επικτητο αγγειοοιδημα αντίγραφο Koytaλωνης τελικό

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Κληρονομικό & Επίκτητο Αγγειοοίδημα ΙΣΤΟΡΙΚΗ ΑΝΑΔΡΟΜΗ

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Transcript of επικτητο αγγειοοιδημα αντίγραφο Koytaλωνης τελικό

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  • 2010

    1876 John Laws Milton giant urticaria

    * 16 , John Laws Milton 1876, giant urticaria

  • 2011

    1882Quincke

    * 1882 Quinke 3 - , ,

  • 2011

    1888 William Osler

    * (1888) William Osler 5 4. .

  • 2011

    1962N. Landerman A &

    *And in 1962, Landerman and his coworkers published this paper in the Journal of Allergy,identifying that patients with hereditary angioedema had a deficiency of an inhibitor for serumglobulin permeability factor and/or plasma kallikrein. Unfortunately, while they identified theabsence of this activity, they never identified the protein. So this observation really didnt get theattention that it deserved.

  • 2011

    1963 Donaldson Evans : C1

    *A year later, however, Virginia Donaldson made what was the seminal observation, which is thatthe sera of patients with hereditary angioedema lacked a protein, which was called C1 inhibitor.And that name basically stuck with the protein, and it really focused a lot of attention in the fieldon complement proteins. Ill discuss the implications of that in a couple of minutes.

  • F XIIF XIIaHMWK-F XIF XIa

    TPA/

    10if anything activates factor XII, the patient is set up to make massive amounts of bradykinin without stopping the cascade, for a long period of time. The bradykinin-forming cascade becomes linked to the complement cascade and further activates it.

  • 65.

    08/ 2008 1 9 /2009 2 // 11 /2009 3 // 3 /2010 4 // 5/2010 5 // 8/2010 6 // ( )

  • C4 = 2.5 ( : 10.0 40.0)C1q= 7.9 (: 10.5 17.6)C1inh= 11.8 (: 17.00 32.00)

    ( . )

  • . Ag c1-inh N N ./c1-inh NN or c3 NNN c4 N c1q NNN

  • 10/12/ 2011 (ing FYRAZYR ) 14/1/2011 // // // 31/1/2011 // // // 17/2/2011 // // // ????????

  • C1inh. : ( )Churg-Straus (IgM ) C1inh.

  • , 1. . . ..C..(Epsilon Animo CaproicAcid 2.5g -5gx4 /.) . TRANXAIC ACID(transamin 250-500mgx3/ .)2. . Danazol (danatrol 200mg x3 .)3. ( )4.( & C1inh)

  • C 1inh iv10U/Kg UI (2Fl) 30-45 min 2

    ()30 mg sc 45 min 2-4

  • F XIIF XIIaHMWK-F XIF XIa

    TPA/

    10if anything activates factor XII, thepatient is set up to make massive amounts of bradykinin without stopping the cascade, for a long period of time.The bradykinin-forming cascade becomes linked to the complement cascade and further activates it.

  • (FYRAZYR) (, , ) , BKR-2 >1250 subjects phase I-III Firazyr 2008.

  • DANAZOL : , , , , , libido ACA (10-20gr/): , , TRANSAMINE : 1-2gr/d EACA , .

  • Icatibant , 1-2 h

  • * 16 , John Laws Milton 1876, giant urticaria * 1882 Quinke 3 - , , * (1888) William Osler 5 4. .*And in 1962, Landerman and his coworkers published this paper in the Journal of Allergy,identifying that patients with hereditary angioedema had a deficiency of an inhibitor for serumglobulin permeability factor and/or plasma kallikrein. Unfortunately, while they identified theabsence of this activity, they never identified the protein. So this observation really didnt get theattention that it deserved.

    *A year later, however, Virginia Donaldson made what was the seminal observation, which is thatthe sera of patients with hereditary angioedema lacked a protein, which was called C1 inhibitor.And that name basically stuck with the protein, and it really focused a lot of attention in the fieldon complement proteins. Ill discuss the implications of that in a couple of minutes.10if anything activates factor XII, the patient is set up to make massive amounts of bradykinin without stopping the cascade, for a long period of time. The bradykinin-forming cascade becomes linked to the complement cascade and further activates it.

    10if anything activates factor XII, thepatient is set up to make massive amounts of bradykinin without stopping the cascade, for a long period of time.The bradykinin-forming cascade becomes linked to the complement cascade and further activates it.