title: Intracranial haemorrhage in the Italian population of haemophilia patients with and without inhibitors.
abstract: Intracranial haemorrhage (ICH) is the most serious bleeding symptom in haemophiliacs, resulting in high rates of mortality and disabling sequelae. The Association of Italian Haemophilia Centres carried out a retrospective survey (1987-2008) of ICH occurring in haemophiliacs with the goals to establish: (i) incidence, location of bleeding, death rate and disabling sequels; (ii) risk factors for ICH; and (iii) treatment used during the acute phase of ICH and for recurrence prevention. A total of 112 ICH episodes had occurred in 88 patients (78 haemophilia A, 10 haemophilia B), 24 of whom experienced recurrences. The cumulative hazard of ICH for the whole cohort over the entire follow-up period was 26.7 per 1000 patients, and the annualized rate of ICH was 2.50 events per 1000 patients (95% CI 1.90-3.31). The risk of ICH was higher in the youngest children (24.4 per 1000, 95% CI 12.7-47.0 in the first year of age and 14.9, 95% CI 7.1-31.4 in the second year of age) and then progressively rose again after the age of 40. Univariate, bivariate (age-adjusted) and multivariate analysis investigating the effects of patient characteristics on ICH occurrence showed that haemophilia severity and inhibitor status were strongly associated with ICH [severe vs. mild, HR 3.96 (2.39-6.57); inhibitor vs. non-inhibitor 2.52 (1.46-4.35)]. HCV infection was also associated with the risk of ICH [HR 1.83 (1.25-2.69)]. Therapeutic suggestions based upon our experience to control ICH recurrence are provided.
authors.affiliation: Haemophilia Center, 2nd Chair Internal Medicine, Department of Cardiologic, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy. simona.siboni@unimi.it
authors.forename: E
authors.initials: E
authors.lastname: Zanon
authors.forename: A
authors.initials: A
authors.lastname: Iorio
authors.forename: A
authors.initials: A
authors.lastname: Rocino
authors.forename: A
authors.initials: A
authors.lastname: Artoni
authors.forename: R
authors.initials: R
authors.lastname: Santoro
authors.forename: A
authors.initials: A
authors.lastname: Tagliaferri
authors.forename: A
authors.initials: A
authors.lastname: Coppola
authors.forename: G
authors.initials: G
authors.lastname: Castaman
authors.forename: P M
authors.initials: PM
authors.lastname: Mannucci
authors.forename: Giovanni
authors.initials: G
authors.lastname: Barillari
authors.forename: Alfredo
authors.initials: A
authors.lastname: Dragani
authors.forename: Gabriella
authors.initials: G
authors.lastname: Gamba
authors.forename: Anna
authors.initials: A
authors.lastname: Giuffrida
authors.forename: Mario
authors.initials: M
authors.lastname: Lapecorella
authors.forename: Giacomo
authors.initials: G
authors.lastname: Mancuso
authors.forename: Liguori
authors.initials: L
authors.lastname: Lucia
authors.forename: Maria Gabriella
authors.initials: MG
authors.lastname: Mazzucconi
authors.forename: Maria
authors.initials: M
authors.lastname: Messina
authors.forename: Roberto
authors.initials: R
authors.lastname: Musso
authors.forename: Francesco
authors.initials: F
authors.lastname: De Martis
authors.forename: Gina
authors.initials: G
authors.lastname: Rossetti
authors.forename: Piercarla
authors.initials: P
authors.lastname: Schinco
authors.forename: Luca
authors.initials: L
authors.lastname: Spiezia
authors.forename: Lelia
authors.initials: L
authors.lastname: Valdrè
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D006467:Hemophilia A / Q000150:complications* / Q000276:immunology; D002836:Hemophilia B / Q000150:complications* / Q000276:immunology; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D020300:Intracranial Hemorrhages / Q000453:epidemiology* / Q000517:prevention & control; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D011795:Surveys and Questionnaires; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D001779:Blood Coagulation Factors