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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 »

MRI evaluation of right heart functions in children with mild cystic fibrosis

Hafize Otcu Temur, Alpay Alkan, Can Yilmaz Yozgat, Erkan Cakir, Hakan Yazan, Fatma Celik Yabul, Dilek Hacer Cesme, Yilmaz YozgatBezmialem Vakif University. Istanbul Medipol University.Turkey Cardiology in the YoungCardiol Youg 2022; 13: DOI: 10.1017/S1047951122003249 AbstractBackground: This study aimed to assess the ventricular anatomy, function of the right ventricle, and the haemodynamic findings of pulmonary artery in children

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Expected small left heart size in the presence of congenital diaphragmatic hernia: Fetal values and Z-scores for infants confirmed to have no heart disease postnatally

Anita J. Moon-Grady, Francesca A. Byrne, Leslie A. Lusk, Roberta L. KellerUniversity of California San Francisco and Benioff Children’s Hospital.United States Frontiers in PediatricsFront Pediatr 2022; DOI: 10.3389/fped.2022.1083370 AbstractObjectives: In fetuses with left-sided congenital diaphragmatic hernia (CDH), left heart structures may appear small, but usually normalize after birth in the absence of structural cardiac anomalies. To decrease

Expected small left heart size in the presence of congenital diaphragmatic hernia: Fetal values and Z-scores for infants confirmed to have no heart disease postnatally Read More »

Use of Vasopressin as Rescue Therapy in Refractory Hypoxia and Refractory Systemic Hypotension in Term Neonates with Severe Persistent Pulmonary Hypertension-A Prospective Observational Study

Sachin Shah, Saleha Dhalait, Anurag Fursule, Jayant Khandare, Amita KaulSurya Mother & Child Superspeciality Hospital.India America Journal of PerinatologyAm J Perinatol 2022; DOI: 10.1055/a-1969-1119 AbstractObjective: Persistent pulmonary hypertension of the newborn (PPHN) is a serious cardiorespiratory problem. PPHN is frequently associated with refractory hypoxia and hypotension, and optimal management has the potential to improve important clinical outcomes

Use of Vasopressin as Rescue Therapy in Refractory Hypoxia and Refractory Systemic Hypotension in Term Neonates with Severe Persistent Pulmonary Hypertension-A Prospective Observational Study Read More »

TGF-β receptor mutations and clinical prognosis in Chinese pediatric patients with idiopathic/hereditary pulmonary arterial hypertension

Xinyu Zhang, Chen Zhang, Qiangqiang, Li, Hong GuBeijing Anzhen Hospital and Capital Medical University.China Pulmonary CirculationPulm Circ 2022; 12: DOI: 10.1002/pul2.12076 AbstractThe relationship between clinical prognosis and transforming growth factor-β (TGF-β) receptor mutations in Chinese pediatric patients with idiopathic/hereditary pulmonary arterial hypertension (IPAH/HPAH) remains unclear. We retrospectively studied the clinical characteristics and outcomes of pediatric patients

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Dichotomous role of integrin-β5 in lung endothelial cells

Neil Blanchard, Patrick A. Link, Daniela Farkas, Brennan Harmon, Jaylen Hudson, Srimathi Bogamuwa, Bryce Piper, Kayla Authelet, Carlyne D. Cool, Rebecca L. Heise, Robert Freishtat, Laszlo FarkasUniversity of Virginia. Ohio State University. Children’s National Health System. University of Colorado. Virginia Commonwealth University.United States Pulmonary CirculationPulm Circ 2022; DOI: 10.1002/pul2.12156 AbstractPulmonary arterial hypertension (PAH) is a progressive,

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Echocardiographic measures of ventricular-vascular interactions in congenital diaphragmatic hernia

Sanjeev Aggarwal, Christina Shanti, Prashant Agarwal, Joseph Lelli, Girija NatarajanCentral Michigan University and Children’s Hospital of Michigan.United States Early Human DevelopmentEarly Hum Dev 2022; 165: DOI: 10.1016/j.earlhumdev.2021.105534 AbstractObjective: To evaluate the utility of echocardiographic measures of ventricular-vascular interactions in predicting death or ECMO in congenital diaphragmatic hernia (CDH).Design: In this single center retrospective study, early (<48 hour age)

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Morbidity in children after fetoscopic endoluminal tracheal occlusion for severe congenital diaphragmatic hernia: Results from a multidisciplinary clinic

Shelby R. Sferra, Melanie K. Nies, Jena L. Miller, Alejandro V. Garcia, Erica I. Hodgman, Annalise B. Penikis, Abigail J. Engwall-Gill, Vera Joanna Burton, Jessica L. Rice, Peter J. Mogayzel, Ahmet A. Baschat, Shaun M. KunisakiJohns Hopkins University School of Medicine. United States Journal of Pediatric SurgeryJ Pediatr Surg 2023; 58: 14-19DOI: 10.1016/j.jpedsurg.2022.09.042 AbstractBackground: Although fetoscopic endoluminal

Morbidity in children after fetoscopic endoluminal tracheal occlusion for severe congenital diaphragmatic hernia: Results from a multidisciplinary clinic Read More »

Long-term outcomes of congenital diaphragmatic hernia: A single institution experience

Claire D. Gerall, Latoya A. Stewart, Jessica Price, Sandra Kabagambe, Shelby R. Sferra, Rebecca Hernan, Julie Khlevner, Usha S. Krishnan, Alive De, Gudrun Aspelund, Vincent P. DuronColumbia University Vagelos College of Physicians and Surgeons and NewYork-Presbyterian Morgan Stanley Children’s Hospital.United States Journal of Pediatric SurgeryJ Pediatr Surg 2022; 57: 563-569DOI: 10.1016/j.jpedsurg.2021.06.007 AbstractBackground/purpose: As survival rates for patients

Long-term outcomes of congenital diaphragmatic hernia: A single institution experience Read More »

Association between Genotype, Presentation, and Outcome in Childhood Idiopathic and Hereditary Pulmonary Arterial Hypertension

Zhuoyuan Xu, Hongsheng Zhang, Chen Zhang, Qiangqiang Li, Hong GuBeijing Anzhen Hospital and Capital Medical University, No. 2.China Journal of Clinical MedicineJ Clin Med 2022; 11: DOI: 10.3390/jcm11247331 AbstractBackground: Paediatric-onset idiopathic/hereditary pulmonary arterial hypertension (IPAH/HPAH) is partially linked to genetic factors that may also affect treatment response and outcome. The relation between clinical characteristics and pathogenicity of

Association between Genotype, Presentation, and Outcome in Childhood Idiopathic and Hereditary Pulmonary Arterial Hypertension Read More »

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