Congenital acinar dysplasia: a lethal entity
Autops. Case Rep
;
9(4): e2019119, Oct.-Dec. 2019. ilus
Article
in English
| LILACS
| ID: biblio-1024159
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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Respiratory Insufficiency
/
Lung
/
Lung Diseases
Limits:
Female
/
Humans
/
Infant, Newborn
Language:
English
Journal:
Autops. Case Rep
Journal subject:
Anatomia
/
Patologia Cl¡nica
/
Patologia Legal
Year:
2019
Type:
Article
Affiliation country:
United States
Institution/Affiliation country:
Florida International University/US
/
Mount Sinai Medical Cente/US
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