How to manage patients with hereditary haemorrhagic telangiectasia
| Autor: | Urban W. Geisthoff, Ha-Long Nguyen, U.T. Seyfert, Alexander Röth |
|---|---|
| Rok vydání: | 2015 |
| Předmět: |
Intracranial Arteriovenous Malformations
medicine.medical_specialty Gastrointestinal bleeding Percutaneous medicine.drug_class Hypertension Pulmonary Medizin Telangiectases Hemostatics Arteriovenous Malformations Fibrinolytic Agents Transforming Growth Factor beta medicine Humans Thrombophilia Antibiotic prophylaxis Lung Hereditary haemorrhagic telangiectasia Anemia Iron-Deficiency Neovascularization Pathologic business.industry Molecular genetic testing Vascular malformation Anticoagulant Anticoagulants Disease Management Hematology Antibiotic Prophylaxis medicine.disease Embolization Therapeutic Surgery Epistaxis Liver Telangiectasia Hereditary Hemorrhagic Radiology business Gastrointestinal Hemorrhage Signal Transduction |
| Zdroj: | British journal of haematology. 171(4) |
| ISSN: | 1365-2141 |
| Popis: | Hereditary haemorrhagic telangiectasia is a rare systemic autosomal dominantly inherited disorder of the fibrovascular tissue with a wide variety of clinical manifestations. Diagnosis is based on the clinical Curacao criteria or molecular genetic testing. Dilated vessels can develop into telangiectases or larger vascular malformations in various organs, calling for an interdisciplinary approach. Epistaxis and gastrointestinal bleeding can result from these vascular defects. Various conservative and interventional treatments have been described for these conditions. However, no optimal therapy exists. Treatment can become especially difficult due to progressive anaemia or when anticoagulant or anti-thrombotic therapy becomes necessary. Screening for pulmonary arteriovenous malformations (PAVM) should be performed in all confirmed and suspected patients. Treatment by percutaneous transcatheter embolotherapy and antibiotic prophylaxis is normally effective for PAVM. Cerebral or hepatic vascular malformations and rare manifestations need to be evaluated on a case-by-case basis to determine the best course of action for treatment. |
| Databáze: | OpenAIRE |
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