Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease affecting 1 in 5000 individuals. Epistaxis is seen in more than 90% of patients with HHT. Severe recurrent epistaxis can significantly decrease quality of life and may be resistant to standard treatment measures. Dysregulation of angiogenesis has been shown to cause the proliferation of abnormal blood vessels. As such, antiangiogenic treatments have been investigated including beta-blockers.
Objective: A systematic review of the efficacy of beta-blockers in topical treatment of epistaxis in patients with HHT based on epistaxis duration, frequency, and severity.
Methods: A systematic search was performed using the PubMed, Embase via Ovid, and Cochrane databases. The Preferred Items for Systematic Reviews and Meta-Analyses guidelines were followed. Studies that measured the efficacy of beta-blocker treatment of epistaxis in patients with HHT were included for qualitative analysis.
Results: Five studies (3 randomized controlled trials and 2 case series) with a total of 132 patients were included. Administration (systemically or topically via a spray or gel) of timolol and propranolol showed mixed evidence of improvement in epistaxis frequency, severity, and duration when compared with control groups. The evidence for propranolol appears more promising than timolol.
Conclusion: There are significant limitations in the included studies, and further investigation with larger longitudinal or randomized prospective trials is recommended. The available evidence suggests that beta-blocker treatment may have a positive effect on HHT-related epistaxis.
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http://dx.doi.org/10.1177/19458924221118131 | DOI Listing |
Rev Esp Enferm Dig
January 2025
Gastroenterology and Hepatology, Hospital Universitario Virgen de las Nieves, España.
Hereditary haemorrhagic telangiectasia (HHT) is a rare genetic disease characterised by mucocutaneous telangiectasias and arteriovenous malformations that can affect multiple organs. Although rare, ischaemic cholangiopathy can occur, a serious complication that can even lead to death. We present the case of a patient with HHT disease with previous mucocutaneous and gastrointestinal manifestations in whom 8 weeks after cholecystectomy a saccular dilatation of the intrahepatic bile duct was observed.
View Article and Find Full Text PDFActa Neurol Belg
January 2025
Intensive Care Department, Cliniques Universitaire Saint-Luc (CUSL), Université Catholique de Louvain (UCL), Brussels, Belgium.
Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia (HHT), is a rare vascular disorder characterized by arteriovenous malformations (AVMs) in various organs, including the lungs. Pulmonary AVMs (PAVMs) are especially worrisome due to their potential to form right-to-left shunts, resulting in life-threatening complications such as paradoxical embolism and stroke . We present a case of fatal air embolism in a young patient with a known history of HHT and recurring hemoptysis.
View Article and Find Full Text PDFPediatr Pulmonol
January 2025
Department of Pulmonology, St. Antonius Hospital, Nieuwegein, the Netherlands.
Introduction: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disease and screening to detect pulmonary arteriovenous malformations (PAVMs) is important to prevent complications. In adults, transthoracic contrast echocardiogram (TTCE) is used to screen PAVMs. In children, a conservative screening method seems to be sufficient to rule out major PAVMs and prevent them from PAVM-related complications.
View Article and Find Full Text PDFJ Clin Med
December 2024
Clinic of Diagnostic and Interventional Radiology, Saarland University Medical Center, 66421 Homburg, Germany.
The aim of this study was to evaluate patients with hereditary hemorrhagic telangiectasia (HHT) for the potential reperfusion of pulmonary arteriovenous malformations (PAVM) treated by catheter embolization using coils or embolization plugs and to analyze causes of possible reperfusion in order to further improve treatment. This retrospective study analyzed the data of 345 patients who underwent screening for pulmonary arteriovenous malformations in cases of suspected or confirmed HHT (Osler's disease). Of these, 118 patients with PAVM that underwent catheter embolization and had at least one follow-up study were included in our study and evaluated for potential reperfusion.
View Article and Find Full Text PDFBiomedicines
December 2024
Division of Hematology, Department of Internal Medicine, University Hospital Dubrava and Medical School, University of Zagreb, 10000 Zagreb, Croatia.
Obinutuzumab was approved for front-line treatment of chronic lymphocytic leukemia in combination with chlorambucil pulses administered every 2 wks. Alternative schedules of chlorambucil enable the administration of higher total chlorambucil doses, and have better antileukemia activity. So far, evidence on the feasibility of combining obinutuzumab with alternative chlorambucil schedules is lacking.
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