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KMID : 0391519960040010185
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Journal of the Korean Child Neurology Society
1996 Volume.4 No. 1 p.185 ~ p.192
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A case of Congenital Hypoventilation Syndrome
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Abstract
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Congenital central hypoventilation syndrome (CCHS) is a rare disorder of chronic respiratory failure in infancy and childhood. It is unknown ctiology but considered as brain stem lesion which shows decreased sensitivity of central chemoreceptor
on
brain
stem or disorder of neural of signal from central and peripheral chemoreceptor. We experienced 4 month-old girl with CCHS who had been brought to our hospital due to respiratory failure and convulsion. She had hypopnea, bradypnea hypercapnea, and
respiratory acidosis when sleeping. But there were no such a symptom or sign when crying. And there were no evidence of primary abnormality on respiratory, cardiovascular and neuromuscular system. Brain MRI showed no abnormal findings on brain
parenchyme, especially on brain stem. She was diagnosed as congenital central hypoventilation syndrome and has been managed by intermittent positive pressure ventilation via tracheosotmy site.
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KEYWORD
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