Histologic features and decreased lung FOXF1 gene expression in severe bronchopulmonary dysplasia without a genetic diagnosis of alveolar capillary dysplasia.
Pediatr Pulmonol
; 58(10): 2746-2749, 2023 10.
Article
em En
| MEDLINE
| ID: mdl-37401868
ABSTRACT
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.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Síndrome da Persistência do Padrão de Circulação Fetal
/
Displasia Broncopulmonar
Tipo de estudo:
Diagnostic_studies
Limite:
Humans
/
Infant
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article