期刊
PEDIATRIC PULMONOLOGY
卷 -, 期 -, 页码 -出版社
WILEY
DOI: 10.1002/ppul.26571
关键词
alveolar capillary dysplasia; bronchopulmonary dysplasia; FOXF1; histopathology; lung development; TMEM
We present the case of a preterm infant with severe bronchopulmonary dysplasia (sBPD) who exhibited histologic features consistent with alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) but lacked genetic confirmation. We also found that sBPD is associated with decreased levels of lung FOXF1 and TMEM100, suggesting potential shared mechanistic links between ACDMPV and sBPD involving impaired FOXF1 signaling.
We report the case of a preterm infant who died at 10 months of age with severe bronchopulmonary dysplasia (sBPD) with refractory pulmonary hypertension and respiratory failure who had striking histologic features compatible with the diagnosis of alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) but without genetic confirmation of the diagnosis. We further demonstrate dramatic reductions in lung FOXF1 and TMEM100 content in sBPD, suggesting common mechanistic links between ACDMPV and sBPD with impaired FOXF1 signaling.
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