Alveolar capillary dysplasia

Naomi B. Bishop; Pawel Stankiewicz; Robin H Steinhorn

doi:10.1164/rccm.201010-1697CI

Alveolar capillary dysplasia

Naomi B. Bishop, Pawel Stankiewicz, Robin H Steinhorn

Research output: Contribution to journal › Article

109 Scopus citations

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	https://doi.org/10.1164/rccm.201010-1697CI
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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title = "Alveolar capillary dysplasia",

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.",

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AU - Stankiewicz, Pawel

AU - Steinhorn, Robin H

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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

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