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Cystic Fibrosis

Fozia Akhtar Ali, MD, Reethu K. Nayak, MD and Deepika Ram, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Cystic fibrosis (CF) is an autosomal recessive genetic mutation (CFTR gene) that most prominently affects the pulmonary and pancreatic systems.

  • The GI, endocrine, and reproductive sys...

DIAGNOSIS

HISTORY

  • Suspect with failure to thrive, steatorrhea, and recurrent respiratory problems.

    • Chronic/recurrent respiratory symptoms, including airway obstruction and infections

    • Persistent infiltr...

TREATMENT

GENERAL MEASURES

  • CF Foundation Guidelines call for yearly evaluation:

    • Four office visits, four respiratory cultures, PFTs q6mo, and at least 1 evaluation by a multidisciplinary team includin...

ONGOING CARE

FOLLOW UP RECOMMENDATIONS

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

  • Routine clinic visits every 3 months, with airway cultures and pulmona...

REFERENCES

1
1 Cystic Fibrosis Foundation. Patient Registry: Annual Data Report to the Center Directors 2013. Bethesda, MD: Cystic Fibrosis Foundation; 2014.
2
2 Grigoriadis C, Tympa A, Theodoraki K. Cys...

ADDITIONAL READING

  • Conwell LS, Chang AB. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev.  2014;(3):CD002010. {L-End} {L-End} [View Abstract on Ovid...

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.

  • CF must be considered in any child with chronic diarrhea, especially i...

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