A 31-year-old man presents to the clinic for evaluation of worsening shortness of breath on exertion. He has severe kyphoscoliosis due to cerebral palsy. He reports no other symptoms of chest discomfort, fever, chills, cough, or sputum production. On physical examination, he is not in distress; he has a severe scoliosis to the left; and decreased air entry to that side. His right lung is clear, the JVP is at 3 cm, and heart sounds are normal. Pulmonary function tests are performed. Which of the following is the most likely abnormality to be seen on the pulmonary function tests?
Correct Answer: decreased TLC
Description: Bony deformities of the chest can lead to respiratory failure with raised PCO2 , as well as recurrent pulmonary infection. Most patients develop a restrictive lung disease pattern with decreased compliance, reduced TLC and VC. In addition, because of the reduced compliance, the resting position of the lung changes such that FRC is reduced. Finally, the net effect is an increase in the work of breathing. The pattern on pulmonary function testing is usually that of a restrictive pattern.
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