Cut-off value of C1-inhibitor function for the diagnosis of hereditary angioedema due to C1-inhibitor deficiency
نویسندگان
چکیده
منابع مشابه
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...
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 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 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...
متن کاملUS Hereditary Angioedema Association Medical Advisory Board 2013 recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency.
BACKGROUND The treatment of hereditary angioedema (HAE) has undergone dramatic changes as newer medicines have become available in recent years. Optimal care of these patients requires a comprehensive management plan. Although several consensus papers have been published concerning the diagnosis and treatment of HAE, guidelines for a comprehensive management plan have not been developed. OBJE...
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ژورنال
عنوان ژورنال: Intractable & Rare Diseases Research
سال: 2021
ISSN: 2186-361X,2186-3644
DOI: 10.5582/irdr.2020.03099