A case of Alveolar capillary dysplasia with misalignment of pulmonary veins（ACD/MPV） with no extrapulmonary complications histologically diagnosed by pathological autopsy

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  • 病理解剖で組織診断した肺外合併症がないAlveolar capillary dysplasia with misalignment of pulmonary veins（ACD/MPV）の一例

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Abstract

<p> Alveolar capillary dysplasia with misalignment of the pulmonary veins（ACD/MPV）is a rare disorder characterized by abnormal development of pulmonary capillaries, and with severe persistent pulmonary hypertension of the newborn（PPHN）, and prolonged progressive respiratory impairment. It is most often suspected based on the presence of refractory PPHN and concomitant anomalies of the cardiovascular, gastrointestinal and urogenital systems, and is diagnosed by FOXF1 genetic testing and/or lung histological examination. We experienced a case of ACD/MPV, which was diagnosed by the characteristic findings of pathological autopsy after death, and was difficult to diagnose without extrapulmonary complications. Even in the absence of complications, it is one disease that should be suspected in cases of refractory PPHN of unknown cause, and appropriate genetic counseling and active genetic analysis should be considered while being alive.</p>

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