CONGENITAL MISALIGNMENT OF PULMONARY VESSELS - AN UNUSUAL SYNDROME-ASSOCIATED WITH PPHN
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摘要We report a case of misalignment of pulmonary vessels and review the clinical features of all 13 cases reported to date. All were term infants dying from severe persistent pulmonary hypertension of the newborn. We have identified a triad of features that will alert neonatal clinicians to the possibility of this diagnosis: association with other non-lethal congenital malformations; delayed onset of presentation (especially after 12 h); and severe hypoxaemia refractory to conventional therapy. We recommend that any autopsy on newborn infants include a specific search for misalignment of pulmonary vessels to outline the pathophysiology and clinical significance of this disorder.
著者NG PC, LEWINDON PJ, SIU YK, TO KF, WONG W
期刊名稱Acta Paediatrica
出版年份1995
月份3
日期1
卷號84
期次3
出版社SCANDINAVIAN UNIVERSITY PRESS
頁次349 - 353
國際標準期刊號0803-5253
語言英式英語
關鍵詞CONGENITAL ALVEOLAR CAPILLARY DYSPLASIA; CONGENITAL ANOMALIES; MISALIGNMENT OF PULMONARY VESSELS; PERSISTENT PULMONARY HYPERTENSION OF THE NEWBORN
Web of Science 學科類別Pediatrics; PEDIATRICS

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