signs & symptoms of cystic fibrosis

Signs & Symptoms

(To get definitions of the medical jargon, hold your mouse over the word.)

  • Thick secretions and dehydration due to ionic imbalance.
  • Chronic airway infections by Staphylococcus aureus(1), Pseudomonas aeruginosa(2), and Pseudomonas cepacia,(3) possibly due to abnormal airway surface fluids and failure of lung defenses.
  • Dyspnea due to accumulation of thick secretions in bronchioles and alveoli.
  • Paroxysmal cough due to stimulation of the secretion-removal reflex.
  • Barrel chest, cyanosis, and clubbing of fingers and toes from chronic hypoxia.
  • Crackles on auscultation due to thick, airway-occluding secretions.
  • Wheezes hard on auscultation due to constricted airways.
  • Retention of bicarbonate and water due to the absence of the CFTR chloride channel in the pancreatic ductile epithelia; limits membrane function and leads to retention of pancreatic enzymes, chronic cholecystitis and cholelithiasis, and ultimate destruction of the pancreas.
  • Obstruction of the small and large intestines due to inhibited secretion of chloride and water and excessive absorption of liquid.
  • Biliary cirrhosis due to retention of biliary secretions.
  • Fatal shock and arrhythmias due to hyponatremia and hypochloremia from sodium lost in sweat.
  • Failure to thrive: poor weight gain, poor growth, distended abdomen, this extremities, and sallow skin with poor turgor due to malabsorption.
  • Clotting problems, retarded bone growth, and delayed sexual development due to deficiency of fat-soluble vitamins.
  • Rectal prolapse in infants and children due to malnutrition and wasting of perirectal supporting tissues.
  • Esophageal varices due to cirrhosis and portal hypertension.

  1. Staphylococcus aureus is more commonly known as MRSA, a type of bacteria that is resistant to certain antibiotics. These antibiotics include methicillin and other more common antibiotics such as oxacillin, penicillin and amoxicillin. Staph infections, including MRSA, occur most frequently among persons in hospitals and healthcare facilities (such as nursing homes and dialysis centers) who have weakened immune systems. (Courtesy of the CDC.)
  2. Pseudomonas aeruginosa is motile bacteria that grow as free living organisms in soil, river water, marshes, and coastal marine, habitats and as pathogens of plants and animals. P. aeruginosa are resistant to antibiotics and disinfectants and are responsible for many infections in humans. This species is the predominant cause of mortality in patients with cystic fibrosis. (1)
  3. Pseudomonas cepacia is one type of the ‘complex’ of bacteria known as Burkholderia cepacia, which can be found in soil and water. B. cepacia bacteria are often resistant to common antibiotics. B. cepacia poses little medical risk to healthy people. However, people who have certain health problems like weakened immune systems or chronic lung diseases, particularly cystic fibrosis (CF), may be more susceptible to infections with B. cepaciaB cepacia is a known cause of infections in hospitalized patients. (Courtesy of the CDC.)

  1. “Pseudomonas”   A Dictionary of Genetics. Robert C. King, William D. Stansfield and Pamela K. Mulligan. Oxford University Press, 2007. Oxford Reference Online. Oxford University Press.

Professional Guide to Pathophysiology. Philadelphia: Lippincott Williams & Wilkins, 2006. Print.

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One comment on “signs & symptoms of cystic fibrosis

  1. Pingback: Cystic Fibrosis Symptoms among Adults

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