Prophylaxis in hereditary angioedema (HAE) with C1 inhibitor deficiency
نویسندگان
چکیده
منابع مشابه
Tryptase and histamine in patients with angioedema due to C1-inhibitor deficiency (Hereditary Angioedema, HAE) and in patients with mastocytosis
Material and methods. The determinations were performed in 14 mastocytotic patients, including 6 patients with the systemic mastocytosis and 8 with urticaria pigmentosa, in 14 with innate angio-motor oedema due to C1 inhibitor deficiency, and in 10 healthy controls. Tryptase levels were determined using PhadiaUniCAP TRYPTASE reagents. Histamine concentrations were measured using HISTAMINE ELISA...
متن کاملPediatric 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...
متن کاملHereditary angioedema without deficiency of C1 inhibitor: response to therapy
Results Nineteen patients have been identified (2M:17F; 20-60 years old). The following therapies were oriented: combined contraceptive substitution for progestagen (10/19); treatment with progestagen (2/19); tranexamic acid (15/19): 1250mg (2), 1000mg (1), 750 (5), 500 mg (4), 250 mg (1); oxandrolon (5/19) (0.5 mg-5mg/day), danazol 200mg/day (1/19) and combined therapy woth oxandrolon and tran...
متن کامل[Hereditary angioedema due to C1-esterase inhibitor deficiency].
The paper presents a case of family oedema by shortage of C1-esterase inhibitor, characterized by appearance at an early age (8 months), participation in some attacks of nasal mucous membrane, with nasal obstruction and sinusal manifestations and also the abnormally long period, up to 9 days, of some localized oedemas. The crises were absent during pregnancy. The study of four generations of th...
متن کاملAcquired 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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ژورنال
عنوان ژورنال: JDDG: Journal der Deutschen Dermatologischen Gesellschaft
سال: 2016
ISSN: 1610-0379
DOI: 10.1111/ddg.12856