Original ArticlesRetinal vascular occlusion and deficiencies in the protein C pathway
Section snippets
Patients and methods
In our study, 76 consecutive patients admitted to the Mainz University Hospital for evaluation of retinal vascular occlusive disease (central retinal vein occlusion in 35 patients, branch retinal vein occlusion in 21 patients, central retinal artery occlusion in 13 patients, and branch retinal artery occlusion in seven patients) were evaluated. The hospital serves as a referral center for approximately 4.4 million people. Within this area, referral practice is similar.
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Results
This series of 76 patients with retinal vascular disease consisted of 35 (46%) patients with central retinal vein occlusion, 21 (28%) with branch retinal vein occlusion, 13 (17%) with central retinal artery occlusion, and seven patients with branch retinal artery occlusion. All patients in the retinal vascular occlusion group and in the group with deep vein thrombosis were white. TABLE 1, TABLE 2 summarize the demographic data for the patient population with retinal vascular occlusion and the
Discussion
Much attention has been drawn to the protein C pathway since a new thrombophilic defect, resistance to activated protein C, was described by Dahlbäck.9, 34, 35 Until then, defects in other pathway components (for example, protein C and protein S) were detected rarely, even in patients with spontaneous venous thrombosis.8, 17, 36, 37 The Leiden Thrombophilia Study determined the prevalence of protein C and protein S deficiency in patients with an objectively confirmed diagnosis of thrombosis to
References (40)
- et al.
Color Doppler imaging of arterial blood flow in central retinal vein occlusion
Ophthalmology
(1994) - et al.
The natural course of central retinal vein occlusion
Ophthalmology
(1979) - et al.
Screening for resistence to activated protein C and the mutant gene for factor V506 in patients with central retinal vein occlusion
Am J Ophthalmol
(1997) - et al.
The role of abnormalities in the anticoagulant and fibrinolytic systems in retinal vascular occlusions
Surv Ophthalmol
(1993) - et al.
The inherited basis of venous thrombosis
Pathology
(1997) - et al.
Linkage between inherited resistance to activated protein C and factor V gene mutation in venous thrombosis
Lancet
(1994) - et al.
World distribution of factor V Leiden
Lancet
(1995) - et al.
Venous thrombosis due to poor anticoagulant response to activated protein CLeiden Thrombophilia Study
Lancet
(1993) - et al.
Peripheral retinal neovascularization and retinal vascular occlusion associated with activated protein C resistence
Am J Ophthalmol
(1997) - et al.
Retinal arterial occlusion in a child with factor V Leiden and thermolabile methylene tetrahydrofolate reductase mutations
Am J Ophthalmol
(1997)
Myocardial infarction associated with homozygous resistance to activated protein C
Lancet
Modified test for activated protein C resistance
Lancet
Activated protein C resistance as an additional risk factor for thrombosis in protein C–deficient families
Blood
Resistance to activated protein C as an additional genetic risk factor in hereditary deficiency of protein S
Blood
Central retinal vein occlusionsa prospective histopathologic study of 29 eyes in 28 cases
Retina
Familial thrombophiliagenetic risk factors and management
J Intern Med
Familial thrombophilia due to a previously unrecognized mechanism characterized by poor anticoagulant response to activated protein C
Proc Natl Acad Sci U S A
Resistance to activated protein C caused by the R506Q mutation in the gene for factor V is a common risk factor for venous thrombosis
J Intern Med
Venous thrombosisthe interaction of genes and environment
N Engl J Med
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