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原发性纤毛运动障碍:新生儿呼吸窘迫的一个原因。

Primary ciliary dyskinesia: a cause of neonatal respiratory distress.

作者信息

Bhutta Z A

机构信息

Department of Neonatal Services, Aga Khan University, Karachi.

出版信息

J Pak Med Assoc. 1995 Mar;45(3):70-3.

PMID:7596016
Abstract

Primary ciliary dyskinesia (PCD) is a rare disorder associated with chronic respiratory problems and even more infrequently as a cause of neonatal respiratory distress. Of consecutive 12 children seen with a diagnosis of PCD, the disorder presented within the neonatal period in 11, with a positive family history in 50%. The diagnosis was delayed in several cases, despite suggestive radiological findings. The data highlights the importance of recognizing PCD in newborns presenting with early respiratory distress and isolated dextrocardia.

摘要

原发性纤毛运动障碍(PCD)是一种罕见的疾病,与慢性呼吸问题相关,甚至更罕见地作为新生儿呼吸窘迫的原因。在连续12例被诊断为PCD的儿童中,11例在新生儿期出现该疾病,50%有阳性家族史。尽管有提示性的放射学表现,但在几例病例中诊断被延迟。这些数据突出了在出现早期呼吸窘迫和孤立性右位心的新生儿中识别PCD的重要性。

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