A Case of Hereditary Angioedema with Laryngeal Edema Leading to Suffocation

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Treatment of 193 episodes of laryngeal edema with C1 inhibitor concentrate in patients with hereditary angioedema.

BACKGROUND Hereditary angioedema (HAE) is an autosomal dominant disease (Mendelian Inheritance in Man 106100) caused by an inherited deficiency of C1 inhibitor (C1-INH) function. The clinical symptoms include skin swelling, abdominal pain, and life-threatening episodes of upper airway obstruction. We evaluated the efficacy of C1-INH concentrate for treating sudden airway compromise. METHODS A...

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آنژیوادم ارثی: تشخیص نامناسب،‌ درمان ناکافی و گزارش 7 مورد از یک خانواده

Hereditary angioedema is a rare disorder of complement system which is often seen with autosomal dominant hereditary. Clinical characteristics include non- pruritic and non-pitting mucocutaneous edema that could involve all parts of the body. This study reports seven cases of hereditary angioedema with classical manifestations accompanied by low function of C1INH (type 2). One death occurred du...

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Management 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...

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A Case of Hereditary Angioedema Presenting with Ileocolic Intussusception

Case Presentation: A 38-year-old female with a past medical history of type 2 HAE presents with seven days of progressive right lower quadrant abdominal pain and distension, nausea, constipation, and decreased oral intake. Unlike her previous HAE episodes, her symptoms persisted despite two icatibant 30 mg subcutaneous injections. At presentation, she was afebrile, normotensive, and non-tachyca...

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Hereditary angioedema is a rare autosomal dominant disease characterized by the edema of subcutaneous tissues, respiratory tract and bowel. It is caused by the deficiency of C1 esterase inhibitor. Hereditary angioedema may be associated with autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis, autoimmune thyroiditis and glomerulonephritis. We report a 34-year-old mal...

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ژورنال

عنوان ژورنال: Nihon Naika Gakkai Zasshi

سال: 2013

ISSN: 0021-5384,1883-2083

DOI: 10.2169/naika.102.2685