Pulmonary disease is the primary cause of morbidity and mortality in people with cystic fibrosis (CF), but significant involvement within gastrointestinal, pancreatic, and hepatobiliary systems occurs as well. As in…
Close attention to nutrition and growth is essential in caring for children with cystic fibrosis (CF). Growth and nutritional status should be monitored as part of routine CF care. Children…
Respiratory system involvement in cystic fibrosis is the leading cause of morbidity and mortality. Defects in the cystic fibrosis transmembrane regulator (CFTR) gene throughout the sinopulmonary tract result in recurrent…
The earliest descriptions of lung disease in people with cystic fibrosis (CF) showed the involvement of 3 interacting pathophysiologic elements in CF airways: mucus obstruction, inflammation, and infection. Over the…
The diagnosis of cystic fibrosis (CF) has evolved over the past decade as newborn screening has become universal in the United States and elsewhere. The heterogeneity of phenotypes associated with…
The cystic fibrosis (CF) transmembrane conductance regulator ( CFTR ) gene encodes an epithelial ion channel. Although one mutation remains the most common cause of CF (F508del), there have been…
Cystic fibrosis (CF) is the most common autosomal-recessive disease in white persons. Significant advances in therapies and outcomes have occurred for people with CF over the past 30 years. Many of…
Susan G. Marshall, MD, Editor Drucy Borowitz, MD, Editor Living with Cystic Fibrosis (CF) is a challenge, but advances in medical care for people with CF have opened new opportunities…
Bonita F. Stanton, MD, Consulting Editor Our understanding of cystic fibrosis (CF) has exploded over the last several decades, with substantial advances in disease treatment and life expectancy. Given dramatic…
PEDIATRIC CLINICS OF NORTH AMERICA www.pediatric.theclinics.com Consulting Editor BONITA F. STANTON August 2016 • Volume 63 • Number 4
