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Cystic Fibrosis, Emergency Medicine

Reviewed 06/2017
 


Basics

Description

  • Defect of the cystic fibrosis transmembrane conductance regulator (CFTR)

  • CFTR functions as an ATP-regulated chloride channel that regulates the activity of chloride and sodium channel...

Diagnosis

Signs and Symptoms

  • General:

    • Failure to thrive

    • Recurrent respiratory tract infections

    • Anasarca in infancy

    • Salty taste of skin

  • Head, ears, eyes, nose, and throat (HEENT):

    • Nasal polyps; severe headach...

Treatment

Pre-Hospital

  • Transcutaneous pacing for unstable type II 2nd- or 3rd-degree block

  • Atropine:

    • Avoid with type II 2nd-degree block because it may precipitate 3rd-degree block.

Ed Treatment/Procedures

Follow-Up

Disposition

Admission Criteria

  • Pulmonary exacerbation with significant deterioration from baseline, hypoxemia, resistant bacteria, failure of outpatient therapy

  • Pneumothorax

  • Hemoptysis

  • Hematemesi...

Pearls and Pitfalls

  • With CF patients in respiratory distress, always consider pneumothorax: Obtain CXR.

  • For CF patients with abdominal pain/vomiting, always consider DIOS and intussusception

Additional Reading

  • Hoffman  LR, Ramsey  BW. Cystic fibrosis therapeutics: The road ahead. Chest.  2013;143:207–213.

  • Ryan  G, Jahnke  N, Remmington  T. Inhaled antibiotics for pulmonary exacerbatio...

Codes

ICD9

  • 277.00 Cystic fibrosis without mention of meconium ileus

  • 277.02 Cystic fibrosis with pulmonary manifestations

  • 277.03 Cystic fibrosis with gastrointestinal manifestations

  • 277.01 Cystic fibrosis ...

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