What is the underlying pathophysiology

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A 6 year-old boy was brought to the ER by his parents because of breathing difficulty and cough productive of purulent green mucus. He has had many recurrent respiratory infections and episodes of foul-smelling diarrhea since infancy with overall failure to thrive. On exam, chronically ill appearing child, small for his age, tachycardic, tachypneic, and lungs have bibasilar crackles to auscultation. Chest x-ray shows hyperinflation and patchy atelectasis. Lab shows high levels of sodium and chloride in the sweat test, sputum culture grows H. influenza and S. aureus. Stool has increased fat.

What is the relevant objective and subjective assessment data from the case study?

What does this data indicate? What conclusions can you make? Using Cantellani (2018) "ECFS best practice guidelines: the 2018 revision", what additional diagnostic testing is indicated? (See 4. Diagnosis on p. 158).

What is your preliminary diagnosis(es)?

What is the underlying pathophysiology and mode of inheritance that produces the clinical

manifestations in this case study? Please use your own words and do not "cut and paste" from a reference.

Based on the pathophysiology, what clinical course and complications could you anticipate?

What patient education and follow up is required for the case study patient?

Reference no: EM133544614

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