A5055242632- Vascular Anomalies and Treatments
- Tracheal and airway disorders
- Vascular Malformations and Hemangiomas
- Sharing Economy and Platforms
- Shoulder Injury and Treatment
- Esophageal and GI Pathology
- Cardiac Valve Diseases and Treatments
- Pulmonary Hypertension Research and Treatments
- Connective tissue disorders research
Hôpital Femme Mère Enfant
2016-2025
Hospices Civils de Lyon
2014-2025
Université Claude Bernard Lyon 1
2016
Centre Hospitalier Vétérinaire Frégis
2014-2015
Laboratoire National de Référence
2014
Background Bevacizumab, an anti-VEGF monoclonal antibody, has recently emerged as a new option for severe forms of hereditary hemorrhagic telangiectasia (HHT). Its utilization in this orphan disease rapidly spread despite the lack randomized trials and international guidelines. The objective study is to report main clinical data (baseline characteristics, dose schedule, efficacy, adverse events deaths) HHT patients treated by intravenous bevacizumab France. Methods Retrospective...
<h3>Background</h3> Epistaxis is the most frequent and disabling manifestation of hereditary hemorrhagic telangiectasia (HHT). The efficacy intravenous bevacizumab (an anti–vascular endothelial growth factor monoclonal antibody) for epistaxis has been shown. However, intranasal yet to be evaluated. <h3>Objective</h3> To evaluate 3 different doses administered as a nasal spray in repeated manner duration nosebleeds patients with HHT. <h3>Design, Setting, Participants</h3> Randomized,...
Bevacizumab-a humanized monoclonal antibody-has been widely used to treat patients with hereditary hemorrhagic telangiectasia (HHT), but no randomized trial has yet conducted.This study is a double-blind multicenter phase 2 1:1 active-treatment-to-placebo ratio. We included over the age of 18 confirmed diagnosis and need for at least four red blood cell (RBC) units transfused in 3 months before enrollment. Bevacizumab was administered dose 5 mg/kg every 14 days total six injections. The...
Hereditary haemorrhagic telangiectasia (HHT) is a rare vascular multisystemic disease that leads to epistaxis, anaemia due blood loss, and arteriovenous malformations (AVMs) in organs such as the lungs, liver brain. HHT prevalence estimated at 1/6000, i.e. around 85,000 European citizens, served by Reference Network for Rare Multisystemic Vascular Diseases (VASCERN). treatments depend on clinical manifestations, span multiple different medical, surgical interventional disciplines. Separate...
Background: Hereditary hemorrhagic telangiectasia (HHT) is a dominantly inherited genetic vascular disorder in which epistaxis the most frequent manifestation, responsible for high morbidity. Management of this symptom has no standard, and local treatments are often aggressive. Their efficacy variable not been proven. Anti-angiogenic drugs, such as bevacizumab, new treatment strategy. Its systemic administration patients with HHT improves liver damage-related symptoms epistaxis. To limit...
Background: Hereditary haemorrhagic telangiectasia (HHT) is a genetic vascular disorder characterised by telangiectases, which cause nasal and gastrointestinal (GI) bleeding, visceral arteriovenous malformations. Since 2012 bevacizumab, monoclonal antibody targeting endothelial growth factor, has been promising treatment for HHT-related bleeding was evaluated in the phase II BABH study. Objective: To follow describe evolution treatments of patients with HHT post-BABH Design: This study...
Hereditary hemorrhagic telangiectasia (HHT), a genetic vascular disorder associated with epistaxis and hepatic shunts, is responsible for high-output cardiac failure in rare cases. Bevacizumab, which targets endothelial growth factor, was shown to decrease both index (CI) duration HHT patients severe liver involvement. The relationship between its serum concentration change CI investigated design the bevacizumab maintenance dosing regimen of future therapeutic studies. Twenty-five dyspnea...
To retrospectively describe a large series of pregnancies in women with hereditary haemorrhagic telangiectasia followed our reference centre, plus neonatal outcomes, to better understand the risks complications and improve their prevention.A retrospective descriptive study conducted through phone questionnaire.Reference centre for Lyon, France.Women meeting following criteria: (1) alive aged ≥18 years; (2) definite clinical and/or genetic diagnosis telangiectasia; (3) at least one full-term...
The purpose of this study was to compare ultra-low dose (ULD) and standard low-dose (SLD) chest computed tomography (CT) in terms radiation exposure, image quality diagnostic value for diagnosing pulmonary arteriovenous malformation (AVM) patients with hereditary hemorrhagic telangiectasia (HHT). In prospective board-approved consecutive HHT referred a reference center screening and/or follow-up CT examination were prospectively included from December 2020 January 2022. Patients underwent...
Abstract Hereditary hemorrhagic telangiectasia is a rare vascular genetic disease. Epistaxis the most frequent and disabling manifestation, timolol appears to be new therapeutic option as non-selective beta-blockers have in vitro vivo anti-angiogenic properties. Our main objective was evaluate efficacy of TIMOLOL nasal spray treatment for epistaxis hereditary telangiectasia. This study single-center, randomized, phase 2, double-blind placebo-controlled with an allocation ratio 1:1. It...
Hereditary hemorrhagic telangiectasia is a rare but ubiquitous genetic disease. Epistaxis the most frequent and life-threatening manifestation tacrolimus, an immunosuppressive agent, appears to be interesting new treatment option because of its anti-angiogenic properties. Our objective was evaluate, six weeks after end treatment, efficacy on duration nosebleeds tacrolimus nasal ointment, administered for patients with hereditary complicated by nosebleeds, we performed prospective,...
Purpose: To describe a five-grade classification of ectopia lentis in Marfan syndrome (MFS) and to evaluate the positive predictive value early grades lentis. Methods: We prospectively included MFS patients their healthy relatives. The anterior segment examination was classified into 0 5, we studied sensitivity, specificity, this classification. Results: Seventy-four thirty-six controls were examined. In group, 1, 2, 3, 4 present 15, 24, 17, 7 patients, respectively, whereas 11 group did not...
Epistaxis greatly affects patients with hereditary hemorrhagic telangiectasia (HHT). Although few systemic treatment exist, nintedanib, is a good candidate thanks to its anti-angiogenic activity. Our main objective was evaluate the efficacy of oral nintedanib on epistaxis duration in HHT moderate severe epistaxis. This multicenter phase 2 randomized, placebo-controlled, double-blind trial conducted between June 2020 and February 2023. Inclusion criteria were being over 18 years old having...
<title>Abstract</title> Epistaxis greatly affects patients with Hereditary Hemorrhagic Telangiectasia (HHT). Although few systemic treatment exist, nintedanib, is a good candidate thanks to its anti-angiogenic activity. Our main objective was evaluate the efficacy of oral nintedanib on epistaxis duration in HHT moderate severe epistaxis. This multicenter phase 2 randomized, placebo-controlled, double-blind trial conducted between June 2020 and February 2023. Inclusion criteria were being...