Which complications can affect the prognosis of cystic fibrosis (CF)?

Updated: Oct 22, 2019
  • Author: Girish D Sharma, MD, FCCP, FAAP; Chief Editor: Kenan Haver, MD  more...
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Answer

The clinical presentation, age at diagnosis, severity of symptoms, and rate of disease progression in the organs involved widely vary. Sweat abnormalities may result in heat stroke and salt depletion, especially in infants. Mucocele and mucopyocele associated with chronic sinusitis and nasal polyps can cause erosion of the sinus wall, resulting in CNS complications from the space-occupying effect of mucopyocele or from associated complications. [22]

GI tract complications include pancreatic involvement. Pancreatic tissue damage leads to diabetes mellitus in 8-12% of patients older than 25 years. Excessive administration of exogenous pancreatic enzymes can result in fibrosing colonopathy. Intestinal complications range from meconium ileus with associated complications during the neonatal period (12% of neonates with cystic fibrosis) to distal intestinal obstruction syndrome, rectal prolapse, peptic ulcer, and gastroesophageal reflux.

Liver involvement may result in a fatty liver (30-60% of patients), focal biliary cirrhosis, multinodular biliary cirrhosis, and associated portal hypertension. Portal hypertension occasionally causes death through esophageal varices. The prevalence of cholecystitis and gallstones is higher in patients with cystic fibrosis than in other individuals.

Delayed puberty and reduced fertility are other complications; most males are azoospermic because of agenesis of the vas deferens. Female fertility is probably only mildly impaired, and many successful pregnancies have been reported in women with cystic fibrosis.

Severity of pulmonary disease determines prognosis and ultimate outcome. Pulmonary involvement is progressive: beginning as bronchitis, bronchiolitis, and then bronchiectasis, pulmonary involvement leads to cor pulmonale and end-stage lung disease. Cause of death is generally respiratory failure and cor pulmonale.


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