CP2, cystic fibrosis

recurrent infections secondary to imparied cilia function and airway obstruction. chronic sinusitis, nasal poylps, lower resp infection.

mutant CFTR, Na absorption is increased, water absorption is increased and thick mucous becomes the problem-->predisposition for chronic infections

Cystic fibrosis transmembrane conductance regulator. This is the gene that is mutant in cystic fibrosis

**OBSTRUCTIVE pattern**, air trapping, progressive course, decreased compliance, decreased flow rates at low lung volumes. increased dead space, decreased exercise tolerance

obstructive

chronic infection, persistent productive cough, clubbing (from hypoxia), sinus disease, chronic airway obstruction, nasal polyps

hemoptysis, bronchiectasis, pneumothorax, respiratory failure, cor pulmonale

1) pseudomonas aeruoginosa (lower airway)
2) Staphylococcus Aureus
3) Haemophilus Influenze
4) Burkholderria Cepacia

Meconium ileus, recurrent distal intestinal obstrction, constipation, rectal prolapse, failure to thrive in infancy and childhood

billiary duct obstruction-->cystic duct becomes fibrotic, billiary stasis.
Cirrhosis of the liver, Portal hypertension (end stage liver failure)

mucus plugging of pancreatic ducts. Impaired HCO3 and fluid secretion in pancrease=pancreatic damage. Impaired absorption of ADEK. chronic diarrhea. Steatorrhea=fatty stool
type 1 diabetes

history and physical is primary
DNA analysis-CF mutations
+ sweat test, abnormal nasal transepithelial potential difference value*

Decrease clinical consequences. Direct treatment at sputum problem

regular visits to CF center
Airway clearance=chest PT, postural drainage, breathing exercises
mucus thinners,antibiotics, anti-inflammatory drugs, bronchodilators

high calorie, high protein diet. Pancreatic enzyme supplementation, Fat soluble vitamin replacement (ADEK)

15 million people in US. Increased 42% since 1982. COPD is 5th leading cause of death in US

excess mucus production for at lease 3 months during at least 2 years

an abnormal permanent enlargement of the air spaces accompanied by the destruction of their walls

empysema is an air space problem, bronchitis is a mucous problem

smoking, infection, pollution. Main risk factor is SMOKING

OBSTRUCTIVE disease. airway resistance due to mucus, edema and bronchial narrowing. V/Q mismatch=mucous plugging, hypoxemia, airway obstruction

slow progressiong, minor smoking cough

hypoxemia, dyspnea on exertion--> then at rest, cyanosis, cor pulmonale, clubbing, polycythemia (elevated RBCs because of stimulation of bone marrow in hypoxia)

emphysema

chronic bronchitis

smoking, chronic cough, hyperproduction of sputum

pulmonary funtion, obstructive pattern, elevation of PCO2, decreased PO2, CBC shows elevated hematocrit, Chest x-ray, CT scan

avoidance of respiratory irritants(tobacco primarily) smoking cessation=reduce cough and reduce sputum production
corticosteroids for COPD--short courses only
bronchodilators (B agonists=short acting and also long acting anticholinergics)
theophilline=helps with diaphram function and mucocilliary clearance, Antibiotics during exacerbation only, expectorants (not significantly helpful) cough suppressants (only short term if tired from nighttime coughing) nutritional supplementation, anti-anxiety/anti-depressants

improves quality of life in resp failure. goal is to keep saturatin >90%