MMW - Fortschritte der Medizin

, Volume 155, Supplement 2, pp 1–5

Fortschritte im Management des hereditären Angioödems

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Zusammenfassung

Das hereditäre Angioödem (HAE) ist eine seltene genetische Krankheit, die durch einen quantitativen oder funktionellen Mangel an C1-Esterase-Inhibitor (C1-INH) verursacht wird, was eine übermäßig starke Zunahme der Bradykininproduktion zur Folge hat. Dies kann zu akuten, episodenartig auftretenden, umschriebenen Schwellungen von Haut und Schleimhäuten des Mundes, Kopfes und Halses, der Extremitäten, des Gastrointestinaltrakts, der Genitalien, des Rumpfs und Larynx führen. Im Respirationstrakt kann ein Angioödem zu einer Obstruktion der Atemwege führen. Bei einem Angioödem im Gastrointestinaltrakt kann es zu starken Bauchschmerzen, Erbrechen und Durchfall kommen. Ein HAE kann, abhängig von den jeweiligen Umständen, von HAE-Spezialisten oder Primärärzten diagnostiziert und behandelt werden. Für eine richtige Behandlung ist die Differenzierung von anderen Angioödemarten erforderlich. Bei Patienten mit HAE, die die richtigen Medikamente zur Behandlung und Vorbeugung akuter Episoden erhalten, kann das Risiko, an einem Larynxödem zu sterben, wahrscheinlich reduziert und die Lebensqualität gebessert werden. Wenn die Episoden seltener auftreten und weniger schwer sind, sind die mit HAE einhergehenden Schmerzen und Verunstaltungen geringer. Dem Patienten wird ermöglicht, sich regelmäßiger Arbeit, Schule und Freizeitbeschäftigungen zu widmen.

Schlüsselwörter

Hereditäres Angioödem Primärarzt C1-Esterase-Inhibitor 

Progress with management of hereditary angioedema

Abstract

Hereditary angioedema (HAE) is a rare type of angioedema caused by a quantitative or functional deficit of C1 inhibitor (C1 INH) that leads to excess production of bradykinin, which can result in acute localized swelling attacks in the skin or mucous membranes of the mouth, head and neck, extremities, gastrointestinal (GI) tract, genitals, trunk, and larynx. Angioedema in the respiratory tract may cause airway obstruction; severe abdominal pain, vomiting, or diarrhea may occur in the GI tract. Patients with HAE may be diagnosed and managed by HAE specialists or by primary care physicians depending on individual circumstances. Proper treatment requires differentiation from other forms of angioedema. Patients with HAE who are managed appropriately with medications that treat and prevent atttacks may have a lower risk of death from laryngeal edema and a better quality of life. Less frequent attacks may allow them to attend work, school, and leisure activities more regularly and be free of the pain and disfigurement of HAE attacks more often.

Keywords

Hereditary angioedema Primary care physicians C1-esterase-inhibitor 

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Copyright information

© Urban & Vogel 2013

Authors and Affiliations

  1. 1.Edward Via College of Osteopathic Medicine, Carolina CampusAsthma & Allergy SpecialistsCharlotteUSA
  2. 2.Institut für Klinische Pharmakologie und Toxikologie, Charité – Universitätsmedizin, Freie Universität BerlinBerlinDeutschland

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