منابع مشابه
INVESTIGATIVE OPHTHALMOLOGY Electron microscopic study of hereditary corneal edema
Dtiring the first year of life, diffuse corneal clouding is usually caused by infantile glaucoma or infections and inflammatory processes. Although several of the mucopolysaccharidases may also cause corneal clouding, other signs and symptoms predominate. Maumenee* described a congenital, hereditary corneal dystrophy characterized by diffuse corneal edema during the first year of life. His repo...
متن کاملManagement of upper airway edema caused by hereditary angioedema
Hereditary angioedema is a rare disorder with a genetic background involving mutations in the genes encoding C1-INH and of factor XII. Its etiology is unknown in a proportion of cases. Recurrent edema formation may involve the subcutis and the submucosa - the latter can produce obstruction in the upper airways and thereby lead to life-threatening asphyxia. This is the reason for the high, 30-to...
متن کاملFurther electron microscopic study of hereditary corneal edema.
This report presents further observation on the fine structure of hereditary corneal edema. The anterior and middle stroma showed almost normal collagen lamellar formation. In the posterior stroma irregularity of interfibrillary distance icas noted. Cross-sections of collagen fibrils varied in diameter. Tioo Descemet's membranes were found, the second one contained unusual banded materials. Mos...
متن کاملMechanisms of Activation of C'1 Esterase in Hereditary Angioneurotic Edema Plasma in Vitro
The generation of C'1 esterase activity in siliconed plasma obtained from individuals with hereditary angioneurotic edema in remission tends to occur spontaneously, but can be hastened during its incubation with preparations of activated Hageman factor. This effect of activated Hageman factor could not be shown during its incubation with normal siliconed plasma, nor could consumption of normal ...
متن کاملGenetically determined heterogeneity of the C1 esterase inhibitor in patients with hereditary angioneurotic edema.
Normal human serum contains 18 +/-5 mg/100 ml of C1 esterase inhibitor (alpha-2 neuraminoglycoprotein) as estimated by immunochemical means. Of 118 patients with hereditary angioneurotic edema, the sera of 80, from 42 kindred, contained a mean concentration of 3.15 mg/100 ml or 17.5% of normal. The mean serum concentration in 35 patients in 7 other kindred was 20 mg/100 ml or 111% of normal, an...
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ژورنال
عنوان ژورنال: The Journal of Nervous and Mental Disease
سال: 1922
ISSN: 0022-3018
DOI: 10.1097/00005053-192211000-00019