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Chapter 16. Cystic Fibrosis

Airway clearance therapy in patients with cystic fibrosis (CF) should be performed by the patient or patient’s caregiver:

A. Only when the patient is symptomatic

B. At least three times per day during acute exacerbations

C. Every Monday, Wednesday, and Friday

D. Only under the direct supervision of a respiratory therapist

E. With at least two different methods

A 5-year-old male CF patient presents with poor nutritional status. Height is 105 cm (25th percentile), and weight is 14.5 kg (< 3rd percentile). He takes Zenpep 10,000 two caps with meals and one cap with snacks, and he has four loose stools per day. Which of the following are appropriate nutritional interventions?

A. Dietary counseling to increase caloric intake

B. Increase Zenpep 10,000 to three caps with meals and two caps with snacks

C. Admit to hospital for initiation of intravenous parenteral nutrition

D. A and B

E. A, B, and C

Dornase alfa is used in CF for which of the following reasons:

A. Chronic Pseudomonas aeruginosa suppression

B. Mucolytic activity

C. Sputum induction

D. A and B

E. B and C

The following culture and sensitivity results are reported for a 13-year-old CF patient:

Current month: P. aeruginosa (sensitive to cefepime, ceftazidime, piperacillin-tazobactam, meropenem, aztreonam, gentamicin, tobramycin, amikacin, ciprofloxacin).

One month ago: S. aureus (sensitive to vancomycin, sulfamethoxazole-trimethoprim, minocycline, linezolid; resistant to cefazolin, clindamycin, erythromycin, nafcillin, gentamicin) and P. aeruginosa (sensitive to cefepime, ceftazidime, piperacillin-tazobactam, meropenem, aztreonam, tobramycin, amikacin; resistant to gentamicin, ciprofloxacin)

Six months ago: A. xylosoxidans (sensitive to ceftazidime, sulfamethoxazole-trimethoprim, and piperacillin-tazobactam; resistant to ticarcillin-clavulanate, minocycline, levofloxacin)

Based on these results, the most appropriate inpatient antibiotic regimen is:

A. Piperacillin-tazobactam, ciprofloxacin, and vancomycin

B. Piperacillin-tazobactam, tobramycin, and vancomycin

C. Cefepime, tobramycin, and clindamycin

D. Cefepime, tobramycin, and minocycline

E. Ceftazidime, gentamicin, and sulfamethoxazole-trimethoprim

Patients with a history of anaphylactic reaction to ticarcillin-clavulanate and cefepime ...

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