Pulmonary System

Chronic lung disease is a hallmark of CF, leading to death in 90% of patients.3 Pulmonary disease is characterized by thick mucus secretions, impaired mucus clearance, chronic airway infection and colonization, obstruction, and an exaggerated neutrophil-dominatedinflammatory response.4 Over time, chronic obstruction and inflammations lead to air trapping, atelectasis, mucus plugging, bronchiectasis, cystic lesions, pulmonary hypertension, and eventual respiratory failure. In the U.S.-CF population, pulmonary function declines at an average yearly rate of 2%, as measured by forced expiratory volume in 1 second (FEV1). The rate in an individual patient may be higher or lower depending on severity of CFTR dysfunction and comorbidit-ies. Patients may show a slow steady decline over time, or they may have stable lung function with intermittent periods of sharp decline.1 In the upper airways, sinusitis and nasal polyps are also common, and microbial colonization is similar to that of the lungs.

FIGURE 16-1. Electrolyte transport in the airway epithelial cell. (Ca, calcium; cAMP, cyclic-3', 5'-adenosine monophosphate; CI, chloride; Na, sodium; K potassium.) (From Milavetz G, Smith JJ. Cystic fibrosis. In: DiPiro JT, Talbert RL, Yee GC, et al. (eds.) Pharmacotherapy: A Pathophysiologic Approach. 7th ed. New York: McGraw-Hill, 2008: 536.)

FIGURE 16-1. Electrolyte transport in the airway epithelial cell. (Ca, calcium; cAMP, cyclic-3', 5'-adenosine monophosphate; CI, chloride; Na, sodium; K potassium.) (From Milavetz G, Smith JJ. Cystic fibrosis. In: DiPiro JT, Talbert RL, Yee GC, et al. (eds.) Pharmacotherapy: A Pathophysiologic Approach. 7th ed. New York: McGraw-Hill, 2008: 536.)

Bacterial pathogens are often acquired in an age-dependent sequence, and prevalence is tracked in the Cystic Fibrosis Foundation Patient Registry. Early infection is most often caused by Staphylococcus aureus and nontypeable Haemophilus influenzae (and thus is not prevented by childhood H. influenzae type b immunization). Pseudomonas aeruginosa infection also occurs early in life and is the most significant CF pathogen among all age groups. P. aeruginosa expresses extracellular toxins that perpetuate lung inflammation. Mucoid strains of P. aeruginosa produce an alginate biofilm layer that interferes with antibiotic penetration. Other organisms identified later in the disease course include Stenotrophomonas maltophilia, Achromobacter (Al-caligenes) xylosoxidans, Burkholderia cepacia, fungi including Candida and Aspergillus species, and nontuberculous mycobacteria.1 Other organisms may also present chronically or intermittently. Similarly, cultured organisms may represent an initial infection, chronic colonization, or microbial overgrowth in an acute exacerbation.

How To Get Rid Of Yeast Infections Once And For All

How To Get Rid Of Yeast Infections Once And For All

No more itching, odor or pain or your money is refunded! Safe and DRUG FREE Natural Yeast Infection Solutions Are you looking for a safe, fast and permanent cure for your chronic yeast infection? Get Rid of that Yeast Infection Right Now and For Good!

Get My Free Ebook


Post a comment