Abstract
Cystic fibrosis (CF) is recognized as a serious, life-limiting autosomal recessive inherited condition, affecting multiple organs. Improvements in both diagnosis and management of CF has led to the recognition of a range of phenotypes, from mild to severe, with varying rates of disease progression. Pulmonary disease is still the main predictor of morbidity and mortality associated with CF (1). There are currently nearly 2000 recognized cystic fibrosis trans-membrane regulator (CFTR) mutations.
Keywords
Cystic Fibrosis
Shwachman
Copyright
© 2013, Author(s). This open-access article is available under the Creative Commons Attribution 4.0 (CC BY 4.0) International License (https://creativecommons.org/licenses/by/4.0/), which allows for unrestricted use, distribution, and reproduction in any medium, provided that the original work is properly cited.