4. Laryngoscope, 128:2237-2244, 2018.

Background: Epistaxis is the most common symptom in patients with hereditary hemorrhagic telangiectasia (HHT), with the greatest negative impact on quality of life (QoL). Repeated intranasal submucosal bevacizumab injections (RISBI) is a relatively new treatment option for moderate or severe grades of epistaxis in HHT. However, the effect of RISBI on QoL is not fully evaluated.Study Design: Prospective, non-comparative study. Materials and Methods: Patients treated by RISBI for HHT-associated epistaxis between June 2011 and August 2013 were prospectively invited to the present study. The end of follow-up was October 2013. The patients were requested to answer QoL questionnaires before the first treatment, and 6-8 weeks after the last treatment. Three levels of QoL were assessed: Overall QoL using Cantril's Self-Anchoring Ladder; Health-related QoL using Short Form 36 (SF-36), and Disease-specific QoL. Psychological distress was measured with the Hospital Anxiety and Depression scale (HADS).Results: Thirty-three patients were treated with RISBI during the period referred to above. Twenty-three patients completed the QoL questionnaires. The average number of treatments per patient was 2.15 AE 1.3 (Range: 1-5). The mean overall QoL improved from 6.47 AE 1.9 to 7.26 AE 1.6 (P < .05). Several dimensions measured by SF-36 were significantly improved with a medium to strong effect size. HADS demonstrated a significant decrease in psychological distress after the last treatment.Conclusion: HHT patients treated by RISBI improved in several aspects of quality of life, and psychological distress decreased. RISBI was an effective treatment option for moderate and severe grades of HHT-associated epistaxis.

Intranasal bevacizumab injections have been used in treating hereditary hemorrhagic telangiectasia (HHT)-related epistaxis since 2009. It is believed to be a safe and effective treatment for a selected group of HHT patients in reducing frequency and intensity of epistaxis, with few or none adverse effects. In this case report, however, we will describe a patient who developed bilateral osteonecrosis in the knees while undergoing regular intranasal submucosal bevacizumab injections. Although osteonecrosis previously has been documented in patients receiving bevacizumab intravenously in oncologic doses, thus far it has not been reported in patients treated with intranasal submucosal injections. Laryngoscope, 128:593-596, 2018.

Objectives/Hypothesis We aimed to investigate if vascular endothelial growth factor (VEGF) and other angiogenic and inflammatory factors correlated with the clinical presentation in hereditary hemorrhagic telangiectasia (HHT) patients, particularly in regard to the severity of epistaxis. Study Design Prospective, comparative, single‐center study. Methods One hundred nine samples were collected from 75 HHT patients attending the ear, nose, and throat department at Oslo University Hospital from February 2012 to August 2013. For comparison, samples were collected from 16 healthy controls. Angiogenic and inflammatory factors related to endothelial cell activation were analyzed by enzyme immunoassays. The grade of epistaxis was evaluated using the Epistaxis Severity Score and epistaxis Intensity, Frequency, and Need for Blood Transfusion score at the day of blood sampling. The presence of internal organ manifestations in the HHT group was recorded. Results Pentraxin 3 (PTX3) was the only factor that was significantly higher in the HHT patients than the controls and showed significant correlation to the epistaxis severity grade and the hemoglobin level. The VEGF level was higher in the HHT patients compared to controls but not to a significant degree. In addition, a significant correlation of the level of VEGF and the grade of epistaxis could not be observed. Also, no significant correlations were observed between the presence of internal organ manifestations and the level of angiogenic factors. Conclusions PTX3, at least partly reflecting vascular inflammation, can be a potential biomarker for the severity of HHT associated epistaxis. The serum level of VEGF was not correlated with the severity of epistaxis in the HHT patients. Level of Evidence 2 Laryngoscope, 129:E44–E49, 2019

BACKGROUND: Different institutions use different grading systems for hereditary haemorrhagic telangiectasia (HHT)-associated epistaxis. It is important to have a universal, standardized grading system to compare and evaluate the effectiveness of different treatment options. We introduced the “Intensity, Frequency and need for Blood Transfusion” (IFT) grading system for HHT-associated epistaxis in 2008. Hoag et al. proposed the “Epistaxis Severity Score” (ESS) for the International HHT foundation in 2010. This study aimed to evaluate the potential correlation between the ESS and IFT grading systems. METHODS: The study included 354 simultaneous reports using the IFT and ESS from 106 patients. The correlation between the ESS, IFT and haemoglobin levels was measured using Pearson’s correlation coefficient. The ESS and IFT were scored simultaneously by the patient and doctor in 48 cases to evaluate if there was a discrepancy in the scoring applied by either set of responders. RESULTS: The measured correlation between the two grading systems was good (0.75). The grade of epistaxis reported by patients and doctors respectively showed no significant difference. Both the IFT and ESS grading systems correlate significantly to the haemoglobin level. CONCLUSIONS: Both the IFT and ESS scores correlate to each other, and their results are comparable. Whether the IFT or ESS scoring was performed by the patient or doctor had no significant impact.

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