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

Autor: Chalmers EA; Department of Haematology, Royal Hospital for Children, Glasgow, UK., Alamelu J; Department of Paediatric Haematology, Evelina Children's Hospital, London, UK., Collins PW; School of Medicine, Cardiff University, Cardiff, UK., Mathias M; Department of Haematology, Great Ormond Street Hospital for Children NHS Trust, London, UK., Payne J; Department of Haematology, Sheffield Children's Hospital, Sheffield, UK., Richards M; Department of Haematology, Leeds Children's Hospital, Leeds, UK., Tunstall O; Bristol Haemophilia Comprehensive Care Centre, Bristol Royal Hospital for Children, Bristol, UK., Williams M; Haemophilia Centre, Birmingham Childrens' Hospital, Birmingham, UK., Palmer B; The National Haemophilia Database, Manchester, UK., Mumford A; Department of Cellular and Molecular Medicine, University of Bristol, Bristol, UK.
Jazyk: angličtina
Zdroj: Haemophilia : the official journal of the World Federation of Hemophilia [Haemophilia] 2018 Jul; Vol. 24 (4), pp. 641-647. Date of Electronic Publication: 2018 Apr 10.
DOI: 10.1111/hae.13461
Abstrakt: 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 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.
(© 2018 John Wiley & Sons Ltd.)
Databáze: MEDLINE
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