Generation of three iPSC lines from two patients with heterozygous FOXF1 mutations associated to Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins.
Sign Up to like & getrecommendations! Published in 2020 at "Stem cell research"
DOI: 10.1016/j.scr.2020.101745
Abstract: Diagnosing Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins (ACD/MPV) based on a genetic alteration in the FOXF1 gene, is complicated by the poor understanding of the causal relation between FOXF1 variants and the… read more here.
Keywords: misalignment pulmonary; pulmonary veins; dysplasia misalignment; alveolar capillary ... See more keywords
Two cases of different genetic variants of alveolar capillary dysplasia associated with left-sided obstructive CHDs
Sign Up to like & getrecommendations! Published in 2021 at "Cardiology in the Young"
DOI: 10.1017/s1047951121000676
Abstract: Abstract Alveolar capillary dysplasia with misalignment of the pulmonary veins is an uncommon disorder that affects the lung vasculature development in the neonatal period and leads to pulmonary hypertension. We describe two patients with alveolar… read more here.
Keywords: associated left; alveolar capillary; capillary dysplasia; left sided ... See more keywords
Using a Novel Microfabricated Model of the Alveolar-Capillary Barrier to Investigate the Effect of Matrix Structure on Atelectrauma
Sign Up to like & getrecommendations! Published in 2017 at "Scientific Reports"
DOI: 10.1038/s41598-017-12044-9
Abstract: The alveolar-capillary barrier is composed of epithelial and endothelial cells interacting across a fibrous extracelluar matrix (ECM). Although remodeling of the ECM occurs during several lung disorders, it is not known how fiber structure and… read more here.
Keywords: topography; barrier; alveolar capillary; matrix ... See more keywords
Gene transfer of MRCKα rescues lipopolysaccharide-induced acute lung injury by restoring alveolar capillary barrier function
Sign Up to like & getrecommendations! Published in 2021 at "Scientific Reports"
DOI: 10.1038/s41598-021-99897-3
Abstract: Acute Lung Injury/Acute Respiratory Distress Syndrome (ALI/ARDS) is characterized by alveolar edema accumulation with reduced alveolar fluid clearance (AFC), alveolar-capillary barrier disruption, and substantial inflammation, all leading to acute respiratory failure. Enhancing AFC has long… read more here.
Keywords: barrier; acute lung; alveolar capillary; gene ... See more keywords
Neonatal Diagnosis of Alveolar Capillary Dysplasia via Rapid Genomic Sequencing: A New Gold Standard?
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DOI: 10.1159/000529439
Abstract: Classic alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a rare congenital lung disorder presenting in the early neonatal period with refractory hypoxemic respiratory failure and pulmonary hypertension. No curative treatment is currently… read more here.
Keywords: capillary dysplasia; diagnosis; via rapid; genomic sequencing ... See more keywords
Novel FOXF1-Stabilizing Compound TanFe Stimulates Lung Angiogenesis in Alveolar Capillary Dysplasia.
Sign Up to like & getrecommendations! Published in 2022 at "American journal of respiratory and critical care medicine"
DOI: 10.1164/rccm.202207-1332oc
Abstract: RATIONALE Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins (ACDMPV) is linked to heterozygous mutations in the FOXF1 gene, a key transcriptional regulator of pulmonary vascular development. There are no effective treatments for ACDMPV other… read more here.
Keywords: compound; tanfe; alveolar capillary; foxf1 ... See more keywords
Intractable Pulmonary Hypertension in a Late Preterm Neonate: Alveolar Capillary Dysplasia, a Lethal Disease
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DOI: 10.1542/peds.142.1_meetingabstract.147
Abstract: Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins (ACDMPV) is a rare disorder, with only approximately 200 cases reported to date. The most common presentation of ACDMPV is hypoxic respiratory failure, a condition commonly… read more here.
Keywords: pulmonary hypertension; alveolar capillary; capillary dysplasia; late preterm ... See more keywords
Yu-Ping-Feng Formula Ameliorates Alveolar-Capillary Barrier Injury Induced by Exhausted-Exercise via Regulation of Cytoskeleton
Sign Up to like & getrecommendations! Published in 2022 at "Frontiers in Pharmacology"
DOI: 10.3389/fphar.2022.891802
Abstract: Background: Yu-ping-feng powder (YPF) is a compound traditional Chinese medicine extensively used in China for respiratory diseases. However, the role of YPF in alveolar-capillary barrier dysfunction remains unknown. This study aimed to explore the effect… read more here.
Keywords: exhausted exercise; ypf; alveolar capillary; capillary barrier ... See more keywords
Alveolar–Capillary Barrier Protection In Vitro: Lung Cell Type-Specific Effects and Molecular Mechanisms Induced by 1α, 25-Dihydroxyvitamin D3
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DOI: 10.3390/ijms24087298
Abstract: Low serum levels of 1α, 25-dihydroxyvitamin D3 (VD3) are associated with a higher mortality in trauma patients with sepsis or ARDS. However, the molecular mechanisms behind this observation are not yet understood. VD3 is known… read more here.
Keywords: vd3; cell; molecular mechanisms; alveolar capillary ... See more keywords
Viable Allogeneic Mitochondria Transplantation Improves Gas Exchange and Alveolar-Capillary Permeability in Rats with Endotoxin-Induced Acute Lung Injuries
Sign Up to like & getrecommendations! Published in 2022 at "International Journal of Medical Sciences"
DOI: 10.7150/ijms.73151
Abstract: Background: Acute lung injuries (ALI) cause disruption of the alveolar-capillary barrier and is the leading cause of death in critically ill patients. This study tested the hypothesis that the administration of freshly isolated viable allogeneic… read more here.
Keywords: lung; transplantation; alveolar capillary; allogeneic mitochondria ... See more keywords