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Page 1 / Case 09.03

PRESENTING FELLOW

Dr. Steve Ponde
Respiratory Fellow, Hospital for Sick Children
Toronto, Ontario, Canada

THE CASE

Exacerbations in a girl with cystic fibrosis.

HISTORY

Initial Presentation

ETV is a 14-year-old girl with moderately severe pulmonary manifestations of cystic fibrosis. She is Canadian born of El Salvadorian parents and an older sibling had died of CF related lung complications at the age of 3 years in El Salvador.

She has pancreatic insufficiency but no CF related glucose intolerance or insulin dependent diabetes mellitus. ETV's usual CF treatment consists of pancreatic enzyme replacements, vitamins, an aerosol regime (budesonide, salbutamol and tobramycin) together with regular chest physiotherapy. Compliance with treatment and follow up is excellent and she has no major adverse psychosocial factors.

ETV has grown staph aureus, and Aspergillus fumigatus from her sputum in the last year but no Pseudomonas aeruginosa. During the last 10 months, ETV's clinical status has worsened. She has had more frequent pulmonary exacerbations and has been hospitalized 6 times in this period. She has more cough, sputum production without haemoptysis, fevers, malaise, loss of appetite, poor weight gain and is missing more school. Her pulmonary function has deteriorated. Her FEV1 has dropped from a baseline of 61% in May 2002 to 47% in April 2003.She has no significant bronchodilator response and has only transient improvement following her hospitalizations.

WHAT ARE THE INFECTIOUS CAUSES OF DETERIORATION OF PULMONARY STATUS IN CYSTIC FIBROSIS?


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