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

CONCLUSIONS: Our study showed that most patients with HHT using nasal self-packings could stop the bleeding after a while. Nasal self-packing is a user-friendly and secure method leaving patients more self-confident and independent. PMID: 30739126 [PubMed - as supplied by publisher]
Source: Rhinology - Category: ENT & OMF Tags: Rhinology Source Type: research

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This study was carried out in accordance with approval of the Melbourne Health and Walter and Eliza Hall Institute of Medical Research's Human Research Ethics Committee (approval number: 2013.081). All subjects gave written informed consent for participation and publication. Results and Discussion TGF-β signaling in NK cells is associated with: phosphorylation in SMAD2 and 3, inhibition of IL-15-induced metabolism/proliferation, simultaneous downregulation of CD44, CD49e, and Eomes, and upregulation of CD16 and CD49a expression (7, 10). SMAD family member 4 (SMAD4) belongs to the SMAD family of transcription...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
For people with a rare bleeding disorder called "hereditary hemorrhagic telangiectasia," or HHT, simple issues such as a nosebleed can be life-threatening. The disease has no cure. But Dr. Vivek Iyer, a Mayo Clinic pulmonary and critical care physician, found that a drug commonly used for cancer can stop the bleeding and restore quality of [...]
Source: News from Mayo Clinic - Category: Databases & Libraries Source Type: news
Pulmonary arteriovenous malformations (PAVMs) are rare and commonly caused by an autosomal dominant disorder known as hereditary hemorrhagic telangiectasia (HHT). HHT is an often-missed vascular disorder causing symptoms of epistaxis, mucosal telangiectasia, AVMs, hemoptysis, dyspnea, and iron deficiency anemia. Large AVMs in the lungs, liver, and brain can be life-threatening. Early diagnosis and intervention are imperative to prevent serious complications of massive hemoptysis, stroke, cerebral abscess, and gastrointestinal bleeding.
Source: The Journal for Nurse Practitioners - Category: Nursing Authors: Tags: Featured Article Source Type: research
Conditions:   Hereditary Hemorrhagic Telangiectasia;   Epistaxis Nosebleed;   Anemia Interventions:   Drug: Pazopanib;   Drug: Placebo oral capsule Sponsor:   Cure HHT Not yet recruiting
Source: - Category: Research Source Type: clinical trials
​Tranexamic acid (TXA) was invented by a Japanese husband-and-wife research team in the 1960s. Years earlier, this same research team had discovered epsilon-aminocaproic acid, a derivative and an analogue of the amino acid lysine. In their search for a more potent antifibrinolytic agent, they discovered tranexamic acid, a synthetic analog of the amino acid lysine. Tranexamic acid is eight to 10 times more powerful than epsilon-aminocaproic acid.The antifibrinolytic actions of TXA result from the binding of four or five lysine receptor sites on plasminogen. This binding prevents plasmin from binding to and degrading fibri...
Source: M2E Too! Mellick's Multimedia EduBlog - Category: Emergency Medicine Tags: Blog Posts Source Type: blogs
We report a female neonate with hematochezia on the 1st day of life secondary to multiple gastrointestinal arteriovenous malformations (AVMs) along with intracranial hemorrhage. We describe her clinical course and management, as well as her novel family mutation in ENG. This is the first reported HHT case with significant gastrointestinal bleeding in the newborn. We review neonatal HHT and raise the consideration for more directed prenatal imaging and delivery options for fetuses at high risk of HHT. [...] Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals: Table of contents&n...
Source: American Journal of Perinatology Reports - Category: Perinatology & Neonatology Authors: Tags: Case Report Source Type: research
Introduction: Hereditary hemorrhagic telangiectasia (HHT) is a rare hereditary multisystem vascular disorder causing visceral arteriovenous malformations (AVMs) and mucocutaneous bleeding. Chronic gastrointestinal bleeding and epistaxis frequently produce profound iron deficiency anemia refractory to conventional treatment. Patients with HHT have elevations in vascular endothelial growth factor (VEGF), so the anti-VEGF agent bevacizumab, a recombinant, humanized monoclonal IgG1 antibody that binds to and neutralizes circulating VEGF, is a promising systemic HHT therapy. Currently, data pertaining to the efficacy of bevaciz...
Source: Blood - Category: Hematology Authors: Tags: 102. Regulation of Iron Metabolism: Translating Iron Biology to the Clinic Source Type: research
In conclusion, we observed an improvement in Hgb and/or epistaxis in all treated patients. This occurred at a dose much lower than typically used for oncologic indications, with no serious AE. Further studies of pazopanib efficacy are warranted.
Source: Angiogenesis - Category: Molecular Biology Source Type: research
This study is designed to analyze the clinical features and treatment options for patients with HHT. Hospitalized patients with a definitive diagnosis of HHT from November 1973 to July 2016 in Peking Union Medical College Hospital were identified after reviewing medical records and electronic databases. Further follow-up data of these patients were collected from outpatient clinical visits and/or telephone interviews. We identified a total of 20 patients, 7 males and 13 females. The mean age was 42.4 ± 20.3 years. Epistaxis (18/20) was the most common presentation, followed by telangiectases of the oral buccal...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Observational Study Source Type: research
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