Prescrire international
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Prescrire international · Oct 2013
Controlled Clinical TrialFentanyl buccal film. New oral transmucosal form has no advantages in breakthrough pain.
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Cystic fibrosis is a serious genetic disease due to mutations in the gene encoding CFTR (Cystic FibrosisTransmembrane conductance Regulator), a protein involved in cellular transmembrane transport, particularly of chloride ions. Ivacaftor is described as a selective potentiator of CFTR protein. It is now licensed for the treatment of cystic fibrosis patients 6 years of age and older who carry the CFTR G551D mutation, which is the case for about 4% to 5% of cystic fibrosis patients. ⋯ Ivacaftorcarries a risk of numerous pharmacokinetic interactions that can require dose adjustment, particularly for patients with hepatic impairment and those receiving cytochrome P450 isozyme 3A inhibitors or inducers. Given the absence of data, ivacaftor should be avoided during pregnancy. In practice, in 2013, it is not possible to determine the harm-benefit balance of ivacaftor due to the high risk of drug interactions, as well as a possible increased risk of infections and liver toxicity.