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- W2114941279 abstract "HEREDITARY ANGIOEDEMA is a rare autosomal dominant disorder based on decreased functional activity of the C1 esterase inhibitor (C1-INH). C1-INH is the main regulatory protein of the initial steps of the complement, contact activation, and kinin pathways. 1 Leimgruber A Jaques WA Spaeth PJ Hereditary angioedema: Uncomplicated maxillofacial surgery using short-term C1 inhibitor replacement therapy. Int Arch Allergy Immunol. 1993; 101: 107-112 Crossref PubMed Scopus (41) Google Scholar Decreased C1-INH causes an episodic and uncontrolled release of vasoactive mediators and increased vascular permeability. Clinical symptoms of an attack of hereditary angioedema are painless edema of the skin (face, limbs, and genitals) and mucous membranes (respiratory and gastrointestinal tract). Laryngeal edema is a life-threatening manifestation leading to airway obstruction and death. Mortality rates are ≤40%. 2 Bork K Siedlecki K Bosch S et al. Asphyxiation by laryngeal edema in patients with hereditary angioedema. Mayo Clin Proc. 2000; 75: 349-354 Abstract Full Text Full Text PDF PubMed Scopus (222) Google Scholar Only a few case reports about patients with hereditary angioedema undergoing cardiac surgery exist. 3 Umebayashi Y Morishita Y Arikawa K et al. Hereditary angioneurotic edema: Report of a case undergoing open heart surgery: A case report. Vasc Surg. 1987; 21: 138-141 Crossref Scopus (3) Google Scholar , 4 Bonser RS Dave J Morgan J et al. Complement activation during bypass in acquired C1 esterase inhibitor deficiency. Ann Thorac Surg. 1991; 52: 541-543 Abstract Full Text PDF PubMed Scopus (26) Google Scholar , 5 Haering JM Comunale ME Cardiopulmonary bypass in hereditary angioedema. Anesthesiology. 1993; 79: 1429-1433 Crossref PubMed Scopus (13) Google Scholar , 6 Bainbridge DT Mackensen GB Newman MF et al. Off-pump coronary artery bypass surgery in a patient with C1 esterase inhibitor deficiency. Anesthesiology. 2001; 95: 795-796 Crossref PubMed Scopus (7) Google Scholar" @default.
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- W2114941279 date "2002-08-01" @default.
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- W2114941279 title "Successful off-pump coronary artery bypass graft surgery in a patient with hereditary angioedema" @default.
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- W2114941279 doi "https://doi.org/10.1053/jcan.2002.125132" @default.
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