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congenital acinar dysplasia

Wednesday 3 December 2003

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