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Cystic Fibrosis

Reviewed 06/2020
 


BASICS

DESCRIPTION

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

DIAGNOSIS

Criteria for diagnosis of cystic fibrosis (1)[A
  • At least one of the following:

    • One or more typical phenotypic features of CR:

      • Chronic pulmonary disease

      • Chronic sinusitis

      • Characteristic gastroin...

TREATMENT

GENERAL MEASURES

  • Cystic Fibrosis Foundation guidelines call for yearly evaluation:

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

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
Farrell PM, White TB, Ren CL, et al. Diagnosis of cystic fibrosis: consensus guidelines from the Cystic Fibrosis Foundation. J Pediatr.  2017;181S:S4.e1–S15.e1.
2
Mogayzel PJJr, Naureck...

ADDITIONAL READING

  • Conwell LS, Chang AB. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev.  2014;(3):CD002010.

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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