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Review Question - QID 100812

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QID 100812 (Type "100812" in App Search)
A 5-year-old Caucasian boy presents with a productive cough that yields thick sputum. His past medical history is significant for three prior pulmonary infections, two of which cultured S. aureus and one that was positive for Pseudomonas. Chest radiograph today is shown in Figure A. Which of the following pharmacologic agents act most directly to counteract the defective physiologic process in these patients?
  • A

Ipratroprium

10%

11/111

Beclomethasone

13%

14/111

Albuterol

6%

7/111

Zileuton

9%

10/111

N-acetylcysteine

61%

68/111

  • A

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This child has cystic fibrosis (CF), and thus produces viscous mucous as a result of impaired chloride channels (CFTR). N-acetylcysteine (NAC) is a mucolytic that reduces the viscosity of the mucous.

N-acetylcysteine's mechanism of action is achieved through the cleavage of disulfide bonds of glycoproteins in the mucus. This helps to loosen the plug and allows the patient to clear the accumulated mucous more easily. NAC is not routinely given CF patients unlike inhaled DNase or hypertonic saline for mucous clearance.

The review article by Boyle explains that CF is characterized by chronic pulmonary infection and bronchiectasis, pancreatic exocrine impairment, and elevated sweat chloride. Combined changes in dietary management and new treatments have extended the life expectancy of patients with CF to a median age of 35. However, this improvement in life expectancy may also be confounded by the enhanced recognition and management of mild, previously under-diagnosed cases.

Weiss explains that in the evaluation of children with persistent wheeze, CF should be considered in those with recurrent respiratory infections or those with failure to thrive. More common causes of wheeze in children include asthma, allergies, infections, gastroesophageal reflux disease, and obstructive sleep apnea. When suspicious for CF, further work-up can include ciliary function testing, immunoglobulin levels to test for immune deficiency, and sweat chloride testing.

Figure A displays a chest radiograph characteristic of CF with patchy atelectasis.

Incorrect answers:
Answer 1: Ipratroprium is an inhaled anti-muscarinic drug used to relieve cholinergic broncoconstriction in asthma therapy.
Answer 2: Beclomethasone is an inhaled corticosteroid that prevents upregulation of NF-KB and subsequent inflammatory mediators in in chronic asthma therapy.
Answer 3: Albuterol is a short acting B2-agonist used for symptomatic relief of acute asthma.
Answer 4: Zileuton inhibits 5-lipoxygenase and prevents formation of inflammatory leukotrienes.

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