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先天性肺静脉排列异常合并肺泡毛细血管发育不良导致持续性新生儿肺动脉高压:两例患病同胞报告

Congenital misalignment of pulmonary veins with alveolar capillary dysplasia causing persistent neonatal pulmonary hypertension: report of two affected siblings.

作者信息

Gutierrez C, Rodriguez A, Palenzuela S, Forteza C, Rossello J L

机构信息

Pediatric Pathology Laboratory, Hospital Pereira Rossell, Boulevard Artigas 1550, Casilla de Correo 16059, Montevideo, Uruguay,

出版信息

Pediatr Dev Pathol. 2000 May-Jun;3(3):271-6. doi: 10.1007/s100249910035.

Abstract

Misalignment of pulmonary vessels with alveolar capillary dysplasia is a rare cause of persistent pulmonary hypertension of the newborn. Most of the reported cases have been sporadic. We present two consecutive affected siblings with this disorder. This is the fifth reported family occurrence of this condition. In addition to the pulmonary abnormality, one of our cases had duodenal atresia.

摘要

肺血管与肺泡毛细血管发育异常的错位是新生儿持续性肺动脉高压的罕见原因。大多数报道的病例为散发性。我们报告了两名患有这种疾病的连续患病的兄弟姐妹。这是该疾病第五次报道的家族性发病情况。除了肺部异常外,我们的一名病例还患有十二指肠闭锁。

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