title: [Perioperative treatment of a patient with hereditary angioedema (HAE) in a case of a young patient with osteosynthesis from a femoral fracture].
abstract: The following case report describes hereditary angioedema (HAE) in a juvenile male patient presenting with femoral fracture. The clinical characteristics, pathophysiological changes, diagnostics and management of anaesthesia for patients with hereditary angioedema will be discussed. Hereditary angioedema (HAE) is a rare autosomal dominant disorder, which is caused by congenital deficiency of functional C1-inhibitor (C1-INH). Patients are suffering from episodic and painless edema of the skin (face and limbs) and mucous membranes of the respiratory and gastrointestinal tracts (the latter causing abdominal cramps due to edema of the intestine). Life-threatening airway obstruction may occur when patients develop laryngeal edema. It is important to differentiate HAE from the more frequent allergic angioedema because of differences in the pharmacological treatment of acute attacks of HAE. C1-INH-concentrate is effective in both treatment of acute attacks and prevention of edema, especially in children, juveniles and young women.
authors.affiliation: Klinik für Anästhesiologie, Universitäts-Krankenhaus Eppendorf, Hamburg.
authors.forename: S
authors.initials: S
authors.lastname: Eckert
authors.forename: B
authors.initials: B
authors.lastname: Eifrig
authors.forename: T
authors.initials: T
authors.lastname: Standl
mesh_terms: D000293:Adolescent; D000799:Angioedema / Q000150:complications* / Q000175:diagnosis / Q000235:genetics*; D019411:Clinical Laboratory Techniques; D005264:Femoral Fractures / Q000150:complications* / Q000628:therapy; D006801:Humans; D008297:Male; D010375:Pedigree
vernacular_title: Die perioperative Behandlung von Patienten mit hereditärem Angioödem (HAE) am Beispiel eines Jugendlichen mit Osteosynthese einer Oberschenkelfraktur.