Common clinical features of CF (respiratory disease and exocrine pancreatic insufficiency)

Ranjani Somayaji; Kathleen J Ramos; Siddhartha G Kapnadak; Moira L Aitken; Christopher H Goss

doi:10.1016/j.lpm.2017.03.021

Common clinical features of CF (respiratory disease and exocrine pancreatic insufficiency)

Presse Med. 2017 Jun;46(6 Pt 2):e109-e124. doi: 10.1016/j.lpm.2017.03.021. Epub 2017 May 26.

Authors

Ranjani Somayaji¹, Kathleen J Ramos², Siddhartha G Kapnadak², Moira L Aitken², Christopher H Goss³

Affiliations

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 Medicine, Division of Pulmonary and Critical Care Medicine, Seattle, WA, USA; University of Washington, Department of Pediatrics, Division of Pediatric Pulmonology, Seattle, WA, USA; Seattle Children's Research Institute, Cystic Fibrosis Foundation Therapeutics Development Network Coordinating Center, Seattle, WA, USA. Electronic address: goss@u.washington.edu.

PMID: 28554722
DOI: 10.1016/j.lpm.2017.03.021

Abstract

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.

Publication types

Review

MeSH terms

Adolescent
Adult
Child
Child, Preschool
Cystic Fibrosis / diagnosis*
Cystic Fibrosis / mortality
Cystic Fibrosis / therapy
Disease Progression
Forced Expiratory Volume
Hemoptysis / diagnosis
Hemoptysis / mortality
Hemoptysis / therapy
Humans
Infant
Infant, Newborn
Longitudinal Studies
Lung Transplantation
Opportunistic Infections / diagnosis
Opportunistic Infections / mortality
Opportunistic Infections / therapy
Pneumothorax / diagnosis
Pneumothorax / mortality
Pneumothorax / therapy
Prognosis
Registries
Risk Factors
Survival Rate
Tomography, X-Ray Computed
Treatment Outcome
Young Adult