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

Chalmers, E. A., Alamelu, J., Collins, P. W., Mathias, M., Payne, J., Richards, M., Tunstall, O., Williams, M., Palmer, B. and Mumford, A. 2018. Intracranial haemorrhage in children with inherited bleeding disorders in the UK 2003-2015: a national cohort study. Haemophilia 24 (4) , pp. 641-647. 10.1111/hae.13461

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Abstract

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 of age). In neonates, delivery was spontaneous vaginal (SV) in 11, instrumental in 6, caesarean in 4 and unknown in 1. In children with haemophilia, the risk of ICH after instrumental delivery was 10.6 times greater than after SV delivery. Trauma was more common in 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.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Publisher: Wiley: 12 months
ISSN: 1351-8216
Date of First Compliant Deposit: 20 April 2018
Date of Acceptance: 14 February 2018
Last Modified: 10 Apr 2019 01:33
URI: http://orca.cf.ac.uk/id/eprint/110816

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