Congenital acinar dysplasia: a lethal entity
Autops. Case Rep
; 9(4): e2019119, Oct.-Dec. 2019. ilus
Artículo
en Inglés
| LILACS
| ID: biblio-1024159
Biblioteca responsable:
BR26.7
ABSTRACT
Congenital acinar dysplasia is a lethal, developmental lung malformation resulting in neonatal respiratory insufficiency. This entity is characterized by pulmonary hypoplasia and arrest in the pseudoglandular stage of development, resulting in the absence of functional gas exchange. The etiology is unknown, but a relationship with the disruption of the TBX4-FGF10 pathway has been described. There are no definitive antenatal diagnostic tests. It is a diagnosis of exclusion from other diffuse embryologic lung abnormalities with identical clinical presentations that are, however, histopathologically distinct.
Texto completo:
Disponible
Colección:
Bases de datos internacionales
Base de datos:
LILACS
Asunto principal:
Insuficiencia Respiratoria
/
Pulmón
/
Enfermedades Pulmonares
Límite:
Femenino
/
Humanos
/
Recién nacido
Idioma:
Inglés
Revista:
Autops. Case Rep
Asunto de la revista:
Anatomia
/
Patologia Cl¡nica
/
Patologia Legal
Año:
2019
Tipo del documento:
Artículo
País de afiliación:
Estados Unidos
Institución/País de afiliación:
Florida International University/US
/
Mount Sinai Medical Cente/US