Cystic fibrosis

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

Gene

Clinical features

Mnemonic CF PANCREAS:[2]

  • 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:

Gastrointestinal tract:

Genitourinary tract:

See also

References

  1. Online 'Mendelian Inheritance in Man' (OMIM) 219700
  2. URL: http://www.valuemd.com/pediatrics_mnemonics.php. Accessed on: 14 March 2012.
  3. Lloyd-Still, JD. (Apr 1994). "Crohn's disease and cystic fibrosis.". Dig Dis Sci 39 (4): 880-5. PMID 8149854.
  4. McCallum, TJ.; Milunsky, JM.; Cunningham, DL.; Harris, DH.; Maher, TA.; Oates, RD. (Oct 2000). "Fertility in men with cystic fibrosis: an update on current surgical practices and outcomes.". Chest 118 (4): 1059-62. PMID 11035677.