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Contents


Supplement Contents


Pathophysiology 02 Pathophysiology of cystic fibrosis: an overview Martin J Hug


Mechanism of disease 06 CFTR and the cystic fibrosis disease mechanism Iwona M Pranke and Isabelle Sermet-Gaudelus


Novel therapeutic approaches 11 Novel therapeutic approaches addressing the basic defect in cystic fibrosis Margarida D Amaral and Carlos M Farinha


Existing drug therapies 14 Existing supportive drug therapies Jane C Davies and Andrew Bush


Editor-in-Chief Theresa Saklatvala PhD


Commissioning Editor Andrea Porter


Art Director Evelyn Bercott


Adherence and burden 18 Adherence and burden of cystic fibrosis therapy Doris Staab


Ivacaftor 20 Ivacaftor: a quantum leap in treating cystic fibrosis? Martin J Hug


Sales Director Neil Morris


General Manager Alex Beaumont


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