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■ Nasal potential difference: Directly evaluates CFTR function by measur-

ing ion transport in the epithelial cells lining the interior of the nose

■ CXR:Shows hyperinflation, bronchiectasis, and upper lobe infiltrates;

nodules often represent mucoid impaction in the airways

TREATMENT

■ Acute pulmonary exacerbations: Treat with chest physical therapy, bron-

chodilators, DNase, and usually two antipseudomonal antibiotics.

■ Chronic stable CF

■ Inhaled tobramycin: Slows the decline in FEV 1 and is used for long-

term therapy

■ Nebulized DNase: Improves FEV 1 and should be offered to patients

with daily cough, sputum production, and airflow obstruction

■ Azithromycin:Improves FEV 1 and reduces pulmonary exacerbations

in those infected with Pseudomonas

■ Aerobic exercise, flutter devices, external percussive vests: Help with

regular airway clearance

■ Pancreatic enzymes and the fat-soluble vitamins A, D, E, and K:

Given for malabsorption

■ Nutritional counseling: Essential for proper health maintenance and

to help prevent diabetic complications, osteoporosis, and weight loss

■ Double-lung transplantation: Remains an option for severe progres-

sive pulmonary disease

SARCOIDOSIS

This is a systemic autoimmune disorder that primarily affects the lungs and

lymphatics and is characterized by noncaseating granulomas. It is primarily a

self-limited disease of young and middle-aged adult black Americans or those

of Scandinavian descent.

SYMPTOMS/EXAM

■ Nonspecific constitutional symptoms such as fever, fatigue, anorexia,

weight loss, and arthralgias

■ Physical examination may reveal dry crackles, lymphadenopathy, parotid

enlargement, splenomegaly, uveitis, or skin changes (erythema nodosum).

DIFFERENTIAL

Tuberculosis, fungal infections, rheumatoid arthritis, lymphoma, Wegener

granulomatosis

DIAGNOSIS

■ Suspect based on findings of bilateral hilar adenopathy, pulmonary infil-

trates, and skin lesions

■ Diagnosis is made by a combination of clinical, radiographic, and histo-

logic findings along with exclusion of other diseases that have a similar

clinical picture.

TREATMENT

Systemic corticosteroids

THORACIC AND RESPIRATORY

DISORDERS