ITEMS IN AFP WITH MESH TERM:
ABSTRACT: Hereditary hemorrhagic telangiectasia is an uncommon autosomal dominant disease that occurs in approximately one in 5,000 to 8,000 persons. This multisystem disorder can affect the nose, skin, gastrointestinal tract, lungs, liver, and brain. Epistaxis is the most common presenting problem, occurring in 90 percent of affected patients. Approximately 15 to 30 percent of patients with hereditary hemorrhagic telangiectasia will have an arteriovenous malformation in the lungs and more than 10 percent will have one in the brain. The symptoms of hereditary hemorrhagic telangiectasia are often unrecognized. Many patients, even those with affected family members, may go undiagnosed. Hereditary hemorrhagic telangiectasia is a clinical diagnosis that is based on the presence of three of four criteria (i.e., epistaxis, telangiectasias, visceral arteriovenous malformations, or family history of the disease). Screening and treatment recommendations have been created in an attempt to limit the morbidity and mortality associated with this disease. Patients with confirmed or suspected hereditary hemorrhagic telangiectasia should be screened for brain and lung arteriovenous malformations using magnetic resonance imaging of the brain and contrast echocardiography. Pulmonary arteriovenous malformations can be treated with embolization. Patients with a history of pulmonary arteriovenous malformations or those who have not been screened should use antibiotic prophylaxis before dental treatment, endoscopy, or other procedures that could cause bacteremia because of the risk of paradoxical brain embolism or infection.
ABSTRACT: Age-related macular degeneration is the leading cause of severe vision loss among the elderly. In this condition, central vision is lost, but peripheral vision almost always remains intact. Affected persons rarely require canes or guide dogs. The diagnosis of age-related macular degeneration is based on symptoms and ophthalmoscopic findings, and the disease can be classified into atrophic and exudative forms. The two currently proven treatments are laser photocoagulation and photodynamic therapy, but these measures are effective in only a small fraction of eyes with the exudative form of macular degeneration. Vision rehabilitation can help patients maximize their remaining vision and adapt so that they can perform activities of daily living. Families need encouragement in providing support and helping patients adjust to being partially sighted.