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Life threatening hypoventilation in kyphoscoliosis: successful treatment with a molded body brace-ventilator.

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TLDR
S sustained successful treatment of end-stage hypoventilation in a 37-yr-old patient with kyphoscoliosis caused by childhood poliomyelitis is reported, and he now has normal daytime arterial blood gas values 20 months later.
Abstract
We report sustained successful treatment of end-stage hypoventilation in a 37-yr-old patient with kyphoscoliosis caused by childhood poliomyelitis. The patient presented with progressive ventilatory failure, pure restrictive lung function, and no concurrent illness. He first responded to respiratory stimulation with medroxyprogesterone acetate and aminophylline. Because of skeletal deformity and the need for a chronic brace he was then fitted with a customized polyethylene foam body brace with an anterior free space and connecting hole for a Monaghan ventilator. No longer taking any medication, and with only nocturnal assisted ventilation, he now has normal daytime arterial blood gas values 20 months later. This approach may be useful in other patients with severe kyphoscoliosis.

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Journal ArticleDOI

Respiratory muscles in disease.

TL;DR: Although the respiratory muscles are placed at a mechanical disadvantage in patients with obstructive lung disease, there is little clinical evidence to suggest that respiratory muscle dysfunction plays a primary role in the development of ventilatory failure.
Journal ArticleDOI

Inspiratory muscle function in patients with severe kyphoscoliosis.

TL;DR: Impaired inspiratory muscle function is related to the development of ventilatory failure in kyphoscoliosis, and the degree of spinal deformity is not found.
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The effects of one year of nocturnal cuirass-assisted ventilation in chest wall disease

TL;DR: Nocturnal cuirass-assisted ventilation has a role in long-term management of patients with neuromuscular and skeletal chest wall disorders and a randomized comparison with nasal intermittent positive pressure ventilation is now indicated.
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Long term non-invasive domiciliary assisted ventilation for respiratory failure following thoracoplasty.

TL;DR: These results show that, even when ventilatory failure has developed, the prognosis with non-invasive assisted ventilation is good and the physiological abnormalities can be partially reversed.
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Assisted ventilation using cuirass respirators

TL;DR: The effects of cuirass-assisted ventilation have been studied in 25 subjects with chest wall disease and jacket-type respirators can produce larger tidal volumes than thecuirass at the same peak negative pressure, but are associated with greater air leakage.
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