title: Renal diseases in haemophilic patients: pathogenesis and clinical management.
abstract: Haemophilia A and B are genetic X-linked bleeding disorders, caused by mutations in genes encoding factors VIII and IX, respectively. Clinical manifestations of haemophilia are spontaneous haemorrhage or acute bleeding caused by minor trauma, resulting in severe functional consequences that can culminate in a debilitating arthropathy. Life expectancy and quality of life of patients with haemophilia have dramatically improved over the last years, mainly for new therapeutic options and the awareness to the risk of HCV and HIV infections. Different clinical problems arise from this important change in history of patients with haemophilia. In particular, ageing-related diseases, such as diabetes, hypertension and cancer, and chronic viral infections are emerging as new challenges in this patient population. Among the different types of chronic illnesses, renal diseases are of special interest as they involve some difficult management issues. In fact, decisions regarding adequate preventive strategies and viral infection treatment, the choice of the dialytic modality, placement of vascular access and prescription of dialytic treatments are particularly complicated, because only few data are available. In this review, we discuss the pathogenesis of renal damage in patients with haemophilia, especially in those with blood-transmitted viral infections, and the major issues about the management of renal diseases, including problems related to dialytic treatment and kidney transplantation, providing practical algorithms to guide the clinical decision-making process.
authors: Unit of Nephrology, Dialysis and Transplantation, Fondazione IRCCS Policlinico S.Matteo and University of Pavia, Pavia, Italy.
authors: Pasquale
authors: P
authors: Esposito
authors: Teresa
authors: T
authors: Rampino
authors: Marilena
authors: M
authors: Gregorini
authors: Gianluca
authors: G
authors: Fasoli
authors: Gabriella
authors: G
authors: Gamba
authors: Antonio
authors: A
authors: Dal Canton
mesh_terms: D000375:Aging / Q000097:blood / Q000473:pathology; D000998:Antiviral Agents / Q000627:therapeutic use; D005164:Factor IX / Q000235:genetics / Q000378:metabolism; D005169:Factor VIII / Q000235:genetics / Q000378:metabolism; D015658:HIV Infections / Q000097:blood / Q000150:complications / Q000473:pathology / Q000628:therapy*; D006467:Hemophilia A / Q000097:blood / Q000150:complications / Q000473:pathology / Q000628:therapy*; D002836:Hemophilia B / Q000097:blood / Q000150:complications / Q000473:pathology / Q000628:therapy*; D006526:Hepatitis C / Q000097:blood / Q000150:complications / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007674:Kidney Diseases / Q000097:blood / Q000150:complications / Q000473:pathology / Q000628:therapy*; D016030:Kidney Transplantation; D008017:Life Expectancy; D009154:Mutation; D011788:Quality of Life; D006435:Renal Dialysis / Q000379:methods*
chemical_list: D000998:Antiviral Agents; D005169:Factor VIII; D005164:Factor IX
keywords: bleeding; haemodialysis; haemophilia A; peritoneal dialysis; renal diseases; vascular access device; viral infections