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Publication details
Hereditární angioedém v těhotenství
Title in English | Hereditary angioedema in pregnancy |
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Authors | |
Year of publication | 2006 |
Type | Article in Periodical |
Magazine / Source | Praktická gynekologie |
MU Faculty or unit | |
Citation | |
Field | Gynaecology and obstetrics |
Keywords | edema; complement; vasoactive substances; C1 inhibitor; hereditary angioedema; gravidity |
Description | The C1 inhibitor (C1 INH) deficiency is a rare, autosomal dominant imunologic disorder, which causes clinical disease named hereditary angioedema. This diease is characterised by a quick developement of painless and nonpruritic oedemas of subcutaneous and submucosal tissues. If gut mucosa is involved, the clinics resemble an acute abdomen. The laryngeal oedema may be lethal. Oedemas are caused by lack of C1 INH, complement cascade is not regulated properly and different vasoactive peptides are released. C1 inhibitor function is reduced due to impaired transcription type I) or production of non functional protein (type II). Attacks are precipitated by physical insults, stress or infection. Most of the drugs used as prophylactic treatment are contraindicated in pregnancy. Moreover, labor is a kind of physical and psychological stress. Therefore pregnant women with hereditary angioedema should be carefully observed and treated in cooperation with immunologist. |