Common clinical features of CF (respiratory disease and exocrine pancreatic insufficiency) - 01/08/17

Doi : 10.1016/j.lpm.2017.03.021

Ranjani Somayaji ¹, Kathleen J. Ramos ², Siddhartha G. Kapnadak ², Moira L. Aitken ², Christopher H. Goss ^2,^3,^4,^⁎
¹ University of Calgary, Department of Medicine, Calgary, AB, Canada
² University of Washington, Department of Medicine, Division of Pulmonary and Critical Care Medicine, Seattle, WA, USA
³ University of Washington, Department of Pediatrics, Division of Pediatric Pulmonology, Seattle, WA, USA
⁴ Seattle Children's Research Institute, Cystic Fibrosis Foundation Therapeutics Development Network Coordinating Center, Seattle, WA, USA

^⁎Christopher H. Goss, University of Washington Medical Center, Pulmonary and Critical Care Medicine, Campus Box 356522, 1959 N.E. Pacific, Seattle, WA 98195, USA.

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Summary

First described as a disease of the pancreas, cystic fibrosis is a genetically inherited progressive disease affecting multiple organ systems. Pulmonary and pancreatic involvement is common in individuals with cystic fibrosis, and the former is attributable to most of the mortality that occurs with the condition. This chapter provides an overview of a clinical approach to the pulmonary and pancreatic manifestations of cystic fibrosis.

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