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Page 1
Pediatric Pulmonary Hypertension: Definitions, Mechanisms, Diagnosis, and Treatment.
Mukherjee D, Konduri GG. Mukherjee D, et al. Compr Physiol. 2021 Jun 30;11(3):2135-2190. doi: 10.1002/cphy.c200023. Compr Physiol. 2021. PMID: 34190343 Free PMC article.
The best outcomes are for neonates with persistent pulmonary hypertension (PPHN) and reversible lung diseases, while some genetic conditions such as alveolar capillary dysplasia are lethal. 2021 American Physiological Society. Compr Physiol 11:2135-2190, 20 …
The best outcomes are for neonates with persistent pulmonary hypertension (PPHN) and reversible lung diseases, while some genetic conditions …
A Morphomolecular Approach to Alveolar Capillary Dysplasia.
Kamp JC, Neubert L, Ackermann M, Stark H, Plucinski E, Shah HR, Janciauskiene S, Bergmann AK, Schmidt G, Welte T, Haverich A, Werlein C, Braubach P, Laenger F, Schwerk N, Olsson KM, Fuge J, Park DH, Schupp JC, Hoeper MM, Kuehnel MP, Jonigk DD. Kamp JC, et al. Am J Pathol. 2022 Aug;192(8):1110-1121. doi: 10.1016/j.ajpath.2022.05.004. Epub 2022 May 29. Am J Pathol. 2022. PMID: 35649494 Free article.
Alveolar capillary dysplasia (ACD) is a rare lung developmental disorder leading to persistent pulmonary arterial hypertension and fatal outcomes in newborns. ...
Alveolar capillary dysplasia (ACD) is a rare lung developmental disorder leading to persistent pulmonary arterial hyper …
Pulmonary Hypertension in Developmental Lung Diseases.
Danhaive O, Galambos C, Lakshminrusimha S, Abman SH. Danhaive O, et al. Clin Perinatol. 2024 Mar;51(1):217-235. doi: 10.1016/j.clp.2023.12.001. Epub 2023 Dec 28. Clin Perinatol. 2024. PMID: 38325943 Review.
Novel FOXF1-Stabilizing Compound TanFe Stimulates Lung Angiogenesis in Alveolar Capillary Dysplasia.
Pradhan A, Che L, Ustiyan V, Reza AA, Pek NM, Zhang Y, Alber AB, Kalin TR, Wambach JA, Gu M, Kotton DN, Siefert ME, Ziady AG, Kalin TV, Kalinichenko VV. Pradhan A, et al. Am J Respir Crit Care Med. 2023 Apr 15;207(8):1042-1054. doi: 10.1164/rccm.202207-1332OC. Am J Respir Crit Care Med. 2023. PMID: 36480964 Free PMC article.
Rationale: Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is linked to heterozygous mutations in the FOXF1 (Forkhead Box F1) gene, a key transcriptional regulator of pulmonary vascular development. ...
Rationale: Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is linked to heterozygous mutatio …
FOXF1 Regulates Alveolar Epithelial Morphogenesis through Transcriptional Activation of Mesenchymal WNT5A.
Reza AA, Kohram F, Reza HA, Kalin TR, Kannan PS, Zacharias WJ, Kalinichenko VV. Reza AA, et al. Am J Respir Cell Mol Biol. 2023 Apr;68(4):430-443. doi: 10.1165/rcmb.2022-0191OC. Am J Respir Cell Mol Biol. 2023. PMID: 36542853 Free PMC article.
Mutations in the FOXF1 (forkhead box F1) gene, encoding the mesenchymal FOX (forkhead box) transcription factor, are linked to alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV), a severe congenital disorder associated with the loss of …
Mutations in the FOXF1 (forkhead box F1) gene, encoding the mesenchymal FOX (forkhead box) transcription factor, are linked to alveolar …
Bronchopulmonary dysplasia with pulmonary hypertension associates with semaphorin signaling loss and functionally decreased FOXF1 expression.
Shirazi SP, Negretti NM, Jetter CS, Sharkey AL, Garg S, Kapp ME, Wilkins D, Fortier G, Mallapragada S, Banovich NE, Eldredge LC, Deutsch GH, Wright CVE, Frank DB, Kropski JA, Sucre JMS. Shirazi SP, et al. Nat Commun. 2025 May 30;16(1):5004. doi: 10.1038/s41467-025-60371-7. Nat Commun. 2025. PMID: 40442177 Free PMC article.
Loss of semaphorin signaling is replicated in a murine BPD model and in humans with causal FOXF1 mutations for alveolar capillary dysplasia (ACDMPV), suggesting a mechanistic link between developmental programs underlying BPD and ACDMPV and uncovering a criti …
Loss of semaphorin signaling is replicated in a murine BPD model and in humans with causal FOXF1 mutations for alveolar capillary …
Prenatal Rare 16q24.1 Deletion Between Genomics and Epigenetics: A Review.
Fumini V, Bonora R, Busciglio A, Cartisano F, Celli P, Gabbiato I, Guercini N, Mancini B, Saccilotto D, Zilio A, Zuccarello D. Fumini V, et al. Genes (Basel). 2025 Jul 24;16(8):873. doi: 10.3390/genes16080873. Genes (Basel). 2025. PMID: 40869921 Free PMC article. Review.
Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a rare, often fatal congenital disorder characterized by severe neonatal respiratory distress and associated with complex multisystem malformations. ...
Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a rare, often fatal congenital disorder c …
Identification of endothelial and mesenchymal FOXF1 enhancers involved in alveolar capillary dysplasia.
Wang G, Wen B, Guo M, Li E, Zhang Y, Whitsett JA, Kalin TV, Kalinichenko VV. Wang G, et al. Nat Commun. 2024 Jun 19;15(1):5233. doi: 10.1038/s41467-024-49477-6. Nat Commun. 2024. PMID: 38898031 Free PMC article.
Mutations in the FOXF1 gene, a key transcriptional regulator of pulmonary vascular development, cause Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins, a lethal lung disease affecting newborns and infants. ...
Mutations in the FOXF1 gene, a key transcriptional regulator of pulmonary vascular development, cause Alveolar Capillary Dy …
Familial Alveolar Capillary Dysplasia With Misalignment of Pulmonary Veins Caused by Paternal FOXF1 Upstream Enhancer Deletion: A Case Report.
Kawasaki H, Nakabayashi K, Ikeda M, Onda T, Tomotaki S, Torishima M, Saito A, Ohashi H, Minamiguchi S, Hata K, Hayakawa M, Kawai M, Cho K, Kosugi S, Yamada T. Kawasaki H, et al. Am J Med Genet A. 2025 Jul;197(7):e64056. doi: 10.1002/ajmg.a.64056. Epub 2025 Mar 20. Am J Med Genet A. 2025. PMID: 40114448
Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a lethal disease characterized by severe respiratory distress and pulmonary hypertension during the neonatal period, which is seldom diagnosed prenatally. ...
Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a lethal disease characterized by severe …
Nanoparticle Delivery of STAT3 Alleviates Pulmonary Hypertension in a Mouse Model of Alveolar Capillary Dysplasia.
Sun F, Wang G, Pradhan A, Xu K, Gomez-Arroyo J, Zhang Y, Kalin GT, Deng Z, Vagnozzi RJ, He H, Dunn AW, Wang Y, York AJ, Hegde RS, Woods JC, Kalin TV, Molkentin JD, Kalinichenko VV. Sun F, et al. Circulation. 2021 Aug 17;144(7):539-555. doi: 10.1161/CIRCULATIONAHA.121.053980. Epub 2021 Jun 11. Circulation. 2021. PMID: 34111939 Free PMC article.
BACKGROUND: Pulmonary hypertension (PH) is a common complication in patients with alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV), a severe congenital disorder associated with mutations in the FOXF1 gene. ...
BACKGROUND: Pulmonary hypertension (PH) is a common complication in patients with alveolar capillary dysplasia with mis …
53 results