Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Angioid streaks are asymptomatic breaks in Bruch's membrane developing later in life. Secondary macular degeneration and other fundus abnormalities often accompany their development. Angioid streaks are frequently associated with systemic diseases such as pseudoxanthoma elasticum, Paget's disease of bone, and the sickle cell hemoglobinopathies. The clinical manifestations of angioid streaks, related fundus changes, and these three systemic disorders are discussed as well as principles of treatment and management. Two case reports are presented which illustrate some of the important characteristics of angioid streaks.
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PMID:Diagnosis and management of angioid streaks. 318 86

Angioid streaks are often associated with a systemic condition, most frequently pseudoxanthoma elasticum, Paget's disease of the bone, or one of the sickle hemoglobinopathies. The clinical manifestations of angioid streaks and those three systemic conditions are reviewed. A diagnostic survey is suggested for patients discovered to have angioid streaks with no known systemic disease. The results of such a survey in 50 patients are presented. In addition, separate studies of patients with Paget's disease of the bone (50 patients) and of the sickle hemoglobinopathies (100 patients) are described, and the characteristics of patients with angioid streaks as well as the incidence of streaks in these conditions is reviewed. The histopathologic and fluorescein angiographic characteristics of angioid streaks, as well as the possible benefit of photocoagulation for complications of angioid streaks is discussed.
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PMID:Angioid streaks. 704 15

Angioid streaks were observed in two patients with abetalipoproteinemia. The progression of the angioid streaks was minimal over the years that these patients received vitamin A and E supplementation, though in one patient the development of subretinal neovascular membranes within the angioid streaks was the cause of rapid central visual loss. The simultaneous appearance of two rare entities in unrelated individuals strengthens the relationship between these two disorders that has been suggested by previous case studies. The authors propose a common metabolic pathway involving trace element deficiencies that may account for this relationship as well as the association of angioid streaks with other rare disorders such as Paget's disease, hypoparathyroidism, lead poisoning, hyperphosphatemia, and a number of hemoglobinopathies. Their study of these two patients underscores the need for further investigations as to the role of copper, zinc and omega-3 fatty acids in the pathogenesis of retinopathy in abetalipoproteinemia.
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PMID:Angioid streaks associated with abetalipoproteinemia. 774 70

Angioid streaks were first described by Doyne in 1889. Since that time histopathology and diagnostic methods have been greatly improved. Angioid streaks of the fundus are not apparent at birth. The earliest form is known as "peau d'orange". The end stage is disciform macular degeneration, helicoid peripapillary atrophy or diffuse choroidal sclerosis. Moreover, macular hemorrhage and precipitation of angioid streaks have frequently been noted after trauma. Angioid streaks have been described in a diverse group of diseases including pseudoxanthoma elasticum, Paget's disease, hemoglobinopathies such as sickle cell anemia and beta-thalassemia.
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PMID:[Angioid streaks. Pathogenesis and the clinical picture]. 883 55