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Otorhinolaryngology
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  Authority of Polish Society of Allergology




vol 14. no 2. June 2009  
 TABLE OF CONTENT

 Original articles
Hereditary angioedema (HAE) in children (Warsaw register)
Ewa Nowicka, Ewa Najberg, Hanna Gregorek

Introduction. Hereditary angioedema (HAE) is an autosomal dominant
disease characterized by recurrent episodes of potentially
life-threatening angioedema. The disease is caused by mutations in
the C1 inh gene, leading to dysfunction of the C1-esterase inhibitor
(C1-Inh).
Aim of the study. The study objective was the analysis of clinical
symptoms and immunological data in children with HAE recognized
during childhood.
Material and methods. In the Department of Allergology, Children’s
Memorial Health Institute, 15 children (9 girls and 6 boys) aged 7-18
years (median 14.2) were registered. The diagnosis of HAE was established
by personal questionnaire, family history, clinical examination
and complement measurements (serum concentrations of C4 and
C1-Inh, and C1-Inh function).
Results. The most frequent clinical manifestations of HAE were recurrent
subcutaneous edemas of extremities, face, neck and edemas of
gastrointestinal tract mucosa (abdominal pain attacks). Serious laryngeal
episodes were observed in one girl and transient edemas of the
brain in 3 patients, while one child remained asymptomatic during
the time of observation. Based on the combination of total protein
and functional C1-inh levels, 13 children were classified as type I and
2 children as type II HAE. Nobody received chronic long-term prophylaxis.
2 children have been treated periodically with EACA. High
doses of tranexamic acid or C1-inh concentrate were used in acute
serious episodes.
Conclusions. There are indications for complement diagnostic tests
in all members of families with history of hereditary angioedema.
Early diagnosis is very important for the course and prognosis of this
disorder.

keywords: dzieci, dziedziczny obrzęk naczynioruchowy, leczenie, children, HAE, treatment, clinical symptoms

pages: from 106 to 112



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