Hereditary haemorrhagic telangiectasia: a cause of preventable morbidity and mortality.

Hdl Handle:
http://hdl.handle.net/10147/206363
Title:
Hereditary haemorrhagic telangiectasia: a cause of preventable morbidity and mortality.
Authors:
Brady, A P; Murphy, M M; O'Connor, T M
Affiliation:
National HHT Centre, Mercy University Hospital, Cork, Ireland. abrady@muh.ie
Citation:
Ir J Med Sci. 2009 Jun;178(2):135-46. Epub 2008 Oct 16.
Journal:
Irish journal of medical science
Issue Date:
31-Jan-2012
URI:
http://hdl.handle.net/10147/206363
DOI:
10.1007/s11845-008-0220-5
PubMed ID:
18923886
Abstract:
Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant condition whose effects are mediated through deficient blood vessel formation and regeneration, with multisystem involvement. Patients are usually aware of resulting skin telangiectasia and epistaxis, but are also exposed to dangers posed by occult vascular malformations in other organs. About 15-35% of HHT patients have pulmonary AVMs (PAVMs), 10% have cerebral AVMs (CAVMs), 25-33% suffer significant GI blood loss from GI tract telangiectasia, and an unknown but high percentage have liver involvement. In total, 10% of affected individuals die prematurely or suffer major disability from HHT, largely because of bleeding from CAVMs and PAVMs, or paradoxical embolization through PAVMs. Screening for and early intervention to treat occult PAVMs and CAVMs can largely eliminate these risks, and should be undertaken in a specialist centre. The National HHT Center in The Mercy University Hospital in Cork is the referral centre for HHT screening in Ireland.
Language:
eng
MeSH:
Arteriovenous Malformations; Embolization, Therapeutic; Epistaxis; Humans; Intracranial Arteriovenous Malformations; Ireland; Telangiectasia, Hereditary Hemorrhagic/*diagnosis/drug therapy/mortality/surgery
ISSN:
1863-4362 (Electronic); 0021-1265 (Linking)

Full metadata record

DC FieldValue Language
dc.contributor.authorBrady, A Pen_GB
dc.contributor.authorMurphy, M Men_GB
dc.contributor.authorO'Connor, T Men_GB
dc.date.accessioned2012-01-31T16:38:37Z-
dc.date.available2012-01-31T16:38:37Z-
dc.date.issued2012-01-31T16:38:37Z-
dc.identifier.citationIr J Med Sci. 2009 Jun;178(2):135-46. Epub 2008 Oct 16.en_GB
dc.identifier.issn1863-4362 (Electronic)en_GB
dc.identifier.issn0021-1265 (Linking)en_GB
dc.identifier.pmid18923886en_GB
dc.identifier.doi10.1007/s11845-008-0220-5en_GB
dc.identifier.urihttp://hdl.handle.net/10147/206363-
dc.description.abstractHereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant condition whose effects are mediated through deficient blood vessel formation and regeneration, with multisystem involvement. Patients are usually aware of resulting skin telangiectasia and epistaxis, but are also exposed to dangers posed by occult vascular malformations in other organs. About 15-35% of HHT patients have pulmonary AVMs (PAVMs), 10% have cerebral AVMs (CAVMs), 25-33% suffer significant GI blood loss from GI tract telangiectasia, and an unknown but high percentage have liver involvement. In total, 10% of affected individuals die prematurely or suffer major disability from HHT, largely because of bleeding from CAVMs and PAVMs, or paradoxical embolization through PAVMs. Screening for and early intervention to treat occult PAVMs and CAVMs can largely eliminate these risks, and should be undertaken in a specialist centre. The National HHT Center in The Mercy University Hospital in Cork is the referral centre for HHT screening in Ireland.en_GB
dc.language.isoengen_GB
dc.subject.meshArteriovenous Malformationsen_GB
dc.subject.meshEmbolization, Therapeuticen_GB
dc.subject.meshEpistaxisen_GB
dc.subject.meshHumansen_GB
dc.subject.meshIntracranial Arteriovenous Malformationsen_GB
dc.subject.meshIrelanden_GB
dc.subject.meshTelangiectasia, Hereditary Hemorrhagic/*diagnosis/drug therapy/mortality/surgeryen_GB
dc.titleHereditary haemorrhagic telangiectasia: a cause of preventable morbidity and mortality.en_GB
dc.contributor.departmentNational HHT Centre, Mercy University Hospital, Cork, Ireland. abrady@muh.ieen_GB
dc.identifier.journalIrish journal of medical scienceen_GB
dc.description.provinceMunster-

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