Topic
Alveolar capillary dysplasia
About: Alveolar capillary dysplasia is a research topic. Over the lifetime, 219 publications have been published within this topic receiving 5569 citations. The topic is also known as: Alveolar capillary dysplasia with misalignment of pulmonary veins.
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TL;DR: The generation of human iPSC lines from two ACD/MPV patients, each carrying a different heterozygous FoxF1 mutation, enables disease modeling for further research on the effect of FOXF1 variants in vitro.
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01 Jan 2012
TL;DR: Rare lung diseases include a wide spectrum of conditions and are important causes of morbidity and mortality in newborn babies and infants, and the management and care of newborns affected by these diseases can require considerable time and resources within a tertiary care center.
Abstract: Rare lung diseases include a wide spectrum of conditions and are important causes of morbidity and mortality in newborn babies and infants. Despite the low incidence of these conditions, neonatologists are likely to encounter these disorders because they present with respiratory distress. For intensive care staff, radiologists, pathologists, and surgeons, the management and care of newborns affected by these diseases can require considerable time and resources within a tertiary care center. Embryological, pathological, surgical, antenatal and pediatric classifications for rare lung diseases have been reported [1, 2, 3]. The causes of rare respiratory diseases in neonates may be classified as: (a) Bronchial ultrastructural anomalies (e.g., primary ciliary dyskinesia [Kartagener’s syndrome]), parenchymal molecular defects (e.g., congenital surfactant protein abnormalities), vascular malformations (e.g., congenital pulmonary lymphangiectasia and alveolar capillary dysplasia); (b) Developmental parenchymal abnormalities (e.g., pulmonary hypoplasia and cystic lung diseases [Table 66.1]).
01 May 2019
TL;DR: Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a disorder affecting the development of the lungs and their blood vessels.
Abstract: Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a disorder affecting the development of the lungs and their blood vessels. The disorder affects the millions of small air sacs (alveoli) in the lungs and the tiny blood vessels (capillaries) in the alveoli. It is through these alveolar capillaries that inhaled oxygen enters the bloodstream for distribution throughout the body and carbon dioxide leaves the bloodstream to be exhaled.
TL;DR: The result indicated that PVD was one of the important reasons for respiratory failure in these neonates, including pulmonary hypertensive vascular remodeling in 3 cases and alveolar capillary dysplasia in 2 cases.
Abstract: OBJECTIVE We reviewed the data of 38 neonates who died of respiratory failure. Paraffin sections of the autopsy lung samples were examined with HE staining or immunolabeling for CD34, CD68 and CK to observe the development of the pulmonary vessels and detect potential pulmonary vascular diseases (PVDs). Five cases were identified to have PVDs, including pulmonary hypertensive vascular remodeling in 3 cases and alveolar capillary dysplasia in 2 cases. The result indicated that PVD was one of the important reasons for respiratory failure in these neonates.