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 |
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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
Cite this
Alveolar capillary dysplasia. / Bishop, Naomi B.; Stankiewicz, Pawel; Steinhorn, Robin H.
In: American Journal of Respiratory and Critical Care Medicine, Vol. 184, No. 2, 15.07.2011, p. 172-179.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Alveolar capillary dysplasia
AU - Bishop, Naomi B.
AU - Stankiewicz, Pawel
AU - Steinhorn, Robin H
PY - 2011/7/15
Y1 - 2011/7/15
N2 - 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.
AB - 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.
KW - Alveolar air-blood barrier
KW - Comparative microarray analysis
KW - FOXF1 transcription factor gene
KW - Interstitial lung disease
KW - Persistent pulmonary hypertension of the newborn
UR - http://www.scopus.com/inward/record.url?scp=80051560969&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=80051560969&partnerID=8YFLogxK
U2 - 10.1164/rccm.201010-1697CI
DO - 10.1164/rccm.201010-1697CI
M3 - Article
C2 - 21471096
AN - SCOPUS:80051560969
VL - 184
SP - 172
EP - 179
JO - American Journal of Respiratory and Critical Care Medicine
JF - American Journal of Respiratory and Critical Care Medicine
SN - 1073-449X
IS - 2
ER -