title: Low mortality from intracranial haemorrhage in paediatric patients with haemophilia.
abstract: BACKGROUND
  Intracranial haemorrhage (ICH) in patients with haemophilia has an estimated mortality rate of 20%. Advances in haemophilia care have significantly reduced many bleeding complications but it is unclear if these advances have impacted mortality from ICH.
  
  
  OBJECTIVE
  To determine the in-hospital mortality from intracranial ICH in paediatric patients with haemophilia.
  
  
  METHODS
  This retrospective multicentre cohort study utilized the Pediatric Health Information System administrative database with data from 43 paediatric tertiary care hospitals in the United States from January 1, 2002-December 31, 2011. Subjects included were male < 21 years of age with an ICD-9-CM code for haemophilia A or B. ICH events were identified using ICD-9-CM codes.
  
  
  RESULTS
  There were 8325 admissions for 3133 male subjects with haemophilia. About 271 (3.3%) admissions had an ICH event in 236 (7.5%) individual subjects. The proportion of ICH events was stable over time (P = 0.13). The median age of ICH was 2 years (interquartile range 0.6-7.3). In 28.4% (77/271) of the ICH events the subject had an inhibitor. Twenty-one deaths occurred in the entire cohort (0.7%). Six (28.6%) of these deaths were in patients with an ICH for an ICH mortality rate of 2.5% (6/236).
  
  
  CONCLUSIONS
  Mortality from ICH in paediatric patients has significantly improved from prior estimates of 20% to the current estimate of 2.5%. Unfortunately the rate of ICH events remains constant and further efforts are needed to identify alternative strategies of prevention.
authors.affiliation: Division of Hematology, Departments of Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: C M
authors.initials: CM
authors.lastname: Witmer
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006467:Hemophilia A / Q000150:complications*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D020300:Intracranial Hemorrhages / Q000209:etiology* / Q000401:mortality*; D008297:Male; D013997:Time Factors; D055815:Young Adult
keywords: database; haemophilia; inhibitor; intracranial haemorrhage; mortality; paediatrics