✦ LIBER ✦
Acid maltase deficiency: treatment of respiratory insufficiency with cuirass respirator
✍ Scribed by A. E. J. Jager; A. F. Meinesz
- Book ID
- 104717911
- Publisher
- Springer
- Year
- 1983
- Tongue
- English
- Weight
- 364 KB
- Volume
- 230
- Category
- Article
- ISSN
- 0340-5354
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✦ Synopsis
Three patients with the late-onset form of acid maltase deficiency showed a gradual weakening of proximal limb and trunk muscles leading to severe respiratory insufficiency. Considerable deterioration of pulmonary function occurred owing to a vicious cycle of hypoventilation and exhaustion. Treatment by nocturnal cuirass ventilation with tailor-made shells was successful. The muscle weakness still progressed very slowly but there were no more respiratory problems.