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Background: Hereditary hemorrhagic telangiectasia (HHT) is characterized by extensive telangiectasias and arteriovenous malformations. The primary clinical manifestation is epistaxis that results in iron-deficiency anemia and reduced health-related quality of life., Methods: We conducted a randomized, placebo-controlled trial to evaluate the safety and efficacy of pomalidomide for the treatment of HHT. We randomly assigned patients, in a 2:1 ratio, to receive pomalidomide at a dose of 4 mg daily or matching placebo for 24 weeks. The primary outcome was the change from baseline through week 24 in the Epistaxis Severity Score (a validated bleeding score in HHT; range, 0 to 10, with higher scores indicating worse bleeding). A reduction of 0.71 points or more is considered clinically significant. A key secondary outcome was the HHT-specific quality-of-life score (range, 0 to 16, with higher scores indicating more limitations)., Results: The trial was closed to enrollment in June 2023 after a planned interim analysis met a prespecified threshold for efficacy. A total of 144 patients underwent randomization; 95 patients were assigned to receive pomalidomide and 49 to receive placebo. The baseline mean (±SD) Epistaxis Severity Score was 5.0±1.5, a finding consistent with moderate-to-severe epistaxis. At 24 weeks, the mean difference between the pomalidomide group and the placebo group in the change from baseline in the Epistaxis Severity Score was -0.94 points (95% confidence interval [CI], -1.57 to -0.31; P = 0.004). The mean difference in the changes in the HHT-specific quality-of-life score between the groups was -1.4 points (95% CI, -2.6 to -0.3). Adverse events that were more common in the pomalidomide group than in the placebo group included neutropenia, constipation, and rash., Conclusions: Among patients with HHT, pomalidomide treatment resulted in a significant, clinically relevant reduction in epistaxis severity. No unexpected safety signals were identified. (Funded by the National Heart, Lung, and Blood Institute; PATH-HHT Clinicaltrials.gov number, NCT03910244)., (Copyright © 2024 Massachusetts Medical Society.)

Hereditary hemorrhagic telangiectasia (HHT) is characterized by arteriovenous malformations and telangiectasia, with primary clinical manifestations of epistaxis and gastrointestinal bleeding and resultant anemia. HHT negatively affects health-related quality of life (HR-QoL); however, existing tools to measure HR-QoL are not HHT specific. Our objective was to develop an HHT-specific HR-QoL (HHT-QoL) instrument and evaluate its performance in a cross-sectional survey of individuals with HHT. Four HHT-specific questions were developed to evaluate the impact of HHT on productivity and social and personal interactions. An anonymous e-mail survey was conducted through Cure HHT. Participants also indicated their perceived HHT severity and completed 3 Patient-Reported Outcomes Measurement Information System (PROMIS) questionnaires: Discretionary Social Activities, Social Roles, and Emotional Distress. Complete data were available for 290 participants who self-identified their HHT severity as mild (29%), moderate (46%), or severe (25%). The HHT-QoL scale was reliable (Cronbach's-α, 0.83). Principal components analysis indicated the instrument was unidimensional. Participants had low levels of QoL with their ability to participate in discretionary social activities (PROMIS mean 36.4 [standard deviation 14.3]) and perform in social roles (41.5 [17.2]), and the presence of a high level of emotional distress (64.8 [24.2]). The HHT-QoL score correlated negatively with PROMIS Discretionary Social Activities (r = -0.65) and Social Roles (r = -0.68) and positively correlated with PROMIS Emotional Distress (r = 0.51). In conclusion, the 4-item HHT-QoL instrument provides valuable insight and may be a useful addition to future clinical research in HHT., (© 2022 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)

Background: There are very few studies about general quality of life parameters, standards for the description of health status and comparison with general population data on patients with Hereditary hemorrhagic telangiectasia (HHT), a rare disease in which epistaxis is a cardinal symptom., Purpose: To assess the quality of life in a population of Spanish patients with HHT and compare it with the general population., Design and Methods: Between January 1 st 2005 and December 31 st 2013, 187 adult patients diagnosed with HHT who were admitted to the HHT Unit of the Hospital Sierrallana, completed on their first visit, the EuroQol 5D-3L (five dimensions and three levels) quality of life descriptive test and the visual analog scale (VAS). The numerical social index value was also determined and the subjective effect of the nasal epistaxis on their quality of life was estimated classified as mild, moderate or severe., Results: Patients with HHT had greater problems than the general population in the five dimensions of the EuroQol 5D-3L, particularly considering pain/discomfort and anxiety/depression. In the VAS and the social index value, patients with HHT also scored lower than the general population, particularly older patients, males, and patients with HHT2. They also had values similar to those of populations with chronic illnesses. The subjective perception of the severity of epistaxis correlated strongly with the VAS and social index values., Conclusions: The quality of life of patients with HHT, estimated using the EuroQol 5D-3L scale, is affected across all dimensions. The scores are similar to those seen in cases of other chronic diseases. Older patients, males and the carriers of the ACVRL1 mutation generally have worse scores on these scales. The VAS and the social index value are index that correlate well with the severity of the clinical symptoms associated mainly with epistaxis.

Epistaxis is one of the most frequently encountered emergency problems in our country, as it is throughout the world. In epistaxis, the benefits of implementing clinical guidelines are evident in both daily practice and medical education. In this article, the authors aimed to present their approach to adult patients with epistaxis and to share their experience, which considered the life quality of the patients to be at the forefront, based on some of their patients. In management of epistaxis, the implementation of the simplest method, minimally affecting the life quality of the patient, may be easier with a stepped approach. The authors consider that the stepped approach that they have implemented and presented in their study may lead to using less invasive methods and may accordingly improve the life quality of the patient.

Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease mainly characterized by epistaxis in more than 96% of patients. Recently, a validated questionnaire known as the HHT Epistaxis Severity Score (ESS) was developed. However, little is known about the relationship between epistaxis and quality of life. We hypothesize that epistaxis severity is a major factor predicting health-related quality of life (HR-QoL) in HHT patients., Methods: This is a cross-sectional study. The ESS questionnaire and Medical Outcomes Study 36-item short form (SF-36) were administered to subjects through an Internet survey. All participants had a definitive diagnosis of HHT through Curaçao criteria or genetic testing. Demographic information, genetics, and extensive histories were also collected. Descriptive analyses were performed with calculations of means and standard deviations (SDs) for continuous variables and proportions for categorical variables. Linear regressions were then performed to assess the association between HR-QoL and ESS., Results: A total of 604 subjects participated between April and August 2008. All patients reported epistaxis, 285 (47.2%) had telangiectasias, and 545 (90.2%) had a family history of HHT; 167 (27.6%) patients had mild epistaxis (ESS <4), 285 (47.2%) reported moderate epistaxis (≥4 ESS <7), and 152 (25.2%) reported severe epistaxis (ESS ≥7). Patients with severe epistaxis had lower scores for both the Physical Component Summary (PCS) and the Mental Component Summary (MCS) of HR-QoL when compared to those with mild epistaxis (p < 0.001, p < 0.001)., Conclusion: The ESS is a major determinant of HR-QoL and should be considered as a measurement of treatment efficacy in HHT-related epistaxis., (© 2014 ARS-AAOA, LLC.)

Since 50-60% of the human body consists of water, the physiology of blood circulation might be affected by a full moon differently than during other days of the month. This study analyzed a potential association among lunar phases, seasonal variations, and the rate of spontaneous epistaxis. Consecutive admissions solely for spontaneous epistaxis to an otolaryngology emergency room of a single medical center during 1 year were evaluated. The applied tests failed to show changes of admissions during different lunar phases. According to multiple comparisons, admissions were significantly less frequent during the summer months, similar to findings from other countries of the northern hemisphere with varying climates, suggesting an influence of circannual rhythms rather than of environmental conditions.

Objectives/hypothesis: To identify factors influencing the severity of epistaxis in hereditary hemorrhagic telangiectasia (HHT)., Study Design: Participants with and without HHT were recruited from a specialist service and online following advertisement by the HHT Foundation International. Both groups were asked to complete a nonbiased questionnaire., Methods: The reported effects of specific treatments or lifestyle factors on epistaxis were assigned positive values if beneficial, negative values if detrimental, or zero if "no difference" and were summed to enable statistical analysis., Results: Epistaxis affected 649 of 666 (97%) participants with HHT and was significantly more frequent than in control participants. Specialist invasive treatments were reported as beneficial, laser therapy more frequently than cauterization. Medical treatments commonly used for HHT epistaxis (female hormones, antiestrogens, tranexamic acid, aminocaproic acid, nasal creams, and bevacizumab) also had significantly positive (beneficial) scores. Lifestyle and dietary factors were generally detrimental, but room humidification, nasal lubrication, and saline treatments were all reported as beneficial (95% confidence intervals greater than zero). Multiple food items were volunteered as being detrimental to epistaxis. The most frequently reported items were alcohol (n = 45; 6.8% of participants) and spices (n = 26, 3.9% of participants). Remaining foods reported to exacerbate epistaxis were also found to be high in salicylates (including red wine, spices, chocolate, coffee, and certain fruits), natural antiplatelet activity (garlic, ginger, ginseng, ginkgo biloba, and vitamin E15), or omega-3 acids (oily fish, salmon)., Conclusions: This study supports existing treatments and suggests lifestyle and dietary maneuvers that may also improve nosebleeds in HHT., Level of Evidence: 2c., (Copyright © 2013 The American Laryngological, Rhinological, and Otological Society, Inc.)

Objective: Recurrent epistaxis as a manifestation of hereditary hemorrhagic telangiectasia (HHT) is usually difficult to control. Although no treatment is regarded to be completely efficacious, nostril closure is considered a modality of choice for the most severe cases. The cessation of airflow resulting from this procedure can stop bleeding by minimizing risk factors. However, loss of nasal functions is a disadvantage of nostril closure. We conducted a questionnaire survey of patients who underwent nostril closure surgery, regarding the effects and disadvantages of the operation., Methods: Seven patients were asked questions on issues including frequency and severity of epistaxis pre- and post-operatively, satisfaction of treatment, and impairment in daily living activities., Results: Most patients reported complete cessation of bleeding. Some still had bleeding, but the frequency and severity were far lower. No transfusions were required in any of the cases. Patients reported some disadvantages, for example, respiratory, olfactory, and phonatory issues. Six out of seven patients were very satisfied with the outcome of surgery., Conclusion: Nostril closure surgery can remarkably reduce frequency and volume of epistaxis. Our survey indicated that satisfactory results were achieved. However, difficulties caused by complete nasal obstruction varied. Thus, individualized coping strategies are required., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Purpose: Extensive nasal polyposis could involve the middle turbinate inducing the surgeon to partially remove it. We initiated this retrospective study to evaluate the effect of a partial middle turbinectomy (PMT) on postoperative epistaxis and if sphenopalatine artery ligation (SPAL) could reduce the risk of bleeding in patients without nasal packing., Material and Methods: Twenty-seven patients with extended bilateral nasal polyposis and submitted to primary functional endoscopic sinus surgery (FESS) with PMT on 40 sides were retrospectively selected. Postoperative bleeding and other complications were evaluated and compared with those of a control group of 27 patients who underwent FESS with middle turbinate preservation on 40 sides. The study group was furthermore divided into 2 groups according to the execution of SPAL. The incidence of postoperative bleeding of both groups and of the 2 parts of the study group was compared using the Fisher exact test., Results: A SPAL was necessary to stop intraoperative bleeding in 21 (52.5%) sides of the study group patients and in 7 (17.5%) of the control group patients. After surgery, epistaxis occurred in 8 cases (20%) in the PMT group (1 submitted to SPAL) and in 2 (5%) of the control group. The comparison with the Fisher exact test confirmed the major tendency of postoperative bleeding in the study group and in those not submitted to SPAL (P < .05)., Conclusions: Partial middle turbinectomy causes a higher incidence of postoperative bleeding in patients who are not packed during the FESS operation. The execution of SPAL greatly reduces this risk., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Objective: To examine the effects of oral diazepam on blood pressure and anxiety in patients with acute epistaxis., Study Design and Setting: A prospective comparative study in an otorhinolaryngology tertiary referral centre., Participants: Patients with acute epistaxis requiring hospital admission., Intervention: Oral diazepam., Main Outcome Measures: Anxiety and blood pressure levels., Results: 32 patients received diazepam and 45 did not (control). On average, patients were hypertensive on admission (mean [standard deviation (SD)] systolic blood pressure diazepam group=157 mmHg [26], control=152 mmHg [23]; diastolic blood pressure diazepam group=87 mmHg [16], control=87 mmHg [18]). Both groups showed significant blood pressure reduction on discharge (p<0.0001) but the difference in mean blood pressure reduction between the two groups was insignificant (systolic blood pressure p=0.16, 95% confidence interval [CI]=-5 to +19 mmHg; diastolic blood pressure p=0.43, 95% CI=-8 to +10 mmHg). Anxiety was significantly lower on discharge (p<0.0001) but the difference in mean fall in anxiety scores between the two groups was insignificant (p=0.08, 95% CI=0 to +2). There was no significant correlation between total diazepam and changes in blood pressure (systolic blood pressure p=0.32; diastolic blood pressure p=0.65) or anxiety (p=0.73), nor between blood pressure and anxiety on admission (systolic blood pressure p=0.45; diastolic blood pressure p=0.72)., Conclusions: Elevated blood pressure and anxiety in acute epistaxis patients reduced on epistaxis resolution irrespective of oral diazepam use. The elevated blood pressure does not appear to be directly related to anxiety.

Background: Epistaxis often causes significant anxiety in both patients and clinicians. Various methods of treatment have been advocated. The aim of this paper is to document the pattern of epistaxis in Port Harcourt, determine the etiologies and advocate the best treatment option., Methods: This is a 6-year retrospective study of patients that were managed for epistaxis in the department of Otorhinolaryngology of the University of Port Harcourt Teaching Hospital, Port Harcourt, from January 2000 to December, 2005. The material resources were the records of the Otorhinolaryngology outpatient clinic and Accident & Emergency department of the hospital. These were updated with records from the ward admissions and theatre. The diagnosis of epistaxis was based on clinical history, physical findings, laboratory and radiological investigations with examination under anaestheasia of the nose, nasopharynx and biopsy., Results: There were 30 patients, 16 males and 14 females with a mean age of 30.48 years +/- 15.14 [range 1-70 years]. The most commonly affected age group was 31-40 years. Idiopathic causes accounted for 66.67%, trauma 13.33%, nasopharyngeal carcinoma 10%, chronic maxillary sinusitis 6.67%, and vicarious menstruation 3.33%. The right nasal cavity was more affected than the left. The most commonly used method of treatment was anterior nasal packing (50%). Posterior nasal packing accounted for 26.67% while chemical cauterization was done in 13.3% of cases., Conclusion: The pattern of epistaxis in our study agrees with the pattern found elsewhere. Anterior and posterior nasal packing were cost effective methods of treatment.

Background and Objective: Hereditary hemorrhagic telangiectasia (HHT) is an inherited autosomal dominant vascular disease, which can cause multiple symptoms, the cardinal one being chronic recurrent nosebleeds. The aim of this study was to determine, whether these chronic recurrent episodes are felt by the patients to be merely isolated episodes or affect their quality of life (QoL). Also assessed was the extent to which QoL is more impaired in patients with HHT than in those with other chronic diseases., Patients and Methods: This prospective cross-sectional study was based on a German questionnaire, "Profile of Quality of Life of Chronically Ill Patients"(PLC) together with a newly formulated questionnaire dealing with HHT-specific symptoms. Comparisons were made with German normative reference data, as well as with data from randomly selected patients with various chronic diseases., Results: 90% of patients with HHT considered their recurrent nosebleeds to be the most disturbing symptom, interfering with hobbies and leisure time in 63% of them. Those with HHT had worse results in 5 of 6 PLC scales than those with other chronic diseases such as cardiomyopathy or rheumatic disease. 73% of the patients stated that they would appreciate new ways of treating these nosebleeds., Conclusion: There is a great need for optimizing the treatment of chronic recurrent nosebleeds. Lasting reduction of such bleeds by new therapeutic methods seems unlikely to happen in the near future. For this reason multimodal treatment options, including psychological and relaxation techniques, should be considered. Such an approach may reduce the stress on patients (and subsequent nosebleeds) and result in a direct improvement of their quality of life.

The purpose of this study was to describe the results of Nd:YAG laser application in hereditary hemorrhagic telangiectasia (HHT) patients and to measure the Health-Related Quality of Life (HR-QoL) in patients with HHT before and after Nd:YAG laser application in a prospective, clinical trial at a university hospital. Twenty-seven consecutive patients with HHT and mild to moderate degrees of epistaxis were followed-up for 2 years after Nd:YAG laser treatments. Recurrence of epistaxis after Nd:YAG laser application and measurement of HR-QoL using the International Quality of Life Assessment questionnaire, version 1.1 (IQOLA 1.1), was found. Eight patients (30%) received only one Nd:YAG laser treatment, 15 (56%) had a recurrence and received a second treatment and 4 (14%) had two recurrences and received three Nd:YAG laser treatments. HR-QoL was improved 2 years after the first Nd:YAG laser application in both its Physical Health Dimension (47.5+/-2.9 vs. 38.1+/-2.3 before treatment, P <0.05) and Mental Health Dimension (45.1+/-2.7 vs. 39.6+/-2.4 before treatment, P <0.05). Although no curative treatment for HHT exists, Nd:YAG laser treatment seems to constitute a simple and efficient method of epistaxis control, resulting in a significant improvement in quality of life.

The religious beliefs of Jehovah's Witnesses forbid them to receive blood or blood products. We report a case of life-threatening epistaxis in a Jehovah's Witness, causing severe anaemia (Hb 2.7 g/dl). The successful management of this case and literature relating to this are discussed.

Epistaxis is the most common otolaryngologic emergency requiring hospital admission. Patients with this disorder are usually managed conservatively beginning with the simplest mode of treatment with surgical arterial ligation being reserved for cases of failed nasal packing. The purpose of this retrospective review was to evaluate the efficacy of arterial ligation and document all of the complications secondary to these procedures, especially the minor complications secondary to transantral internal maxillary artery (IMAX) ligation. A total of 402 patients admitted to the Vancouver General Hospital between 1980-1990 with a primary diagnosis of epistaxis were the basis for this review. The hospital charts of those patients who underwent arterial ligation were reviewed. A phone questionnaire was directed to this group. External carotid artery (ECA) ligation was associated with a high rate of rebleeding (9/20-45%) during the 10 year follow-up period. IMAX ligation was an effective procedure for controlling epistaxis with a small number of rebleeds (3/29-10%). Although few major complications were noted in the IMAX ligation group, frequent minor complications were noted on prolonged follow-up. Despite this, patient satisfaction was very good in the IMAX ligation group.

Potentially life threatening self-mutilation of the nose in a patient with a severe passive aggressive character disorder is described. The phenomenological and psychodynamic features of this case are contrasted with those of schizophrenia and factitious dermatoses with similar excoriations.

Epistaxis is discussed. Three unusual cases are reported. The first was a patient with a basal skull fracture due to a motor vehicle accident. He developed a carotid-cavernous fistula causing epistaxis severe enough to cause a cardiac arrest on one of his bouts of severe bleeding. The second was an unusual complication of a rhinoplasty due to damage to the anterior ethmoidal artery from the medial osteotomy. The third was due to psychopathology causing an adult female to traumatize her nose as an attention-getting or defence mechanism causing epistaxis with anemia requiring bi-annual blood transfusions of more than 175 units of blood over a 20 year period. Management is described.