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Titlebook: Cystic Fibrosis; A Multi-Organ System Stephanie Duggins Davis,Margaret Rosenfeld,James C Book 2020 Springer Nature Switzerland AG 2020 cyst

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https://doi.org/10.1057/978-1-137-51683-1ablishment of best practices on duration. This chapter reviews the definition, epidemiology, current treatment practices, prognosis, and associated outcomes related to CF PEx. A patient perspective highlights the burden of PEx on the lives of individuals with CF. Current research is reviewed to clar
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https://doi.org/10.1057/978-1-137-51683-1atment regimens. In addition, clinical practice guidelines have been developed worldwide to standardize the treatment of CF pulmonary disease and updated to include the CFTR modulator therapies. How the CFTR modulator therapies might alter the present recommendations for maintenance pulmonary therap
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Book 2020s throughout, informing practice standards and considerations. This is an ideal guide for pediatric and adult physicians who care for patients with cystic fibrosis, as well as respiratory therapists, physical therapists, nurses, nutritionists, and pharmacists who care for these patients..
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Diagnosis of Cystic Fibrosisentified. Infants with CRMS/CFSPID present with a positive NBS, but inconclusive diagnostic testing. Although most CRMS/CFSPID infants appear healthy, a small proportion may develop clinical features concerning CF. Some of these infants even have their diagnosis changed to CF. In general, sweat chlo
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Fungal Infections and ABPAe diversity, complexity and resistance profile of the CF mycobiome. System-based analyses of microbial-host interaction have further uncovered novel paradigms in CF pathogenesis including inter-kingdom signalling and the gut-lung axis, where fungi play an emerging and critical role. Current treatmen
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Inflammation in CF: Key Characteristics and Therapeutic Discovery drugs and efforts to develop gene-based therapies agnostic to CFTR mutations are key advances in the current era of CF science and medicine. It is exciting to consider how these interventions may influence the inflammatory state within the CF airway, but a need for direct anti-inflammatory interven
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Pulmonary Exacerbationsablishment of best practices on duration. This chapter reviews the definition, epidemiology, current treatment practices, prognosis, and associated outcomes related to CF PEx. A patient perspective highlights the burden of PEx on the lives of individuals with CF. Current research is reviewed to clar
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