An epistaxis severity score for hereditary hemorrhagic telangiectasia

Hoag, Jeffrey B.; Terry, Peter B.; Mitchell, Sally E.; Reh, Douglas D.; Merlo, Christian A.

doi:10.1002/lary.20818

Cited by 218 publications

(245 citation statements)

References 12 publications

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“…High proportion without medical or ENT review 33,34 . New data: Surgery and embolization [35][36][37][38][39] ., medical treatments 31,[40][41][42][43][44][45] …”

Section: New Data On Echo Grading and Predictive Valuesmentioning

confidence: 99%

“…A recent study of 915 HHT-affected individuals indicated that a severity score based on presence of anaemia and need for transfusion in addition to four other independent factors (nose bleed frequency; duration; gushing or pouring quality; or the need for medical attention) was a significant predictor of invasiveness of therapy required for nosebleeds 33 . These data, together with the new evidence that nosebleed frequency correlates with iron and transfusional need (Fig 2), highlight the need to obtain good ENT-based reviews of anaemic patients, for specialist ENT treatments as outlined in Table 1.…”

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confidence: 99%

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Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

2010

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Hereditary haemorrhagic telangiectasia, inherited as an autosomal dominant trait, affects approximately 1 in 5,000 people. The abnormal vascular structures in HHT result from mutations in genes (most commonly endoglin or ACVRL1) whose protein products influence TGF-ß superfamily signalling in vascular endothelial cells. The cellular mechanisms underlying the generation of HHT telangiectasia and arteriovenous malformations are being unravelled, with recent data focussing on a defective response to angiogenic stimuli in particular settings. For affected individuals, there is often substantial morbidity due to sustained and repeated haemorrhages from telangiectasia in the nose and gut. Particular haematological clinical challenges include the management of severe iron deficiency anaemia; handling the intricate balance of antiplatelet or anticoagulants for HHT patients in whom there are often compelling clinical reasons to use such agents; and evaluation of apparently attractive experimental therapies promoted in high profile publications when guidelines and reviews are quickly superseded. There is also a need for sound screening programmes for silent arteriovenous malformations. These occur commonly in the pulmonary, cerebral, and hepatic circulations, may haemorrhage, but predominantly result in more complex pathophysiology due to consequences of defective endothelium, or shunts that bypass specific capillary beds. This review will focus on the new evidence and concepts in this complex and fascinating condition, placing these in context for both clinicians and scientists, with a particular emphasis on haematological settings. Blood Reviews _ HHT 2010_ Shovlin 3 Overview of HHTHHT, also known as Osler Weber Rendu syndrome [1][2][3] , is one of the most common disorders to be inherited as an autosomal dominant trait. Careful epidemiological studies reveal that it affects approximately 1 in 5,000 individuals, 4,5 with regional differences, 6 and isolated communities displaying higher prevalences due to founder effects. 7 8 HHT was first described as a familial disease characterised by severe recurrent nasal and gastrointestinal bleeding with associated anaemia, and visible dilated blood vessels (telangiectasia) on the lips and finger tips. The majority of HHT patients are also affected by larger arteriovenous malformations (AVMs) in the pulmonary, hepatic, cerebral, pancreatic, spinal and other circulations. 1,2,9 These features, presented in more detail within Table 1, are used as criteria to diagnose HHT. 2 The spectrum of disease within the HHT umbrella has extended beyond the telangiectatic/AVM HHT pathology delineated by the Curaçao criteria. 2 More recently recognised features include pulmonary arterial hypertension 10 ; juvenile polyposis 11 ; pulmonary hypertension in the context of high output cardiac failure secondary to hepatic AVMs, when PH may be reversible after hepatic AVM treatment [12][13][14][15][16] ; a prothrombotic state associated with elevated plasma levels of factor VIII 17 , and poten...

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“…High proportion without medical or ENT review 33,34 . New data: Surgery and embolization [35][36][37][38][39] ., medical treatments 31,[40][41][42][43][44][45] …”

Section: New Data On Echo Grading and Predictive Valuesmentioning

confidence: 99%

mentioning

confidence: 99%

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

2010

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“…Within this period, ~180 eligible patients were approached by letter or in person. All recruited participants recorded nosebleed severity by the validated Epistaxis Severity Score (ESS) (16). This rates nosebleed severity on a scale of 0-10, with the minimal important difference recently identified as 0.71 (17).…”

Section: Survey Methodologymentioning

confidence: 99%

Dietary supplement use and nosebleeds in hereditary haemorrhagic telangiectasia ‒ an observational study

Chamali

Finnamore

Manning

et al. 2016

IRDR

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“…Certain foods, often those containing high salicylate levels, have been reported to worsen epistaxis, as have low humidity and changes in temperature (34) . Various subjective grading systems are available (35,36) , and objective classification of the pattern of lesions has also been described (37) . Nasal telangiectasia are tortuous dilated superficial vessels (Figure 1), very sensitive to even the minor trauma of airflow, and a lack of elastin fibres means that they do not vasoconstrict to limit bleeding (38) .…”

Section: Clinical Featuresmentioning

confidence: 99%

Hereditary haemorrhagic telangiectasia

Rimmer

Lund

2015

Rhin

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Background: Hereditary haemorrhagic telangiectasia is an autosomal dominant vascular disease characterized by recurrent epistaxis, mucocutaneous telangiectasia and visceral arteriovenous malformations. Methodology:The genetic basis and pathophysiology of the disease are discussed. Diagnostic criteria and the clinical course of the condition are considered. The current management options, both medical and surgical, are reviewed. Conclusions:Hereditary haemorrhagic telangiectasia requires specialist treatment for the problems it causes, and is best managed in specialist centres. Epistaxis is often the major symptom, significantly affecting patients' quality of life. An understanding of the available treatment options is therefore important for the otorhinolaryngologist.

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An epistaxis severity score for hereditary hemorrhagic telangiectasia

Cited by 218 publications

References 12 publications

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

Dietary supplement use and nosebleeds in hereditary haemorrhagic telangiectasia ‒ an observational study

Hereditary haemorrhagic telangiectasia

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