Angioedema in pediatric heart transplant recipients—reporting C1-esterase inhibitor deficiency without analysing protein

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angioedema in a patient with c1 esterase inhibitor deficiency

angioedema is characterized by recurrent, circumscribed, solitary or multiple subcutaneous and mucosal swelling, involving the extremities, face, larynx, bowel wall. angioedema is due to hereditary or acquired varieties of c1 esterase inhibitor (c1inh) deficiency. a case of atypical acquired angioedema in a 49-year old man, responding favourably to cinnarizine and alcohol abstinence, is present...

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[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...

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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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Hereditary 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...

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Self-administration of intravenous C1 esterase inhibitor in hereditary angioedema.

somal dominant disorder that results in episodes of acute edema in various organs, including the gastrointestinal tract, skin and larynx. It is estimated to affect about 1 in 50 000–100 000 people. The symptoms, including abdominal pain, laryngeal edema and subcutaneous edema, usually begin in childhood and persist throughout life with unpredictable severity. Hereditary angioedema is classified...

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ژورنال

عنوان ژورنال: Pediatric Radiology

سال: 2014

ISSN: 0301-0449,1432-1998

DOI: 10.1007/s00247-014-2965-5