Transplantation
Despite improvement in median life expectancy and overall health, some children with cystic fibrosis (CF) progress to end-stage lung or liver disease and become candidates for transplant. Transplants for children…
PEDIATRICS
Endocrine Disorders in Cystic Fibrosis
Cystic fibrosis is frequently complicated by endocrine disorders. Diabetes can be expected to affect most with CF and pancreatic insufficiency and varies widely in age of onset, but early identification…
Gastrointestinal, Pancreatic, and Hepatobiliary Manifestations of Cystic Fibrosis
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…
Nutrition and Growth in Cystic Fibrosis
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 Disease
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…
Cystic Fibrosis
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…
Cystic Fibrosis Diagnosis and Newborn Screening
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…
Molecular Genetics of Cystic Fibrosis Transmembrane Conductance Regulator
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…
Background and Epidemiology
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…
Cystic Fibrosis
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…
