congenital acinar dysplasia
Congenital acinar dysplasia is a rare cause of death in the first few hours of life, due to an absence of alveoli.
Synopsis
slightly small lungs with increased lobular markings
pulmonary hypoplasia
deranged air spaces lined by ciliated bronchial epithelium without development of alveoli
increased amounts of intervening fibrous tissue
Associations
right aortic arch
bilateral thinned renal cortices
major renal malformations (#15174801#)
See also
congenital pumonary airways malformations (CPAMs)
acinar dyplasias
References
Gillespie LM, Fenton AC, Wright C.Acinar dysplasia: a rare cause of neonatal respiratory failure.Acta Paediatr. 2004 May;93(5):712-3. Review. PMID: #15174801#
Al-Senan KA, Kattan AK, Al-Dayel FH. Congenital acinar dysplasia. Familial cause of a fatal respiratory failure in a neonate. Saudi Med J. 2003 Jan;24(1):88-90. PMID: #12590284#
Moerman P, Vanhole C, Devlieger H, Fryns JP. Severe primary pulmonary hypoplasia ("acinar dysplasia") in sibs: a genetically determined mesodermal defect? J Med Genet. 1998 Nov;35(11):964-5. PMID: #9832050#
Davidson LA, Batman P, Fagan DG. Congenital acinar dysplasia: a rare cause of pulmonary hypoplasia. Histopathology. 1998 Jan;32(1):57-9. PMID: #9522217#
Rutledge JC, Jensen P. Acinar dysplasia: a new form of pulmonary maldevelopment. Hum Pathol. 1986 Dec;17(12):1290-3. PMID: #3793091#