Management strategies of ocular abnormalities in patients with marfan syndrome: Current perspective
نویسندگان
چکیده
منابع مشابه
Musculo-Skeletal Abnormalities in Patients with Marfan Syndrome
BACKGROUND A leptosomic body type is tall and thin with long hands. Marfanoid features may be familial in nature or pathological, as occurs in congenital contractual arachnodactyly (Beal's syndrome) and Shprintzen-Goldberg syndrome mimicking some of the changes of Marfan syndrome, although not accompanied by luxation of lens and dissecting aneurysm of aorta. METHODS In this article we collect...
متن کاملMarfan syndrome: fibrillin expression and microfibrillar abnormalities in a family with predominant ocular defects.
We have found abnormal fibrillin microfibrils isolated from tissues and cell cultures from two cousins with Marfan syndrome whose major clinical abnormality is bilateral ectopia lentis, but who also have skeletal involvement but no cardiovascular defects. Ultrastructural analysis of ciliary zonules showed the presence of abundant loose microfibril bundles which in many places appeared disorgani...
متن کاملMarfan syndrome: current perspectives
Marfan syndrome (MFS) is a pleiotropic connective tissue disease inherited as an autosomal dominant trait, due to mutations in the FBN1 gene encoding fibrillin 1. It is an important protein of the extracellular matrix that contributes to the final structure of a microfibril. Few cases displaying an autosomal recessive transmission are reported in the world. The FBN1 gene, which is made of 66 ex...
متن کاملPERSPECTIVE Management of ocular ischaemic syndrome
Symptoms of carotid artery disease frequently present to ophthalmologists. Though these may be sight threatening, they may represent the first signs of life threatening carotid artery stenosis. These include cerebral transient ischaemic attacks (TIA), transient monocular blindness (amaurosis fugax), central or branch retinal artery occlusion, hypotensive retinopathy (previously known as “venous...
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ژورنال
عنوان ژورنال: Journal of Ophthalmic and Vision Research
سال: 2019
ISSN: 2008-322X
DOI: 10.4103/jovr.jovr_29_18