Angioedema Due To Acquired C1-Inhibitor Deficiency: Spectrum And Treatment With C1-Inhibitor Concentrate

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Acquired angioedema associated with hereditary angioedema due to C1 inhibitor deficiency.

Angioedema caused by C1 inhibitor deficiency is a rare disorder that may be either hereditary or acquired, the latter being mainly associated with lymphoproliferative disorders. A 51-year-old woman who had suffered from episodes of acute peripheral edema since she was 12 was diagnosed with hereditary angioedema at the age of 40 and remained stable with stanozolol. Due to a worsening of her symp...

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Pediatric hereditary angioedema due to C1-inhibitor deficiency

Hereditary angioedema (HAE) resulting from the deficiency of the C1 inhibitor (C1-INH) is a rare, life-threatening disorder. It is characterized by attacks of angioedema involving the skin and/or the mucosa of the upper airways, as well as the intestinal mucosa. In approximately 50 per cent of cases, clinical manifestations may appear during childhood. The complex management of HAE in pediatric...

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C1-inhibitor concentrate for treatment of hereditary angioedema.

BACKGROUND Hereditary angioedema due to C1 inhibitor deficiency is characterized by recurrent acute attacks of swelling that can be painful and sometimes life-threatening. METHODS We conducted two randomized trials to evaluate nanofiltered C1 inhibitor concentrate in the management of hereditary angioedema. The first study compared nanofiltered C1 inhibitor concentrate with placebo for treatm...

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163 The Efficacy of Human Plasma-Derived C1-Inhibitor Concentrate Used for Prophylactic Treatment in Patients With Hereditary Angioedema Due to C1-Inhibitor Deficiency

vidual rhinoconjunctivitis symptom scores. The primary efficacy endpoint, the daily CS during the pollen period while on treatment, was analyzed using a repeated measures ANCOVA model, as were the above secondary efficacy endpoints. The safety of the treatment was documented by means of adverse event reporting, laboratory data and physical examination findings. Results: The 300IR group showed a...

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ژورنال

عنوان ژورنال: Journal of Allergy and Clinical Immunology

سال: 2019

ISSN: 0091-6749

DOI: 10.1016/j.jaci.2018.12.128