors.initials: F
authors.lastname: Manzarbeitia
authors.forename: Alberto
authors.initials: A
authors.lastname: Ortiz
authors.forename: Jesús
authors.initials: J
authors.lastname: Egido
authors.forename: Juan Antonio
authors.initials: JA
authors.lastname: Moreno
mesh_terms: D058186:Acute Kidney Injury / Q000150:complications*; D000328:Adult; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D015415:Biomarkers / Q000378:metabolism*; D051547:Heme Oxygenase-1 / Q000378:metabolism; D006454:Hemoglobins / Q000378:metabolism; D006457:Hemoglobinuria, Paroxysmal / Q000175:diagnosis* / Q000209:etiology*; D006485:Hemosiderin / Q000378:metabolism*; D006801:Humans; D007124:Immunoenzyme Techniques; D007501:Iron / Q000378:metabolism; D007684:Kidney Tubules / Q000503:physiopathology*; D008264:Macrophages / Q000378:metabolism; D008297:Male; D009249:NADP / Q000378:metabolism; D019255:NADPH Oxidases / Q000378:metabolism; D011956:Receptors, Cell Surface / Q000378:metabolism; D055815:Young Adult; D000098902:CD163 Antigen
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D015415:Biomarkers; D000098902:CD163 Antigen; D006454:Hemoglobins; D011956:Receptors, Cell Surface; D009249:NADP; D006485:Hemosiderin; D007501:Iron; D051547:Heme Oxygenase-1; D019255:NADPH Oxidases; C086986:CYBA protein, human