Home
Aktualny numer
Archiwum
Redakcja
Informacje dla autorów
Recenzenzowanie prac
Indeksowanie czasopisma
Dla sponsorów
Kontakt
Tom 23. nr 4
Grudzień 2018
Hereditary angioedema: treatment options and availability. Balance between patients’ needs and stakeholders’ plans
(streszczenie)
Anna Valerieva, Francesca Perego, Grzegorz Porebski, Maria Staevska, Marco Cicardi
Hereditary angioedema is a rare and disabling disease characterized by
severe, acute, self- limiting edema of the subcutaneous and mucosal
tissue. The disease carries significant mortality when the upper airways
are involved. It is determined by a transient dysregulation in vascular
permeability with a sudden increase in fluid extravasation. The most
common genetic defects recognized on the SERPING1 gene leads to
complement C1-inhibitor deficiency (C1-INH-HAE). In the last few years
mutation on the factor XII gene, the angiopoietin, and the plasminogen
genes were recognized to cause angioedema with normal C1-INH
levels (nC1-INH-HAE). However, it is not uncommon to have family history
of angioedema and unknown genetic defect. The burden of the
disease on the individual patients is unpredictable since the number,
location and severity of the attacks spans from silent disease to an
extremely high number of attacks during lifetime. Nowadays effective
and safe treatment for the C1-INH-HAE forms are available and new
drugs are under investigation both for the on demand therapy and for
the prophylaxis of the attacks. Acute treatments has been proved to reduce
severity and duration of attack, prophylaxis reduces the number
of attacks thus positively affecting the quality of life and reducing the
indirect costs of the disease. Unfortunately, the costs of the medications
are high limiting the accessibility and availability of treatment in
different countries. This paper reviews the treatment options and the
differences in the availability of the medications in three European countries,
with practical suggestions for the management of the disease.
Keywords: hereditary angioedema, treatment, prophylaxis, C1 inhibitor,
bradykinin receptor antagonist, kallikrein inhibitors
Prawa autorskie: Wszelkie prawa autorskie (w tym prawo do wydawania drukiem, na nośnikach elektronicznych oraz w Internecie)
są zarezerwowane dla Oficyny Wydawniczej Mediton. Artykuły ani ich fragmenty nie mogą być kopiowane i rozpowszechniane
w żadnej formie (tradycyjnej czy elektronicznej) bez uzyskania pisemnej zgody Wydawcy.