angioneurotic edema
Studies on human plasma C1 inactivator enzyme interactions II Structural features of an abnormal C1 inactivator from a kindred with hereditary angioneurotic edema
... The function and several of the structural features of the C1 inactivator protein isolated from the plasma of a mother and daughter with the variant form of hereditary angioneurotic edema have been ...
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Nonsense mutations affect C1 inhibitor messenger RNA levels in patients with type I hereditary angioneurotic edema
... Members of two unrelated families with type I hereditary angioneurotic edema (HANE) were found to have elevated levels of C1 inhibitor (C1INH) mRNA. DNA sequence analysis of PCR-amplified monocyte C1INH ...
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Transinhibition of C1 inhibitor synthesis in type I hereditary angioneurotic edema
... hereditary angioneurotic edema, we studied expression of C1 INH in fibroblasts in which the mutant and wild type mRNA and protein could be distinguished because of deletion of exon 7 (delta ...
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HEREDITY OF ANGIONEUROTIC EDEMA BASED ON A REVIEW OF THE LITERATURE
... Taking up the matings of affected x unaffected first (table l ) , the figures show con- clusively that familial angioneurotic edema may be transmitted by either parent [r] ...
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Restriction fragment length polymorphism of the C1 inhibitor gene in hereditary angioneurotic edema
... Hereditary angioneurotic edema (HANE) results from the deficiency of the inhibitor of the first component of human complement (C1-INH). It is inherited as an autosomal dominant trait. Heterogeneity of this ...
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Effect of C′1 esterase on vascular permeability in man: studies in normal and complement deficient individuals and in patients with hereditary angioneurotic edema
... hereditary angioneurotic edema. Patients with hereditary angioneurotic edema are unresponsive to intradermal injections of C¢1 esterase immediately after ...
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Variability in purified dysfunctional C1( ) inhibitor proteins from patients with hereditary angioneurotic edema Functional and analytical gel studies
... hereditary angioneurotic edema (HANE) were compared with respect to their inhibitory activity against purified preparations of C1s-, plasma kallikrein, activated forms of Hageman factor, and ...
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Plasma levels of C1 inhibitor complexes and cleaved C1 inhibitor in patients with hereditary angioneurotic edema
... hereditary angioneurotic edema (HANE) was assessed by measuring the complexes formed by C1(-)-Inh with its target proteases (C1-s, Factor XIIa, and kallikrein) and a modified (cleaved) inactive form of ...
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Behavior in vivo of normal and dysfunctional C1 inhibitor in normal subjects and patients with hereditary angioneurotic edema
... The metabolism of normal C1 inhibitor and two dysfunctional C1 inhibitors (Ta and WeI) was studied in 10 normal subjects and 8 patients with hereditary angioneurotic edema (HANE), 4 with low antigen ...
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Permeability increasing activity in hereditary angioneurotic edema plasma: II Mechanism of formation and partial characterization
... Plasma from persons with hereditary angioneurotic edema readily developed the capacity to increase vascular permeability and to induce the isolated rat uterus to contract. Both activities resided in a ...
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Synthesis of C1 inhibitor in fibroblasts from patients with type I and type II hereditary angioneurotic edema
... hereditary angioneurotic edema (HANE) have serum levels of functionally active inhibitor of the first component of complement (C1 INH) between 5 and 30% of normal, instead of the 50% expected from the ...
A Mutation Unique in Serine Protease Inhibitors (Serpins) Identified in a Family with Type II Hereditary Angioneurotic Edema
... (1990) Type II hered- itary angioneurotic edema that may result from a single nucleotide change in the codon for alanine-436 in the Cl inhibitor gene. Parad RB, Kramer J, Strunk RC, Rose[r] ...
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HEREDITARY ANGIONEUROTIC EDEMA: A CLINICAL SURVEY
... esterase which is a specffic alpha-2 globu- lin.8 Sera from over 500 healthy and dis- eased individuals had the capacity to in- hibit C’l-esterase.7 Individuals subject to attacks of her[r] ...
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Molecular basis for the deficiency of complement 1 inhibitor in type I hereditary angioneurotic edema
... The study of C1 INH messenger RNA mRNA by Northern blot analysis indicated that in HANE patients' monocytes a message of normal size is present at about half the concentration of that fr[r] ...
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Action of Complement in Hereditary Angioneurotic Edema: The Role of C′1 Esterase
... The increased amounts of C'1-esterase activity in serum and plasma of patients having attacks of swelling or pain suggested that C'1-esterase might have caused increased vascular permeab[r] ...
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Genetically determined heterogeneity of the C1 esterase inhibitor in patients with hereditary angioneurotic edema
... The nonfunctional inhibitors in patients' sera of these eight kindred were identical with normal C1 esterase inhibitor by Ouchterlony analysis, but they were different from normal and fr[r] ...
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OBSCURE AND UNUSUAL EDEMA
... minemia, severe nutritional deficiency with hypoproteinemia, protein-losing gastrc en- teropathies and hereditary angioneurotic edema have been discussed in the context of unusual and ob[r] ...
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Pulmonary Edema Associated With Croup and Epiglottitis
... upper airway obstruction, and recent comprehen- sive reviews4 of pulmonary edema fail to mention acute upper airway obstruction as a possible cause.. CASE REPORTS.[r] ...
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Hypoglycemia and Unilateral Pulmonary Edema in a Newborn
... Ipsilateral pulmonary edema has been reported in systemic-pulmonary artery shunts, unilateral veno-occlusive disease, bronchial obstruction, prolonged lateral decubitus position, unilate[r] ...
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The Clinical Significance of Placental Villous Edema
... villous edema scores on the two sides of fused dichorionic and monochorionic twin placentas when only one amniotic sac of each placenta had acute chorioamnionitis.. If the factor respons[r] ...
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