Intracranial haemorrhage in children with inherited bleeding disorders in the UK 2003‐2015: A national cohort study

Introduction Intracranial haemorrhage in children with inherited bleeding disorders is a potentially life‐threatening complication and presents a significant therapeutic challenge. Aim To define the characteristics, management and outcomes of intracranial haemorrhage presenting in UK children ≤16 ye...

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Bibliographic Details
Published in:Haemophilia : the official journal of the World Federation of Hemophilia 2018-07, Vol.24 (4), p.641-647
Main Authors: Chalmers, E. A., Alamelu, J., Collins, P. W., Mathias, M., Payne, J., Richards, M., Tunstall, O., Williams, M., Palmer, B., Mumford, A.
Format: Article
Language:English
Subjects:
Age
Bleeding
Children
Cohort analysis
haemophilia
Hemophilia
Hemorrhage
inherited bleeding disorder
inhibitors
intracranial haemorrhage
neonate
Neonates
Neurological complications
Phenotypes
Prophylaxis
Trauma
Vagina
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Summary:Introduction Intracranial haemorrhage in children with inherited bleeding disorders is a potentially life‐threatening complication and presents a significant therapeutic challenge. Aim To define the characteristics, management and outcomes of intracranial haemorrhage presenting in UK children ≤16 years of age with inherited bleeding disorders from 2003 to 2015. Method Retrospective analysis of children treated at UK haemophilia centres. Results Of 66 children presenting with Intracranial haemorrhage (ICH), 82% had haemophilia A or B, 3% VWD and 15% a rare IBD. The IBD was a severe phenotype in 91%. The rates of ICH were 6.4 and 4.2 per 1000 patient years for haemophilia A and B, respectively. Median age at presentation was 4 months (33% neonates; 91% children 2 years (67%) than in children 1 month to 2 years (18%; P = .027). Prior to ICH, only 4.5% of children were on prophylaxis. 6% of haemophiliacs had an inhibitor. The median duration of initial replacement therapy was 15 days. Mortality was 13.5%. Neurological sequelae occurred in 39% of survivors, being more common following intracerebral bleeding. In haemophilia survivors, 52% subsequently developed a FVIII inhibitor. Conclusion Intracranial haemorrhage occurs most frequently in children with severe IBDs, during the first 2 years of life and in children not receiving prophylaxis. Intracranial haemorrhage often occurs without documented trauma.
ISSN:1351-8216
1365-2516
DOI:10.1111/hae.13461