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First Author: M.Davari IRAN
Co Author(s): T. Kazemi H. Gheitasi
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To investigate the natural history of ocular and cardio logical abnormalities in one family with Marfan syndrome during10years follow up.
Birjand Atherosclerosis and Coronary Artery Research Centre ,Ophthalmology Department, Birjand University of Medical Sciences, Birjand, Iran.
11 patients with MFS, from the same family in Birjand, south east of IRAN were followed for 10 years. Occurrence of ocular manifestations and adverse cardiovascular Outcomes was measured clinically and by ultrasound examination.
After 5 years follow up seven had mar fan syndrome and the rest are normal. Father and three of his sons and two of his daughters and First grandchild are involved. Mother and a son are normal. Ocular manifestation were lens ectopia 100% ( 7/7 ) , Flat cornea 28El% (2/7), on gated eyeball 42% (3/7) , Hypo plastic iris or ciliary"s muscle hypoplasia 42% (3/7).Glaucoma (2/7). Cataract (2/7). Retinal detachment (2/7) After 10 years follow up 11 members have mar fan syndrome .Father and three of his sons and two of his daughters and 5 grandchildren are involved. Ocular manifestation were lens ectopia 100% ( 11/11 ) , Flat cornea 28El% (4/11), on gated eyeball 42% (5/11) , Hypo plastic iris or ciliary"s muscle hypoplasia 42% (5/11).Glaucoma (3/11). Cataract (3/11). Retinal detachment (2/11). Three patients needed ocular surgery.
During 10 years follow up in this family with Marfan syndrome, ocular manifestations as well as adverse cardiovascular abnormalities develop and progress gradually, but may cause considerable morbidity and mortality by the end of the second decade. We think MFS with this severe genetic penetration is rare.