RESUMO
We examined members of a family in whom hereditary spherocytosis had appeared in three generations. Angioid streaks were confirmed in the second generation and presumed to exist in the first generation. A woman in the third generation with hereditary spherocytosis did not have angioid streaks but these are age-related and may develop later. The one individual in the second generation without hereditary spherocytosis did not have angioid streaks. Angioid streaks associated with hereditary spherocytosis in this family did not appear to be coincidental. Patients with hereditary spherocytosis should be examined for angioid streaks because of the implications for their vision.
Assuntos
Corioide/patologia , Esferocitose Hereditária/patologia , Adulto , Fatores Etários , Idoso , Calcinose/patologia , Oftalmopatias/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , LinhagemAssuntos
Angiografia , Fundo de Olho/irrigação sanguínea , Oftalmoscopia , Fluoresceínas , Humanos , Midriáticos , Soluções Oftálmicas , Fenilefrina , FotografaçãoRESUMO
Elevated Factor VIII-von Willebrand factor antigen levels are associated with vascular endothelial injury in several disorders, including scleroderma, Raynaud's phenomenon, polymyalgia rheumatica, and temporal arteritis. Eight patients with serpiginous choroidopathy were evaluated with the use of quantitative immunoelectrophoresis for Factor VIII-von Willebrand factor antigen. The serpiginous choroidopathy patients had a mean Factor VIII-von Willebrand factor activity of 226 +/- 47.3%, whereas a disease-free, age- and sex-matched control group had a mean activity of 107 +/- 28%. These findings are statistically significant (P greater than 0.005) and suggest that in some patients, serpiginous choroidopathy represents an occlusive vascular phenomenon that involves the choroidal circulation.
Assuntos
Doenças da Coroide/sangue , Doenças Retinianas/sangue , Fator de von Willebrand/metabolismo , Adulto , Idoso , Feminino , Angiofluoresceinografia , Fundo de Olho , Humanos , Imunoeletroforese , Isquemia/sangue , Masculino , Pessoa de Meia-Idade , Vasos Retinianos/metabolismo , Acuidade VisualRESUMO
Patients with systemic reticulum cell sarcoma often develop evidence of ocular involvement months or several years prior to the onset of neurologic symptoms. This neoplasm appears to have proclivity of arising multicentrically within the subpigment epithelial space, as well as in the vitreous. When this occurs it may produce a peculiar ophthalmoscopic picture of multiple large, solid detachments of the pigment epithelium that is rarely duplicated by any other disease. Three patients illustrating this disease are presented.