A comparative study of two grading systems for epistaxis in hereditary haemorrhagic telangiectasia

Jørgensen, Ole Jakob; Steineger, Johan; Bachmann-Harildstad, Gregor; Dheyauldeen, Sinan

doi:10.4193/rhin20.540

Cited by 2 publications

(3 citation statements)

References 23 publications

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“…The epistaxis grade was evaluated using the Epistaxis Severity Score (ESS) 32 and the Epistaxis Intensity, Frequency, and Need for Blood Transfusion Score (IFT). 33,34 Blood tests were collected with each consultation or treatment and screened for coagulation factors VIII and IX, vWF antigen and activity, hemoglobin, ferritin, iron, and C-reactive protein (CRP). However, the measurements of FIX and vWF were not performed for all patients.…”

Section: Methods and Study Participantsmentioning

confidence: 99%

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Elevated FVIII levels in hereditary hemorrhagic telangiectasia: Implications for clinical management

Jørgensen,

Steineger,

Hillarp

et al. 2023

Laryngoscope Investig Oto

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ObjectivesThe objective of this study was twofold: to determine the prevalence of arterial and venous thromboembolic events in the Norwegian Hereditary Hemorrhagic Telangiectasia (HHT) population, and to explore potential factors linked to such events, with particular emphasis on FVIII.MethodsPatients with an HHT diagnosis attending the Otorhinolaryngology Department at Oslo University Hospital—Rikshospitalet were included consecutively between April 2021 and November 2022. We recorded the participants' medical history with an emphasis on thromboembolic events. Measurements of blood constituents, including FVIII, FIX, vWF, hemoglobin, iron, ferritin, and CRP were performed.ResultsOne hundred and thirty‐four patients were included in the study. The total prevalence of thromboembolic events among the participants was 23.1%. FVIII levels were high (>150 IU/dL) in the majority of HHT patients (n = 84) (68.3%) and were significantly associated with thromboembolic events (p < .001), as was age. Of the patients with high FVIII levels, 28 (33%) had experienced a thromboembolic event. Furthermore, FVIII levels were measured consecutively in 51 patients and were found to fluctuate above or below 150 IU/dL in 25% of these cases.ConclusionThromboembolic events are highly prevalent in the Norwegian HHT population and are significantly associated with FVIII levels. FVIII levels can fluctuate, and measurements should be repeated in HHT patients to assess the risk of thromboembolic events.Level of Evidence4.

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Section: Methods and Study Participantsmentioning

confidence: 99%

“…The epistaxis grade was evaluated using the Epistaxis Severity Score (ESS) 32 and the Epistaxis Intensity, Frequency, and Need for Blood Transfusion Score (IFT). 33 , 34 …”

Section: Methods and Study Participantsmentioning

confidence: 99%

Elevated FVIII levels in hereditary hemorrhagic telangiectasia: Implications for clinical management

Jørgensen,

Steineger,

Hillarp

et al. 2023

Laryngoscope Investig Oto

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“…The Epistaxis Severity Score (ESS) is a gold-standard, patient-reported outcome measure specifically designed to evaluate nosebleed severity in patients with Hereditary Hemorrhagic Telangiectasia (HHT) [ 18 ]. It was proposed by Hoag et al for the International HHT Foundation in 2010 and has been used in various studies to assess the severity and impact of epistaxis in HHT patients [ 19 ]. This score is typically documented in patient charts and is confirmed based on documented patient histories [ 20 ].…”

Section: Introductionmentioning

confidence: 99%

Surgery or No Surgery? Exploring the Dilemma of Epistaxis Management in Patients with HHT

Passali,

Santantonio,

Vecchioli

et al. 2024

JCM

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Background: Epistaxis, particularly in Hereditary Hemorrhagic Telangiectasia (HHT) patients, is a common otolaryngological emergency, often requiring complex management. A hierarchy of increasingly invasive interventions, from external compression of the nasal pyramid to nostril closure, is typically proposed and applied. Methods: We conducted a retrospective study on HHT patients to assess the effectiveness and longevity of invasive procedures postoperatively. Data were collected using the Epistaxis Severity Score (ESS) questionnaire. The primary focus was on changes in the frequency and intensity of epistaxis, while the secondary focus was on the overall quality of life. Results: This study found that invasive procedures initially improved the frequency and intensity of epistaxis in HHT patients. However, within 1 to 9 months postoperatively, these benefits often diminished, with hemorrhagic symptoms recurring at similar or worsened levels. Conclusions: The findings suggest a need for a cautious and restrained approach to using invasive treatments in managing epistaxis in HHT patients. Highly invasive procedures should be reserved for cases where less invasive methods fail, due to their temporary effectiveness and the risk of causing anatomical–functional changes in the rhino-sinus area, complicating future management of severe epistaxis.

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A comparative study of two grading systems for epistaxis in hereditary haemorrhagic telangiectasia

Cited by 2 publications

References 23 publications

Elevated FVIII levels in hereditary hemorrhagic telangiectasia: Implications for clinical management

Elevated FVIII levels in hereditary hemorrhagic telangiectasia: Implications for clinical management

Surgery or No Surgery? Exploring the Dilemma of Epistaxis Management in Patients with HHT

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