RT Journal Article
SR Electronic
T1 Congenital acinar dysplasia. Familial cause of a fatal respiratory failure in a neonate
JF Saudi Medical Journal
JO Saudi Med J
FD Prince Sultan Military Medical City
SP 88
OP 90
VO 24
IS 1
A1 Al-Senan, Kefah A.
A1 Kattan, Abdulhakiem K.
A1 Al-Dayel, Fouad H.
YR 2003
UL http://smj.org.sa/content/24/1/88.abstract
AB Pulmonary hypoplasia is a rare cause of pulmonary insufficiency, and has a significant rate of morbidity and mortality among affected infants. In most cases, pulmonary hypoplasia is secondary to underlying abnormalities. These may include space occupying lesions, as in infants with congenital diaphragmatic hernia; malformation of chest wall resulting in a small thoracic cavity; severe and prolonged oligohydramnios; and neuromuscular disorders, which prevent normal fetal chest expansion. All lead to poor lung development. Primary pulmonary hypoplasia as a result of congenital acinar dysplasia is exceedingly rare and is diagnosed by exclusion of all known etiologies of secondary pulmonary hypoplasia.