Abstract
Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare, fatal developmental lung disorder of neonates and infants. This review aims to address recent findings in the etiology and genetics of ACD/MPV and to raise awareness of this poorlyknowndisease, whichmayalso present as milder, unclassified forms. Successively discussed are what is known about the epidemiology, pathogenesis, pathophysiology, diagnostic indicators and approaches, genetic testing, treatment, and cases of delayed onset. The review concludes with suggestions for future directions to answer the many unknowns about this disorder.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 172-179 |
| Number of pages | 8 |
| Journal | American Journal of Respiratory and Critical Care Medicine |
| Volume | 184 |
| Issue number | 2 |
| DOIs | |
| State | Published - Jul 15 2011 |
| Externally published | Yes |
Keywords
- Alveolar air-blood barrier
- Comparative microarray analysis
- FOXF1 transcription factor gene
- Interstitial lung disease
- Persistent pulmonary hypertension of the newborn
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
- Critical Care and Intensive Care Medicine
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Cite this
Bishop, N. B., Stankiewicz, P., & Steinhorn, R. H. (2011). Alveolar capillary dysplasia. American Journal of Respiratory and Critical Care Medicine, 184(2), 172-179. https://doi.org/10.1164/rccm.201010-1697CI