Guidelines for the diagnosis and management of Cystic Fibrosis

GENETICS
PATHOGENESIS
Respiratory System
Infection
Pancreas
Intestines
Liver
Skin
Reproductive System
CLINICAL FEATURES
Presenting Features
At birth
In the first year
In the pre-school age child
In the school age child
In adolescence and adult life
DIAGNOSIS
The Sweat Test
Genetic Testing
Neonatal Screening
Tests for Pancreatic Insufficiency

MANAGEMENT
The Newly-Diagnosed Patient
Surveillance
Routine Management
Treatment of Pulmonary Manifestations
Physiotherapy
Exercise
Treatment of respiratory infection
Staphylococcus aureus
Haemophilus influenzae
Pseudomonas aeruginosa
Aerosol antibiotics
Burkholderia cepacia
Aspergillus
Other Respiratory Therapy
Gene Therapy
Pulmonary Function Tests
Treatment of Pancreatic Insufficiency
Nutritional Management
Treatment of Meconium Ileus
Treatment of Distal Intestinal Obstruction Syndrome (DIOS)
Management of Late and Less Common Complications
FURTHER READING
ACKNOWLEDGEMENTS
ANNEX Physiotherapy in the Treatment of Cystic Fibrosis