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Published on: May 31, 2024
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
Summary
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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