Review Articles Concerning Pulmonary Vascular Disease

Lymphatic Abnormalities in Noonan Syndrome Spectrum Disorders: A Systematic Review

Julia Sleutjes, Lotte Kleimeier, Erika Leenders, Willemijn Klein, Jos DraaismaRadboud University Medical Center and Amalia Children’s Hospital.Netherlands Molecular SyndromologyMol Syndromol 2022; DOI: 10.1159/000517605 AbstractNoonan syndrome spectrum disorders are a group of phenotypically related conditions, resembling Noonan syndrome, caused by germline pathogenic variants in genes within the Ras/mitogen-activated protein kinase (Ras/MAPK) signalling pathway. Lymphatic dysplasia with a […]

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Lymphatic anomalies in congenital heart disease

Karen I. Ramirez-Suarez, Luis Octavio Tierradentro-Garcia, David M. Biko, Hansel J. Otero, Ammie M. White, Yoav Dori, Christopher L. Smith, Seth Vatsky, Jordan B. RappChildren’s Hospital of Philadelphia and University of Pennsylvania.United States Pediatric RadiologyPediatr Radiol 2022; 52: 1862-1876DOI: 10.1007/s00247-022-05449-w AbstractCongenital heart disease can lead to various lymphatic complications including traumatic leaks, lymphatic overproduction, conduction abnormalities

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Congenital Chylothorax of the Newborn: A Systematic Analysis of Published Cases between 1990 and 2018

Bernhard Resch, Gülsen Sever Yildiz, Friedrich ReitererMedical University of GrazAustria RespirationRespiration 2022; 101: 84-96DOI: 10.1159/000518217 AbstractBackground: Congenital chylothorax (CCT) of the newborn is a rare entity but the most common cause of pleural effusion in this age-group. We aimed to find the optimal treatment strategy.Material and methods: A PubMed search was performed according to the PRISMA criteria. All

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Congenital lung malformations: Dysregulated lung developmental processes and altered signaling pathways

Fabian Doktor, Lina Antounians, Martin Lacher, Augusto ZaniThe Hospital for Sick Children and University of Toronto. University of Leipzig. Canada and Germany Seminars in Pediatric SurgerySemin Pediatr Surg 2022; 31: DOI: 10.1016/j.sempedsurg.2022.151228 AbstractCongenital lung malformations comprise a diverse group of anomalies including congenital pulmonary airway malformation (CPAM, previously known as congenital cystic adenomatoid malformation or CCAM),

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Notch signalling in healthy and diseased vasculature

Francesca Del Gaudio, Dongli Liu, Urban LendahlKarolinska Institutet. The First Affiliated Hospital of Guangxi Medical University.Sweden and China Open BiologyOpen Biol 2022; 12: DOI: 10.1098/rsob.220004 AbstractNotch signalling is an evolutionarily highly conserved signalling mechanism governing differentiation and regulating homeostasis in many tissues. In this review, we discuss recent advances in our understanding of the roles that

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Review of the Pathologic Characteristics in Myhre Syndrome: Gain-of-Function Pathogenic Variants in SMAD4 cause a Multisystem Fibroproliferative Response

Lois J. Starr, Mark E. Lindsay, Deborah Perry, Gregory Gheewalla, Paul A. VanderLaan, Adnan Majid, Charlie Strange, George-Claudiu Costea, Adrian Lungu, Angela E. LinUniversity of Nebraska Medical Center. Harvard Medical School and MassGeneral Hospital for Children. Massachusetts General Hospital. Methodist Hospital. Tufts University School of Medicine. Beth Israel Deaconess Medical Center. Medical University of South

Review of the Pathologic Characteristics in Myhre Syndrome: Gain-of-Function Pathogenic Variants in SMAD4 cause a Multisystem Fibroproliferative Response Read More »

High altitude pulmonary edema in children: A systematic review

Santiago Ucros, Camila Aparicio, Jose Castro-Rodriguez, D. Dunbar IvyUniversidad de los Andes School of Medicine. Pontificia Universidad Católica de Chile. Children’s Hospital Colorado and University of Colorado School of Medicine.Columbia, Chile and United States Pediatric PulmonologyPediatr Pulmonol 2023; 58: 1059-1067DOI: 10.1002/ppul.26294 AbstractIntroduction: High altitude pulmonary edema (HAPE) is a form of acute noncardiogenic pulmonary edema caused by

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Fetal endoscopic tracheal occlusion for moderate and severe congenital diaphragmatic hernia: a systematic review and meta-analysis of randomized controlled trials

Qiyu Li, Siyuan Liu, Xuemei Ma, Jiaping YuGeneral Hospital of Northern Theater Command. China Pediatric Surgery InternationalPediatr Surg 2022; 38: 1217-1226 DOI: 10.1007/s00383-022-05170-7 AbstractBackground: Fetoscopic endoluminal tracheal occlusion (FETO) is considered to increase survival among fetuses with congenital diaphragmatic hernia (CDH). Data from high-quality trials had been lacking until the largest randomized controlled trials (the TOTAL trials)

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Association of the dysfunctional placentation endotype of prematurity with bronchopulmonary dysplasia: a systematic review, meta-analysis and meta-regression

Maria Pierro, Eduardo Villamor-Martinez, Elke van Westering- Kroon, Maria Alvarez-Fuente, Steven H Abman, Eduardo VillamorMaastricht University Medical Centre. Maurizio Bufalini Hospital. Hospital Universitario Ramón y Cajal. University of Colorado – Anschutz Medical Campus. Netherlands, Italy, Spain and United States ThoraxThorax 2022; 77: 268-275DOI: 10.1136/thoraxjnl-2020-216485 AbstractBackground: Antenatal pathological conditions are key in the pathogenesis of bronchopulmonary dysplasia (BPD).

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Chronic Inflammation as the Underlying Mechanism of the Development of Lung Diseases in Psoriasis: A Systematic Review

Mateusz Mleczko, Agnieszka Gerkowicz, Dorata KrasowskaMedical University of LublinPoland International Journal of Molecular ScienceInt J Mol Sci 2022; 23: DOI: 10.3390/ijms23031767 AbstractPsoriasis is a systemic inflammatory disease caused by dysfunctional interactions between the innate and adaptive immune responses. The systemic inflammation in psoriasis may be associated with the development of comorbidities, including lung diseases. In this

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