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无外侧弓状核婴儿的原发性肺泡低通气(翁丁氏诅咒综合征)。病例报告。

Primary alveolar hypoventilation (Ondine's curse syndrome) in an infant without external arcuate nucleus. Case report.

作者信息

Folgering H, Kuyper F, Kille J F

出版信息

Bull Eur Physiopathol Respir. 1979 Jul-Aug;15(4):659-65.

PMID:497494
Abstract

A five month old boy suffered from primary alveolar hypoventilation, with attacks of apnea during sleep (Ondine's curse syndrome). The ventilation did not increase when PACO2 was increased. However, it decreased during hyperoxia. This indicates a strong peripheral chemoreceptor drive and a dysfunction of the central chemoreception. The infant died from circulatory failure following an aspiration pneumonia. Autopsy revealed the absence of the external arcuate nucleus, which may be involved in the central chemocreception.

摘要

一名五个月大的男婴患有原发性肺泡通气不足,睡眠期间出现呼吸暂停发作(翁丁氏诅咒综合征)。当动脉血二氧化碳分压(PACO2)升高时,通气量并未增加。然而,在高氧期间通气量却减少。这表明外周化学感受器驱动强烈而中枢化学感受功能障碍。该婴儿因吸入性肺炎后循环衰竭死亡。尸检发现外侧弓状核缺失,其可能与中枢化学感受有关。

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Primary alveolar hypoventilation (Ondine's curse syndrome) in an infant without external arcuate nucleus. Case report.无外侧弓状核婴儿的原发性肺泡低通气(翁丁氏诅咒综合征)。病例报告。
Bull Eur Physiopathol Respir. 1979 Jul-Aug;15(4):659-65.
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[A case report of central alveolar hypoventilation syndrome (Ondine's curse)].[中枢性肺泡低通气综合征(翁丁氏诅咒)一例报告]
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