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Alveolar capillary dysplasia

Naomi B Bishop1, Pawel Stankiewicz, Robin H Steinhorn

  • 1Pediatric Critical Care Medicine, Weill Cornell Medical College, New York, NY 10065, USA. bishopn@med.cornell.edu

American Journal of Respiratory and Critical Care Medicine
|April 8, 2011
PubMed

Insights

Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare, fatal infant lung disorder. This review covers recent findings on its causes, genetics, and diagnosis, aiming to improve awareness and future research.

Area of Science:

  • Pediatric Pulmonology
  • Developmental Biology
  • Medical Genetics

Background:

  • Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare, fatal neonatal and infant lung disorder.
  • The disease is poorly understood and may present with milder, unclassified forms.
  • Limited awareness and research hinder diagnosis and treatment.

Purpose of the Study:

  • To review recent findings on the etiology and genetics of ACD/MPV.
  • To raise awareness of this rare developmental lung disorder.
  • To consolidate current knowledge on epidemiology, pathogenesis, diagnosis, and treatment.

Main Methods:

  • Comprehensive literature review of recent studies on ACD/MPV.
  • Synthesis of information on genetic testing, diagnostic approaches, and pathophysiology.
  • Discussion of treatment strategies and delayed onset cases.

Main Results:

  • Recent advances in understanding the genetic basis and pathogenesis of ACD/MPV.
  • Identification of diagnostic indicators and emerging diagnostic approaches.
  • Recognition of milder and delayed-onset presentations of the disorder.

Conclusions:

  • ACD/MPV remains a significant challenge due to its rarity and severity.
  • Further research is crucial to elucidate the many unknowns surrounding ACD/MPV.
  • Increased awareness and improved diagnostic tools are needed for better patient outcomes.

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