Review Articles Concerning Pulmonary Vascular Disease

Pharmacotherapy for Pulmonary Hypertension in Infants with Bronchopulmonary Dysplasia: Past, Present, and Future

Candice D. Fike, Judy L. AschnerUniversity of Utah Health. Joseph M. Sanzari Children’s Hospital at Hackensack University.United States PharmaceuticalsPharmaceuticals 2023; 16: DOI: 10.3390/ph16040503 AbstractApproximately 8-42% of premature infants with chronic lung disease of prematurity, bronchopulmonary dysplasia (BPD), develop pulmonary hypertension (PH). Infants with BPD-PH carry alarmingly high mortality rates of up to 47%. Effective PH-targeted pharmacotherapies […]

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Diagnosis and management of pulmonary hypertension in infants with bronchopulmonary dysplasia: a guide for paediatric respiratory specialists

Sarah Chan, Rossa Brugha, Sadia Quyam, Shahin MoledinaGreat Ormond Street Hospital for Children. NHS Foundation Trust. University College London, London. United Kingdom BreatheBreathe 2022; 18: DOI: 10.1183/20734735.0209-2022 AbstractPulmonary hypertension (PH) can develop in babies with bronchopulmonary dysplasia (BPD). PH is common in those with severe BPD and is associated with a high mortality rate. However, in

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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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