Alveolar capillary dysplasia

Naomi B. Bishop, Pawel Stankiewicz, Robin H Steinhorn

Research output: Contribution to journalArticle

97 Citations (Scopus)

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 languageEnglish (US)
Pages (from-to)172-179
Number of pages8
JournalAmerican Journal of Respiratory and Critical Care Medicine
Volume184
Issue number2
DOIs
StatePublished - Jul 15 2011
Externally publishedYes

Fingerprint

Persistent Fetal Circulation Syndrome
Genetic Testing
Epidemiology
Newborn Infant
Lung
Alveolar capillary dysplasia
Therapeutics

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 journalArticle

Bishop, Naomi B. ; Stankiewicz, Pawel ; Steinhorn, Robin H. / Alveolar capillary dysplasia. In: American Journal of Respiratory and Critical Care Medicine. 2011 ; Vol. 184, No. 2. pp. 172-179.
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