This concise manual provides clinicians and other related health care professionals with an essential reference tool to the background of cystic fibrosis, and the management and treatment of this disease. The latest guidelines are reviewed and current and emerging treatments are discussed in the latter chapters. Cystic fibrosis is an inherited condition where a mutation in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) causes loss of function. The dysfunction of CFTR results in the production of thick mucus in the lungs and digestive tract, causing pulmonary and gastrointestinal manifestations. The incidence of cystic fibrosis in Europe and the US ranges between 1 in 2,000 and 1 in 25,000.

Amy G Filbrun, MD, MS, is Clinical Associate Professor of Pediatrics at University of Michigan and C.S. Mott Childrens Hospital, in Ann Arbor, MI USA. She received her BS in Speech from Northwestern University and her MD from Ohio State University. She completed her pediatric residency at The Cleveland Clinic, in Cleveland, OH and fellowship in pediatric pulmonology at Columbus Childrens Hospital (now Nationwide Childrens Hospital) in Columbus, OH USA. She received her MS in Clinical Research Design and Statistical Analysis from University of Michigan. Her research interests include the evaluation of early childhood lung diseases, particularly measures of lung function in infants and young children with chronic respiratory diseases. In addition, Dr. Filbrun participates in numerous clinical trials in patients with cystic fibrosis.

Thomas Lahiri, MD, is Professor of Pediatrics at the University of Vermont College of Medicine and the Director of Pediatric Pulmonology at The University of Vermont Childrens Hospital in Burlington, US. He received his BS in Biology at McGill University and his MD from the University of Connecticut School of Medicine. He completed his pediatric residency training at the University of Vermont and a fellowship in pediatric pulmonology at Boston Childrens Hospital. His clinical interests include airway disorders and cystic fibrosis. He recently co-chaired the Cystic Fibrosis Preschool Guidelines Committee.

Clement L Ren, MD, MS, is Professor of Clinical Pediatrics at Indiana University School of Medicine and the James Whitcomb Riley Hospital for Children, both in Indianapolis, IN USA. He received his AB in Biophysics from the University of California at Berkeley and his MD from the University of Chicago. He completed his pediatric residency and allergy/immunology fellowship at Boston Childrens Hospital and a fellowship in pediatric pulmonology at St. Christophers Hospital for Children in Philadelphia, US. His research has focused on cystic fibrosis epidemiology, newborn screening, and evaluation of early lung disease in infants and young children with cystic fibrosis. He has held numerous leadership positions in the US Cystic Fibrosis Foundation.

Introduction and epidemiology of cystic fibrosis.- Pathophysiology of cystic fibrosis.- Clinical features and complications of cystic fibrosis.- Diagnosis of cystic fibrosis.- Monitoring and evaluation of patients with cystic fibrosis.- Treatment of cystic fibrosis.