Cystic fibrosis

Cystic fibrosis, abbreviated CF, a genetic disease that predominantly affects the lungs. It is inherited autosomal recessive.

Gene

• CFTR gene.

Clinical features

Mnemonic CF PANCREAS:

• Chronic cough.
• Failure to thrive.
• Pancreatic insufficiency, e.g. steatorrhea.
• Alkalosis.
• Nasal polyps & neonatal intestinal obstruction (meconium ileus).
• Clubbing & chest x-ray findings.
• Rectal prolapse.
• Electrolyte elevations in sweat.
• Absence of vas deferens.
• Sputum with Pseudomonas.

Trimmed version

• PAncreatic insufficiency, e.g. steatorrhea.
• Nasal polyps & neonatal intestinal obstruction (meconium ileus).
• Clubbing.
• REctal prolapse.
• Absence of vas deferens.
• Sputum with Pseudomonas.

Associated pathology

Pulmonary:

• Bronchiectasis.
• Fibrosis - secondary to infections.

Gastrointestinal tract:

• Pancreatic insufficiency.
• Meconium ileus.
• Meconium peritonitis.

Genitourinary tract:

• Infertility - bilateral absence vas deferens.

See also

• Lung.
• Pediatric pathology.