Talk:Hereditary angioedema

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Article categorization

This article was initially categorized based on scheme outlined at WP:DERM:CAT. kilbad (talk) 20:18, 20 February 2009 (UTC)[reply]

Pathogenesis

The pathogenesis section contains nothing on pathogenesis, it only discusses the genetic inheritance. Would be good if someone knowledgeable on this topic could separate the two sections and write something on the pathogenesis. Furthermore, do we really need 3 paragraphs to describe how autosomal dominant inheritance works? Just link the relevant wiki page and move on.

other language

I don't know much about the syntax in wikis, so I'll post it rght here: there is a german version of this article: http://de.wikipedia.org/wiki/Hereditäres_Angioödem —Preceding unsigned comment added by 84.167.64.174 (talk) 21:27, 26 May 2009 (UTC)[reply]

Thank you, I'll an and interwiki. --CopperKettle 23:39, 17 November 2009 (UTC)[reply]

Moved from angioedema

;Long-term prophylaxis

Patients in whom episodes occur at least once a month or who are at high risk of developing laryngeal edema require long-term prophylaxis. This often involves male sex hormones (androgens), which increase production of C1-INH in the liver through an as yet unknown mechanism. Danazol is the most commonly used.[1] The dose should be kept as low as possible because of its frequent adverse effects. The use of androgens is particularly problematic in children and they must not be taken during pregnancy. Several cases in which patients developed benign liver tumours during treatment with danazol resulted in the substance being taken off the market in Germany at the beginning of 2005.[citation needed]

As an alternative, drugs known as fibrinolysis inhibitors, such as tranexamic acid, are used, although their effect is comparatively weak and their potential for side effects is questionable.[citation needed]

Short-term prophylaxis

Short-term prophylaxis is normally administered before surgery or dental treatment. In Germany, C1-INH concentrate is used for this and given 1-11/2 hours before the procedure. In countries where C1-inhibitor concentrate is not available or only available in an emergency (laryngeal edema), high-dose androgen treatment is administered for 5–7 days.

Long-term prophylaxis

In those with frequent or unpredictable attacks, regular infusions of plasma or inhibitor concentrate may be used.[citation needed] C1-INH concentrate is not available in the US, so sometimes fresh frozen plasma is used. C1inh concentrate is currently under late-stage development for both acute and prophylactic use.[citation needed]

New treatment options

Clinical development of several new active substances, which intervene in the disease process in different ways, is currently ongoing.

Ecallantide is a peptide inhibitor of kallikrein that has received orphan status for HAE and has shown positive results in phase III trials.[2]

Icatibant (marketed as Firazyr) is a selective bradykinin receptor antagonist, which has been approved in only Europe and not in the USA.[3] After initial borderline results this drug was shown to be effective in phase III trials.[4]

Pharming, a Dutch biotechnology company, is developing a recombinant C1 inhibitor for acute attacks of hereditary angioedema.[5]

Cinryze [1] has been approved by the FDA in October 2008

No studies have been done on these agents in relation to HAE type III.[citation needed]

Doc James (talk · contribs · email) 04:15, 15 December 2010 (UTC) [reply]

References

  1. ^ Gelfand JA, Sherins RJ, Alling DW, Frank MM (1976). "Treatment of hereditary angioedema with danazol. Reversal of clinical and biochemical abnormalities". N. Engl. J. Med. 295 (26): 1444–8. doi:10.1056/NEJM197612232952602. PMID 792688.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  2. ^ Lehmann A (2008). "Ecallantide (DX-88), a plasma kallikrein inhibitor for the treatment of hereditary angioedema and the prevention of blood loss in on-pump cardiothoracic surgery". Expert Opin Biol Ther. 8 (8): 1187–99. doi:10.1517/14712598.8.8.1187. PMID 18613770. {{cite journal}}: Unknown parameter |month= ignored (help)
  3. ^ Jerini AG (2008-07-15). "Jerini Receives European Commission Approval for Firazyr (Icatibant) in the Treatment of HAE - Press release". Retrieved 2008-07-28.
  4. ^ Bernstein JA (2008). "Hereditary angioedema: a current state-of-the-art review, VIII: current status of emerging therapies". Ann. Allergy Asthma Immunol. 100 (1 Suppl 2): S41–6. doi:10.1016/S1081-1206(10)60585-6. PMID 18220151. {{cite journal}}: Unknown parameter |month= ignored (help)
  5. ^ http://news.nationalgeographic.com/news/2009/12/091201-rabbits-milk-human-protein-drug.html

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