Which of the following restrictive thoracic diseases responds least favorably to NPPV?

A. amyotrophic lateral sclerosis
B. kyphoscoliosis
C. postpolio syndrome
D. myopathies

ANS: A (page 372)
Cognitive Level: Recall
A. Correct response: Long-term follow-up studies on several hundred patients using NPPV for 3 to 5 years have demonstrated favorable results among patients with postpolio syndrome, various myopathies, and kyphoscoliosis. Survival among patients with amyotrophic lateral sclerosis (ALS) was less favorable because the ALS (underlying disease) progresses more rapidly than with the other pathologies.
B. Incorrect response: See explanation A.
C. Incorrect response: See explanation A.
D. Incorrect response: See explanation A.

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