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先天性中枢性低通气综合征:最新进展

Congenital central hypoventilation syndrome: an update.

作者信息

Gozal D

机构信息

Constance S. Kaufman Pediatric Pulmonary Research Laboratory, Department of Pediatrics, Tulane University School of Medicine, New Orleans, Louisiana 70112, USA.

出版信息

Pediatr Pulmonol. 1998 Oct;26(4):273-82. doi: 10.1002/(sici)1099-0496(199810)26:4<273::aid-ppul7>3.0.co;2-c.

DOI:10.1002/(sici)1099-0496(199810)26:4<273::aid-ppul7>3.0.co;2-c
PMID:9811078
Abstract

Awareness of the existence of CCHS has led to increasingly frequent reports of such patients from all over the world. However, the exact pathophysiologic mechanisms underlying the clinical manifestations of this congenital disease entity remain unknown. For the respiratory physiologist, CCHS can be viewed as an experiment of nature that provides an important and unique window into central cardiorespiratory regulation. For the pediatrician, CCHS children represent an unique clinical challenge in coordinating the diagnostic and therapeutic procedures required to enhance the patients' quality of life.

摘要

对先天性中枢性低通气综合征(CCHS)存在的认识,已导致来自世界各地关于此类患者的报告日益频繁。然而,这种先天性疾病实体临床表现背后的确切病理生理机制仍不清楚。对于呼吸生理学家而言,CCHS可被视为一种自然实验,它为深入了解中枢心肺调节提供了一个重要且独特的窗口。对于儿科医生来说,CCHS患儿在协调提高患者生活质量所需的诊断和治疗程序方面,代表着一项独特的临床挑战。

相似文献

1
Congenital central hypoventilation syndrome: an update.先天性中枢性低通气综合征:最新进展
Pediatr Pulmonol. 1998 Oct;26(4):273-82. doi: 10.1002/(sici)1099-0496(199810)26:4<273::aid-ppul7>3.0.co;2-c.
2
Pediatric hypoventilation syndromes.小儿通气不足综合征
Curr Opin Pulm Med. 1997 Nov;3(6):445-8. doi: 10.1097/00063198-199711000-00011.
3
Congenital central alveolar hypoventilation syndrome in six infants.六例婴儿先天性中枢性肺泡低通气综合征
Pediatrics. 1982 Nov;70(5):684-94.
4
Heart rate variability in congenital central hypoventilation syndrome.先天性中枢性低通气综合征中的心率变异性
Pediatr Res. 1992 Mar;31(3):291-6. doi: 10.1203/00006450-199203000-00020.
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Sleep apnea studies in an infant with congenital primary hypoventilation ("Ondine's curse").
Int J Neurosci. 1981;13(2-3):155-62. doi: 10.3109/00207458109043315.
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Hypercapneic arousal responses in children with congenital central hypoventilation syndrome.先天性中枢性低通气综合征患儿的高碳酸血症唤醒反应。
Pediatrics. 1991 Nov;88(5):993-8.
7
[Continuous ambulatory monitoring in quality control of home therapy of congenital central hypoventilation syndrome (CCHS)].
Wien Med Wochenschr. 1996;146(13-14):323-4.
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Congenital central hypoventilation syndrome: cardiorespiratory responses to moderate exercise, simulating daily activity.先天性中枢性低通气综合征:对模拟日常活动的适度运动的心肺反应。
Pediatr Pulmonol. 1995 Aug;20(2):89-93. doi: 10.1002/ppul.1950200207.
9
[Home mechanical ventilation with nasal intermittent positive pressure ventilation for a boy with congenital central hypoventilation syndrome].
No To Hattatsu. 1998 May;30(3):250-4.
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[Congenital central hypoventilation--treated with nocturnal biphasic intermittent respiration via nasal mask].[先天性中枢性低通气——经鼻面罩夜间双相间歇性通气治疗]
Ugeskr Laeger. 1995 Mar 20;157(12):1683-4.

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