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Cystic Fibrosis, Pediatric

Samuel B. Goldfarb, MD and Bruce A. Ong, LTC, MC, MD, MPH, USA Reviewed 10/2018
 


BASICS

DESCRIPTION

Cystic fibrosis (CF) is an inherited autosomal recessive disorder, characterized by chronic obstructive lung disease, pancreatic exocrine insufficiency, and elevated sweat chloride c...

DIAGNOSIS

HISTORY

  • Most common presenting respiratory symptoms: chronic cough, recurrent pneumonia, nasal polyps, chronic pansinusitis

  • Most common presenting GI symptoms:

    • Meconium ileus (15–20% of patient...

TREATMENT

MEDICATION

First Line

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Newborns should be seen in a CF center within 48 to 72 hours after a diagnosis has been established.

ALERT

In infants with positive newborn screen...

ADDITIONAL READING

  • Baumer JH. Evidence based guidelines for the performance of the sweat test for the investigation of cystic fibrosis in the UK. Arch Dis Child.  2003;88(12):1126–1127. [View Abs...

CODES

ICD9

  • 277.00 Cystic fibrosis without mention of meconium ileus

  • 277.02 Cystic fibrosis with pulmonary manifestations

  • 277.01 Cystic fibrosis with meconium ileus

  • 277.03 Cystic fibrosis with gastrointest...

FAQ

  • Q: Should relatives be tested?

  • A: All siblings should have a sweat test.

  • Q: How well will a child with CF do?

  • A: The course of the illness is variable. It is difficult to predict the course of diseas...

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