What are GI symptoms of cystic fibrosis (CF)?

Updated: Jun 26, 2019
  • Author: Girish D Sharma, MD, FCCP, FAAP; Chief Editor: Kenan Haver, MD  more...
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Answer

Meconium ileus occurs in 7-10% of patients with cystic fibrosis. Patients with simple meconium ileus usually present with abdominal distension at birth, eventually progressing to failure to pass meconium, bilious vomiting, and progressive abdominal distension.

Patients with complicated meconium ileus present more dramatically at birth with severe abdominal distention, sometimes accompanied by abdominal wall erythema and edema. Abdominal distention may be severe enough to cause respiratory distress.

Other GI manifestations in neonates include intestinal obstruction at birth and various surgical findings (eg, volvulus, intestinal atresia, perforation, meconium peritonitis). Less commonly, passage of meconium may be delayed (>24-48 hours after birth) or cholestatic jaundice may be prolonged.

Infants and children present with increased frequency of stools, which suggests malabsorption (ie, fat or oil drops in stools), failure to thrive, intussusception (ileocecal), or rectal prolapse.

Patients with pancreatic insufficiency have fat-soluble vitamin deficiency and malabsorption of fats, proteins, and carbohydrates (however, malabsorption of carbohydrates is not as severe as that of fats and proteins). Patients present with failure to thrive (despite an adequate appetite), flatulence or foul-smelling flatus, recurrent abdominal pain, and abdominal distention.

Malabsorption results in steatorrhea, characterized by frequent, poorly formed, large, bulky, foul-smelling, greasy stools that float in water. Cloth diapers, if used, are difficult to clean. Alternatively, some patients have anorexia without obvious steatorrhea.

Patients may present with a history of jaundice or gastrointestinal tract bleeding as a result of hepatobiliary involvement.


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