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Alveolar Capillary Dysplasia Associated with Omphalocoele

机译:肺泡扩张相关的肺泡毛细血管发育不良

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Alveolar capillary dysplasia (ACD) is a rare and universally fatal form of persistent pulmonary hypertension of the newborn (PPHN). We report a case of a newborn male baby with a moderate sized omphalocoele who developed respiratory distress 10 hours after birth. He deteriorated and succumbed shortly afterwards despite maximal ventilatory support and inhaled nitric oxide. ACD was diagnosed at postmortem examination, and the association of ACD with omphalocoele and gut malrotation has not been reported. We suggest including ACD in the differential diagnosis of refractory PPHN in a newborn even with the presence of other risk factors for respiratory failure.
机译:肺泡毛细血管发育不良(ACD)是新生儿持续性肺动脉高压(PPHN)的一种罕见且普遍致命的形式。我们报告了一例新生儿,男婴中度大小,在出生后10小时出现呼吸窘迫。尽管有最大程度的通气支持和吸入一氧化氮,但他不久后病情恶化并屈服。在死后检查中诊断出ACD,尚未报道ACD与卵泡腔和肠道异常旋转的关系。我们建议将ACD包括在新生儿难治性PPHN的鉴别诊断中,即使存在呼吸衰竭的其他危险因素。

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