5HYXHGHV0DODGLHV5HVSLUDWRLUHV$FWXDOLWpV ISSN 1877-1203
Revue des
Maladies
Respiratoires Actualités
Organe Officiel de la Société de Pneumologie de Langue Française
'LVSRQLEOH HQ OLJQH VXU
38e Congrès annuel de l’European Cystic Fibrosis Society Bruxelles – 10-13 juin 2015 Coordination : P.-R. Burgel
Cl–
ZZZVFLHQFHGLUHFWFRP
Cl– Cl–
wt-CFTR
Cl–
Cl– Cl–
Cl–
Cl–
I
II
III
IV
V
VI
No protein
No traffic
No Function
Less Function
Less Protein
Less Stable
G542X (a) 394delTT (a) 1717-1G>A (b)
R1066C A561E F508del
G551D S549R G1349D
R117H R334W
A455E 3272-26A>G 3849 + 10 kb C >T
c.120del23 rF508del
85453
Numéro réalisé avec le soutien institutionnel d’Aptalis Pharma SAS
www.splf.org
Décembre Vol. 7 2015
N°
5
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3,0%
11,1%
53,8% 0,4% 0,7%
1,8%
22,8%
0,8%
Aztreonam (3,7%)
2009 (N = 12,900)
Aztreonam (40,3%)
2012 (N = 13,845)
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