Nasal self-packing for epistaxis in Hereditary Hemorrhagic Telangiectasia increases quality of life

Droege, Freya; Lueb, C; Thangavelu, Kruthika; Stuck, Boris A.; Lang, Stephan; Geisthoff, Urban

doi:10.4193/rhin18.141

Cited by 13 publications

(16 citation statements)

References 27 publications

(39 reference statements)

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“…After different international patient advocacy groups informed their members about the online survey, a total of 915 persons responded to it. Evaluation of HHT diagnostic criteria resulted in assignment of 588 with HHT (588/915, 64%, [ 12 ]). Not all patients answered all questions which led to smaller subgroups for the following analysis.…”

Section: Resultsmentioning

confidence: 99%

“…Out of 915 respondents 588 could be diagnosed with hereditary hemorrhagic telangiectasia (HHT; the exact explanation can be found in Figure 2 [ 12 ]. There were 469 patients with HHT who stated if their physician had already diagnosed restless leg syndrome (RLS) (“RLS+” = RLS was diagnosed, “RLS-” = no RLS diagnosis; question 85 in Supplementary data ), and 356 completed the RLS Diagnostic Inventory (RLS-DI).…”

Section: Resultsmentioning

confidence: 99%

“…The diagnosis of HHT was established using the modified Curaçao criteria as published by Hosman et al [ 11 ] and Droege et al [ 12 ]. In addition, the general medical history of HHT contained the epistaxis severity score (ESS) for HHT [ 13 ].…”

Section: Methodsmentioning

confidence: 99%

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Restless Leg Syndrome Is Underdiagnosed in Hereditary Hemorrhagic Telangiectasia—Results of an Online Survey

Droege

Stang

Thangavelu

et al. 2021

JCM

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Background: Recurrent bleeding in patients with hereditary hemorrhagic telangiectasia (HHT) can lead to chronic iron deficiency anemia (CIDA). Existing research points to CIDA as a contributing factor in restless leg syndrome (RLS). The association between HHT-related symptoms and the prevalence of RLS was analyzed. Methods: An online survey was conducted whereby the standardized RLS-Diagnostic Index questionnaire (RLS-DI) was supplemented with 82 additional questions relating to HHT. Results: A total of 474 persons responded to the survey and completed responses for questions pertaining to RLS (mean age: 56 years, 68% females). Per RLS-DI criteria, 48 patients (48/322, 15%; 95% confidence interval (CI): 11–19%) self-identified as having RLS. An analysis of physician-diagnosed RLS and the RLS-DI revealed a relative frequency of RLS in HHT patients of 22% (95% CI: 18–27%). In fact, 8% (25/322; 95% CI: 5–11%) of the HHT patients had RLS which had not been diagnosed before. This equals 35% of the total amount of patients diagnosed with RLS (25/72; 95% CI: 25–46%). HHT patients with a history of gastrointestinal bleeding (prevalence ratio (PR) = 2.70, 95% CI: 1.53–4.77), blood transfusions (PR = 1.90, 95% CI: 1.27–2.86), or iron intake (PR = 2.05, 95% CI: 0.99–4.26) had an increased prevalence of RLS. Conclusions: Our data suggest that RLS is underdiagnosed in HHT. In addition, physicians should assess CIDA parameters for possible iron supplementation.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Restless Leg Syndrome Is Underdiagnosed in Hereditary Hemorrhagic Telangiectasia—Results of an Online Survey

Droege

Stang

Thangavelu

et al. 2021

JCM

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“…Kappa coefficient of agreement and 95% confidence interval. not the most appropriate therapeutic options in HHT patients (31) .…”

Section: Discussionmentioning

confidence: 99%

FID Score: an effective tool in Hereditary Haemorrhagic Telangiectasia - related epistaxis

Pagella¹,

Maiorano²,

Matti³

et al. 2020

Rhin

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Background: Hereditary haemorrhagic telangiectasia (HHT) is a rare disease characterized by a multisystemic vascular dysplasia and epistaxis, that is the most common cause of disability and social impairment. Patient management strictly depends on the severity of this symptom; therefore, it is of paramount importance for the clinicians to effectively grade epistaxis severity. The aim of this report was to validate the Frequency, Intensity and Duration score (FID) for grading epistaxis severity in patients with HHT; we studied repeatability and external validity comparing FID score with Epistaxis Severity Score (ESS). Methods: This is a descriptive, observational study that included 264 adult HHT patients with epistaxis. Diagnosis of HHT was established with Curaçao criteria or positivity at genetic testing. Nosebleed severity was evaluated according to the FID score and the ESS. The first 30 patients were included in the validation of the FID score, which was graded on days 0, 1, 3 and 7. In the remaining 234 patients, a comparison between the ESS and FID score was performed. Results: The statistical analysis performed in order to validate the FID score showed very good agreement between scores calculated on different days; analysis comparing the FID score with the ESS revealed a high correlation between the two grading systems. Conclusions: The FID score is a quick, easy and precise tool for evaluating HHT-related epistaxis and could be a possible alternative to the ESS. The FID score meets the need for an intuitive and smart grading system that is easy to manage in clinicians' hands.

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“…Epistaxis occurs more frequently in patients who are elderly, have co-morbidities like hypertension, are on antiplatelet therapy or have hereditary hemorrhagic telangiectasia (HHT). There are a few first aid measures recommended to patients to reduce the need of medical interventions and to support self-help, like applying pressure to the nose or nasal self-packing [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Effects of tranexamic acid on human nasal ciliary beat frequency

Behr

Horschke

Nastev

et al. 2021

Eur Arch Otorhinolaryngol

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Background Patients with recurrent epistaxis, particularly due to hereditary hemorrhagic telangiectasia (HHT) are recommended to apply topical tranexamic acid (TXA) to reduce bleeding events. Those patients may suffer ciliary dysfunction due to TXA’s effects on ciliary beating frequency (CBF) and their consequences. Methodology/principal Human nasal epithelial cells were harvested with a nasal brush in 30 healthy subjects. We investigated the CBF in RPMI medium using high-frequency video microscopy. TXA was added to the cells in various concentrations ranging from 2 to 5%, including the therapeutic concentration (2%) and a control (0%). Results CBF in the control condition was 6.1 ± 1.6 Hz. TXA reduces CBF in a time and concentration dependent manner, to, e.g. 4.3 ± 1.2 Hz with 2% TXA and 3.3 ± 0.9 Hz with 5% TXA after 16–20 min. The differences in CBF were statistically significant for all concentrations of TXA. Conclusions TXA has the potential to significantly impair nasal epithelial function. Therefore, frequent or regular topical nasal application of TXA should be done under close monitoring of nasal function, especially in patients with co-morbidities like chronic rhinosinusitis.

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Nasal self-packing for epistaxis in Hereditary Hemorrhagic Telangiectasia increases quality of life

Cited by 13 publications

References 27 publications

Restless Leg Syndrome Is Underdiagnosed in Hereditary Hemorrhagic Telangiectasia—Results of an Online Survey

Restless Leg Syndrome Is Underdiagnosed in Hereditary Hemorrhagic Telangiectasia—Results of an Online Survey

FID Score: an effective tool in Hereditary Haemorrhagic Telangiectasia - related epistaxis

Effects of tranexamic acid on human nasal ciliary beat frequency

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