Respiratory
Acute exacerbations lead to increase in baseline cough and sputum production
Pneumonia
Chronic colonisation with multiple organisms
Staph aureus and H. influenzae common in childhood
Most become chronically colonised with pseudomonas aeruginosa and/or virulent gram-negative bacteria
Colonisation with pseudomonas aeruginosa usually occurs by late adolescence
Pseudomonas features include: severe pneumonia, cyanosis, confusion, often bilateral, occasionally empyema
Higher risk for aspergillosis
Increased risk of pneumothorax (8%–20% will develop one in lifetime)
Bronchitis
Sinusitis and nasal polyps
Chronic inflammation and infection lead to bronchiectasis and angiogenesis (may have hemoptysis)
Long-standing disease can lead to cor pulmonale
Gastrointestinal
Meconium ileus: failure to pass meconium within first 48 hours of life
Earliest clinical manifestation of disease
Occurs in 10 - 20% of those diagnosed
90% of babies with meconium ileus have<unk> 
Obstruction due to thick meconium
Can lead to perforation if unrecognized
Diagnosed and treated with hyperosmolar contrast enema
Pancreatic insufficiency
Often leads to failure to thrive in infancy
Pancreatitis
Diarrhea and malnutrition due to resultant malabsorption
Other
Electrolyte disturbances
Chloride wasting and diarrhea can lead to hypokalemic, hypochloremic metabolic alkalosis
Suppurative parotitis
Rapid onset parotitis (warm, swollen, tender gland, fever, trismus)
Purulent drainage from Stensen's duct
Organisms: staph, strep pneumo, strep pyogenes, H. flu, e. coli, pseudomonas
Acute exacerbations lead to increase in baseline cough and sputum production
Pneumonia
Chronic colonisation with multiple organisms
Staph aureus and H. influenzae common in childhood
Most become chronically colonised with pseudomonas aeruginosa and/or virulent gram-negative bacteria
Colonisation with pseudomonas aeruginosa usually occurs by late adolescence
Pseudomonas features include: severe pneumonia, cyanosis, confusion, often bilateral, occasionally empyema
Higher risk for aspergillosis
Increased risk of pneumothorax (8%–20% will develop one in lifetime)
Bronchitis
Sinusitis and nasal polyps
Chronic inflammation and infection lead to bronchiectasis and angiogenesis (may have hemoptysis)
Long-standing disease can lead to cor pulmonale
Chronic colonisation with multiple organisms
Staph aureus and H. influenzae common in childhood
Most become chronically colonised with pseudomonas aeruginosa and/or virulent gram-negative bacteria
Colonisation with pseudomonas aeruginosa usually occurs by late adolescence
Pseudomonas features include: severe pneumonia, cyanosis, confusion, often bilateral, occasionally empyema
Higher risk for aspergillosis
Colonisation with pseudomonas aeruginosa usually occurs by late adolescence
Meconium ileus: failure to pass meconium within first 48 hours of life
Earliest clinical manifestation of disease
Occurs in 10 - 20% of those diagnosed
90% of babies with meconium ileus have<unk> 
Obstruction due to thick meconium
Can lead to perforation if unrecognized
Diagnosed and treated with hyperosmolar contrast enema
Pancreatic insufficiency
Often leads to failure to thrive in infancy
Pancreatitis
Diarrhea and malnutrition due to resultant malabsorption
Earliest clinical manifestation of disease
Occurs in 10 - 20% of those diagnosed
90% of babies with meconium ileus have<unk> 
Obstruction due to thick meconium
Can lead to perforation if unrecognized
Diagnosed and treated with hyperosmolar contrast enema
Often leads to failure to thrive in infancy
Electrolyte disturbances
Chloride wasting and diarrhea can lead to hypokalemic, hypochloremic metabolic alkalosis
Suppurative parotitis
Rapid onset parotitis (warm, swollen, tender gland, fever, trismus)
Purulent drainage from Stensen's duct
Organisms: staph, strep pneumo, strep pyogenes, H. flu, e. coli, pseudomonas
Chloride wasting and diarrhea can lead to hypokalemic, hypochloremic metabolic alkalosis
Rapid onset parotitis (warm, swollen, tender gland, fever, trismus)
Purulent drainage from Stensen's duct
Organisms: staph, strep pneumo, strep pyogenes, H. flu, e. coli, pseudomonas
Cystic fibrosis
Cerebral palsy
Bronchopulmonary dysplasia
Brash syndrome
Tracheomalacia
Scleroderma
Down syndrome
Idiopathic pulmonary fibrosis