Clinical Scoring Systems in Cystic Fibrosis – What Are the Options for Developing Countries?

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.