title: Prophylaxis therapy in paediatric patients with haemophilia: a survey of clinical management trends in Italy.
abstract: BACKGROUND
  Although current treatment guidelines recommend prophylaxis in paediatric patients with haemophilia, specific indications for and barriers to the prescription of prophylaxis in the paediatric haemophiliac population have not been established. The aim of this web-based survey of clinicians at Haemophilia Treatment Centres in Italy was to identify factors for and against the initiation of prophylactic coagulation factor replacement therapy in paediatric patients with haemophilia.
  
  
  METHODS
  A literature search was conducted to identify factors to include in the survey. Seventeen clinicians from Italian Haemophilia Centres were invited to complete the web-based survey and to rank factors in favour of and those that acted as barriers to prophylaxis in terms of "importance" and "influence" on a numerical scale (0=not important to 100=very important). Any factors for which there was a large discrepancy in results from the survey were further "ranked" by clinicians at an interactive question and answer session at a symposium.
  
  
  RESULTS
  A total of 13 web surveys were returned; the most highly scored factors favouring prophylaxis were "bleeding frequency", "bleeding severity" and "presence of target joints", and the most highly scored barriers were "parents' acceptance", "venous access" and "compliance to therapy". Other important factors favouring prophylaxis were "severity of coagulation defect" and "orthopaedic score".
  
  
  CONCLUSIONS
  This survey gives helpful clinician-derived information for people treating haemophiliacs in Italy, to help the treatment-providers orient themselves better regarding the prescription of prophylaxis for paediatric patients.
authors.affiliation: Centre for Bleeding Disorders, Careggi University Hospital, Florence, Italy.|Haemophilia and Thrombosis Centre, Department of Haematology, San Bortolo Hospital, Vicenza, Italy.
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Castaman
authors.affiliation: Haemophilia and Thrombosis Centre, San Giovanni Bosco Hospital, Naples, Italy.
authors.forename: Angiola
authors.initials: A
authors.lastname: Rocino
authors.affiliation: Department of Cellular Biotechnology and Haematology, La Sapienza University, Rome, Italy.
authors.forename: M Gabriella
authors.initials: MG
authors.lastname: Mazzucconi
authors.affiliation: Department of Cardiologic, Thoracic and Vascular Sciences, Haemophilia Center-University of Padua, Italy.
authors.forename: Ezio
authors.initials: E
authors.lastname: Zanon
authors.affiliation: Reference Centre for Paediatric Haemostasis and Thrombosis, G. Di Cristina Hospital, Palermo, Italy.
authors.forename: Fabio
authors.initials: F
authors.lastname: Gagliano
authors.affiliation: Thrombosis and Haemostasis Unit, Haemophilia Treatment Centre, Giannina Gaslini Children's Hospital, Genoa, Italy.
authors.forename: Angelo C
authors.initials: AC
authors.lastname: Molinari
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D019468:Disease Management; D005164:Factor IX / Q000008:administration & dosage / Q000276:immunology / Q000627:therapeutic use*; D005169:Factor VIII / Q000008:administration & dosage / Q000276:immunology / Q000627:therapeutic use*; D019538:Health Care Surveys; D006395:Hemarthrosis / Q000209:etiology / Q000517:prevention & control; D006467:Hemophilia A / Q000150:complications*; D006470:Hemorrhage / Q000209:etiology / Q000517:prevention & control*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007518:Isoantibodies / Q000096:biosynthesis; D007558:Italy; D010290:Parents / Q000523:psychology; D010349:Patient Compliance; D010818:Practice Patterns, Physicians' / Q000706:statistics & numerical data*; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000627:therapeutic use; D012008:Recurrence; D012307:Risk Factors; D012720:Severity of Illness Index
chemical_list: D007518:Isoantibodies; D011994:Recombinant Proteins; D005169:Factor VIII; D005164:Factor IX