View in EDS

Nemaline Myopathy as a Cause of Sleep Hypoventiontilation.

Saved in:
Bibliographic Details
Title: Nemaline Myopathy as a Cause of Sleep Hypoventiontilation.
Authors: Maayan, Ch., Springer, C., Armon, Y., Bar-Yishay, E., Shapira, Y., Godfrey, S.
Source: Pediatrics. Mar86, Vol. 77 Issue 3, p390. 6p.
Subjects: Muscle diseases, Respiratory insufficiency in children, Sleep apnea syndromes, Artificial respiration, Familial diseases
Abstract: Abstract. Two siblings, a 14.5-year-old boy and his 11.5-year-old sister, with congenital nemaline myopathy presented with severe respiratory failure and, in the case of the older patient, with cor pulmonale and systemic hypertension. The children were treated initially by continuous mechanical ventilation, but after a few weeks they only required ventilation at night. At the start of treatment, both were found to have a decreased ventilatory response to C0[sub 2] which apparently improved during 4 to 5 years of follow-up treatment. It has not been possible to wean them from nocturnal mechanical ventilation, but during the daytime they attend school and function almost normally. It is postulated that respiratory failure in nemaline myopathy may not be related to the severity of the muscle weakness but may result from a disturbance of the feedback required for normal control of breathing. Pediatrics 1986;77:390-395; nemaline myopathy, respiratory failure, sleep apnea, breathing control, lung function. [ABSTRACT FROM AUTHOR]
Copyright of Pediatrics is the property of American Academy of Pediatrics and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Database: Psychology and Behavioral Sciences Collection
Description
Abstract:Abstract. Two siblings, a 14.5-year-old boy and his 11.5-year-old sister, with congenital nemaline myopathy presented with severe respiratory failure and, in the case of the older patient, with cor pulmonale and systemic hypertension. The children were treated initially by continuous mechanical ventilation, but after a few weeks they only required ventilation at night. At the start of treatment, both were found to have a decreased ventilatory response to C0[sub 2] which apparently improved during 4 to 5 years of follow-up treatment. It has not been possible to wean them from nocturnal mechanical ventilation, but during the daytime they attend school and function almost normally. It is postulated that respiratory failure in nemaline myopathy may not be related to the severity of the muscle weakness but may result from a disturbance of the feedback required for normal control of breathing. Pediatrics 1986;77:390-395; nemaline myopathy, respiratory failure, sleep apnea, breathing control, lung function. [ABSTRACT FROM AUTHOR]
ISSN:00314005
DOI:10.1542/peds.77.3.390