The changing face of the 'royal disease' - Medicolegal aspects of haemophilia

dc.contributor.authorByard, R.
dc.date.issued2013
dc.description.abstractHaemophilia represents a group of heritable disorders caused by deficiencies in plasma proteins that are involved in coagulation. The case of a two-year-old boy with an established diagnosis of haemophilia B is reported to demonstrate a rare cause of unexpected death. He had a recent history of epistaxis and was found unexpectedly dead. At autopsy the posterior pharynx was obstructed by blood clot with aspirated blood within the distal airways of the lungs, and melena throughout both small and large intestines. His death was due to haemorrhage and airway obstruction complicating epistaxis. The profile of individuals with haemophilia has been changing in recent years with less deaths from haemorrhage due to improved clinical management. As the life expectancy of these patients is increasing, forensic examiners will now have to consider not only possible haemorrhagic causes of death but will also have to determine the significance of more long standing infectious processes related to human immunodeficiency virus (HIV) and hepatitis C from contaminated transfusions, in addition to evaluating the role of the more usual diseases related to age.
dc.description.statementofresponsibilityRoger W. Byard
dc.identifier.citationJournal of Clinical Forensic and Legal Medicine: an international journal of forensic and legal medicine, 2013; 20(5):392-394
dc.identifier.doi10.1016/j.jflm.2012.07.011
dc.identifier.issn1752-928X
dc.identifier.issn1878-7487
dc.identifier.orcidByard, R. [0000-0002-0524-5942]
dc.identifier.urihttp://hdl.handle.net/2440/81609
dc.language.isoen
dc.publisherChurchill Livingstone
dc.rights© 2012 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.
dc.source.urihttps://doi.org/10.1016/j.jflm.2012.07.011
dc.subjectEpistaxis
dc.subjectHaemophilia
dc.subjectHaemorrhage
dc.subjectHepatitis C
dc.subjectHIV
dc.subjectQueen Victoria
dc.subjectRomanov
dc.subjectSudden death
dc.titleThe changing face of the 'royal disease' - Medicolegal aspects of haemophilia
dc.typeJournal article
pubs.publication-statusPublished

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