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MINERVA PNEUMOLOGICA

A Journal on Diseases of the Respiratory System


Official Journal of the Italian Society of Thoracic Endoscopy
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REVIEWS  WHAT’S NEW IN RESPIRATORY MEDICINE IN 2010 - PART II


Minerva Pneumologica 2010 June;49(2):253-8

language: English

Aggiornamento sulla fibrosi cistica

Anselmo M. A. 1, Lands L. C. 2

1 Division of Respiratory Medicine, Alberta Children’s Hospital, Calgary, Alberta, Canada;
2 Division of Respiratory Medicine, Montreal Children’s Hospital, Montreal, Quebec, Canada


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The median survival of the patient with cystic fibrosis (CF) continues to increase and the average age of the population is well into early adulthood. The treatment continues to evolve. Established therapies, such as chronic macrolide use, have been extended to include other potential patients. This success has brought new challenges to our clinics, including CF related bone disease. Although the mechanism of osteopenia is multifactorial, the treatment may lie in simple changes in daily activity and diet. While the successes are accumulating for our patients, the cure remains elusive. Until recently, we have been treating the effects of the dysfunction of the cystic fibrosis transmembrane regulator (CFTR), and not the basic pathophysiology. Newer tailored therapies have been designed to increase chloride channel function of non-CFTR channels, increase the production of functional CFTR or increase the percentage of nascent protein that reaches the cell surface. This tailored therapy will be class defect driven and has the possibility of many being used in a complimentary fashion. This review will make note of specific recent advancements in tailored therapy, while reminding us of the simple things we can do with diet and exercise.

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