Disease relevance of Angioneurotic Edema

• BACKGROUND: Hereditary angioedema results from a congenital deficiency of functional C1 inhibitor and is characterized by episodic bouts of edema, which may be life-threatening when they involve the larynx [1].
• Cyclosporin relieves arthralgia, causes angioedema [2].
• Urticaria and angioedema induced by low-molecular-weight heparin [3].
• Bradykinin is a major mediator of swelling in C1 inhibitor deficiency as well as the angioedema seen with ACE inhibitors and may contribute to bronchial hyperreactivity in asthma [4].
• Acquired angioedema with lymphoproliferative disorder: association of C1 inhibitor deficiency with cellular abnormality [5].

Psychiatry related information on Angioneurotic Edema

• Polymerization of members of the serpin superfamily underlies diseases as diverse as cirrhosis, angioedema, thrombosis and dementia [6].

High impact information on Angioneurotic Edema

• C1 inhibitor and hereditary angioneurotic edema [7].
• Angioedema induced by the angiotensin II blocker losartan [8].
• Altered C1 inhibitor genes in type I hereditary angioedema [9].
• These results suggest that an interaction between the idiotype of monoclonal immunoglobulins and antiidiotypic antibodies causes increased consumption of C1q and C1 inhibitor in patients with acquired angioedema and C1-inhibitor deficiency [10].
• Patients with hereditary angioedema lack C-1 inhibitor, a plasma alpha 2-glycoprotein that inhibits both the proteolytic action of C1, the activated first component of the complement system, and the activity of components of the contact phase of coagulation: kallikrein, factor XIa, and factor XIIa [11].

Chemical compound and disease context of Angioneurotic Edema

• Intravenous administration of the concentrate during acute abdominal or laryngeal attacks of hereditary angioedema in five patients resulted in abatement of symptoms in addition to increased serum C4 activity [12].
• Androgen therapy in hereditary angioneurotic edema [13].
• Epsilon aminocaproic acid for hereditary angioedema [14].
• Angioneurotic edema from doxorubicin [15].
• Hereditary angioedema is an inherited disease transmitted as an autosomal dominant trait and characterized by deficient activity of C1 inhibitor, a glycoprotein that limits intravascular activation of complement [9].

Biological context of Angioneurotic Edema

• C1-esterase inhibitor (C1-Inh) therapy was introduced in clinical medicine about 25 years ago as a replacement therapy for patients with hereditary angioedema caused by a deficiency of C1-Inh [16].
• The molecular genetic basis of C1 inhibitor (C1 INH) deficiency in a patient with type I hereditary angioneurotic edema was studied [17].
• Restriction fragment length polymorphism of the C1 inhibitor gene in hereditary angioneurotic edema [18].
• The effects of the latter parameters have been studied quantitatively on a series of point mutations found in the human C1-inhibitor gene in patients affected by hereditary angioedema [19].
• Gross alterations in the C1 inhibitor gene account for about 20% of the hereditary angioedema chromosomes and consequently make this gene a prime example of the mutagenic liability of Alu repeats [20].

Anatomical context of Angioneurotic Edema

• Synthesis of C1 inhibitor in fibroblasts from patients with type I and type II hereditary angioneurotic edema [21].
• Complexes between C1-inhibitor, kallikrein, high molecular weight kininogen, plasma thromboplastin antecedent, and plasmin in normal human plasma and hereditary angioneurotic edema plasmas containing dysmorphic C1-inhibitors: role of cold activation [22].
• Histamine inhalation challenge in recurrent uvula angioedema [23].
• BACKGROUND: Hereditary angioedema (HAE) is a congenital disorder with recurrent attacks of localized swelling of submucosal tissue, subcutaneous tissue, or both caused by a deficiency of the plasma protein C1 inhibitor (C1 esterase inhibitor [C1INH]) [24].
• Therefore to investigate why reduction of C1-inhibitor to levels seen in angioedema patients results in excessive kallikrein generation we examined the effect of endothelial cells on the inhibition of kallikrein by C1-inhibitor [25].

Gene context of Angioneurotic Edema

• Heterozygosity for C1 inhibitor (C1INH) deficiency results in hereditary angioedema [26].
• Although Alu insertions have been implicated in other diseases, and a closely related AluY element is found as an insert in the C1 inhibitor gene in patients with hereditary angioedema, this is the first case of glycerol kinase deficiency caused by an Alu insertion [27].
• METHODS: Patients with urticaria or angioedema triggered by NSAIDs were challenged with COX-2 inhibitors by the single-blinded, placebo-controlled oral method [28].
• BACKGROUND: The safety of new anti-inflammatory drugs in patients intolerant to classic cyclooxygenase (COX) inhibitors with urticaria and angioedema has not been determined [28].
• It is similar to vibratory angioedema (VA), but sufficient evidence to prove that DDU and VA are identical is not available [29].

Analytical, diagnostic and therapeutic context of Angioneurotic Edema

• Danazol, an androgen derivative, was evaluated for its effectiveness in preventing attacks of hereditary angioedema in a double-blind study with nine patients [30].
• Plasmapheresis in hereditary angioneurotic edema and systemic lupus erythematosus [31].
• Intravenous heparin did not prevent exacerbations of hereditary angioedema in a patient on maintenance hemodialysis [32].
• In the prophylaxis group self-administration of C1-inhibitor concentrate decreased the angioedema attack rate from 4.0 to 0.3 attacks per month [33].
• After treatment, the patient's C4 levels returned to normal and his angioedema resolved, but concurrently he experienced clinical and laboratory exacerbations of his lupuslike illness [34].

References