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Cystic Fibrosis

Ryan D. Lurtsema, MD and Nica E. Lurtsema, MD, MPH Reviewed 06/2022
 


BASICS

DESCRIPTION

  • Cystic fibrosis (CF) is an autosomal recessive mutation that most prominently affects the pulmonary and pancreatic systems, but may involve any organ system.

  • Due to improvements in me...

DIAGNOSIS

Criteria for diagnosis of CF 
  • At least one of the following:

    • One or more typical phenotypic features of CF:

      • Chronic pulmonary disease

      • Chronic sinusitis

      • Characteristic GI and...

TREATMENT

GENERAL MEASURES

  • Cystic Fibrosis Foundation guidelines call for:

    • Four office visits, four respiratory cultures, PFTs q6mo, and at least one evaluation by a multidisciplinary team, including di...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Upon discharge for a pulmonary exacerbation, follow-up with CF specialist within 2 to 4 weeks

  • Routine clinic visits every 3 months with airway cultures and PFTs as...

REFERENCES

1
Cystic Fibrosis Foundation. Understanding Changes in Life Expectancy. https://www.cff.org/Research/Researcher-Resources/Patient-Registry/Understanding-Changes-in-Life-Expectancy/. ...

CODES

ICD10

  • E84.9 Cystic fibrosis, unspecified

  • E84.11 Meconium ileus in cystic fibrosis

  • E84.0 Cystic fibrosis with pulmonary manifestations

  • E84.19 Cystic fibrosis with other intestinal manifestations

  • E84.8...

CLINICAL PEARLS

  • Meconium ileus is virtually pathognomonic for CF.

  • When sweat test is equivocal, CFTR genetic testing is diagnostic.

  • Consider CF in any child with chronic diarrhea, especially with poor g...

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