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TUESDAY, Sept. 8, 2020 (HealthDay News) — In a second edition of international guidelines, published online Sept. 8 in the Annals of Internal Medicine<\/i>, updated recommendations are presented for the diagnosis and management of hereditary hemorrhagic telangiectasia (HHT).<\/p>\n
Marie E. Faughnan, M.D., from St. Michael’s Hospital in Toronto, and colleagues developed guidelines for the management and prevention of HHT-related symptoms and complications. Recommendations were generated in six priority topic areas: epistaxis, gastrointestinal bleeding, anemia and iron deficiency, liver vascular malformations (VMs), pediatric care, and pregnancy and delivery; anemia, pediatrics, and pregnancy and delivery were new areas for guidance.<\/p>\n
The authors recommend moisturizing topical therapies that humidify the nasal mucosa to reduce epistaxis; oral tranexamic acid should be considered for management of epistaxis that does not respond to moisturizing topical therapies. Esophagogastroduodenoscopy is recommended as the first-line diagnostic test for suspected HHT-related bleeding. The severity of HHT-related gastrointestinal bleeding should be graded as mild, moderate, or severe. All adults should be tested for iron deficiency and anemia, as should all children with recurrent bleeding and\/or symptoms of anemia. Screening for liver VMs should be offered to all adults with definite or suspected VMs. Asymptomatic children of a parent with HHT should be offered genetic testing. HHT should be diagnosed using the Cura\u00e7ao criteria or by identification of a causative mutation. Patients with possible or definite HHT should be screened for brain and pulmonary VMs.<\/p>\n
“These recommendations should facilitate implementation of key components of HHT care into clinical practice,” the authors write.<\/p>\n