Angioid streaks associated with abetalipoproteinemia
نویسندگان
چکیده
منابع مشابه
Visual loss associated with angioid streaks in sickle thalassemia.
Angioid streaks (AS) represent ruptures of the elastic lamina of Bruch’s membrane and are typically seen in the fundus of patients with pseudoxanthoma elasticum (PXE), a rare inherited elastic tissue defect. The development of AS in hemoglobinopathies is not a novel observation, as it has been reported in sickle-cell disease since the late 1950s. Despite however the frequent presence of AS in t...
متن کاملAngioid streaks and elastorrhexis.
ANGIOID streaks are thought to be due to breaks in the elastic lamina of Bruch's membrane. Ophthalmoscopically they present a jagged peripapillary ring which may be incomplete and from which radiate irregular spokes of width similar to retinal vessels. With time, they may progress, or become less distinct. Their colour varies from reddish-brown to dark brown according to the density of the over...
متن کاملAngioid Streaks in Psuedoxanthoma Elasticum
Pseudoxanthoma elasticum (PXE) is a hereditary disorder that affects primarily the elastic tissues of skin, eyes and blood vessels. Its estimated prevalence is thought to be 1:25,000 100,000. PXE patients also carry an increased risk for cardiovascular and gastrointestinal diseases. Early diagnosis can play a crucial role in preventing early loss of vision and systemic complications in these pa...
متن کاملClassification of angioid streaks.
ANGIOID streaks of the retina were first described by Doyne (1889). They have since been the subject of much controversy. More than 200 cases have been reported in the literature, but histological studies have been few and the changes described have varied. The condition is frequently associated with pseudoxanthoma elasticum, with cardiovascular disease, and with Paget's disease of bone. A simi...
متن کاملABCC6 Mutation in Patients with Angioid Streaks
Angioid streaks (AS) are hereditary eye conditions caused by breaks in the elastic layer of Bruch's membrane. Patients with AS are also frequently affected with pseudoxanthoma elasticum (PXE). The locus of PXE has been reported to exist in chromosome 16p13.1, and the ABCC6 gene in this locus has been identified as the causal gene of PXE. In this study we investigated the association of the ABCC...
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ژورنال
عنوان ژورنال: Ophthalmic Genetics
سال: 1994
ISSN: 1381-6810,1744-5094
DOI: 10.3109/13816819409057843