Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease

Lymphatic anomalies during lifetime in patients with Noonan syndrome: Retrospective cohort study

Jessie W. Swarts, Lotte E. R. Kleimeier, Erika K. S. M. Leenders, Tuula Rinne, Willemijn M. Klein, Jos M. T. DraaismaAmalia Children’s Hospital, Radboud Institute for Health Sciences and Radboud University Medical CenterNetherlands American Journal of Medical Genetics AAm J Med Genet A 2022; 188: 3242-3261DOI: 10.1002/ajmg.a.62955 AbstractNoonan syndrome (NS) has been associated with an increased […]

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MR lymphangiography of lymphatic abnormalities in children and adults with Noonan syndrome

C. C. Pieper, J. Wagenpfeil, A. Henkel, S. Geiger, T. Köster, K. Hoss, J. A. Luetkens, C. Hart, U. I. Attenberger, A. MüllerUniversity of Bonn and Children’s Hospital.Germany Scientific ReportsSci Rep 2022; 12: DOI: 10.1038/s41598-022-13806-w AbstractNoonan syndrome is associated with complex lymphatic abnormalities. We report dynamic-contrast enhanced MR lymphangiography (DCMRL) findings in children and adults with

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The spectrum of imaging manifestations of Gorham-Stout disease: a novel dynamic contrast-enhanced MR lymphangiography

Yuna Lee, Seunghyun Lee, Saebeom Hur, Yun Soo Jeong, Dong In Suh, Jangsup Moon, Man Jin Kim, Young Hun Choi, Jung‑Eun CheonSeoul National University Hospital. Seoul National University College of Medicine. Republic of Korea Orphanet Journal of Rare DiseasesOrphanet J Rare Dis 2023; 18: DOI: 10.1186/s13023-023-02704-7 AbstractBackground: To describe the radiological features of Gorham-Stout disease (GSD) as

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Brain Abscess and Stroke in Children and Adults With Hereditary Hemorrhagic Telangiectasia: Analysis of a Large National Claims Database

Andrew J. White, Itay Marmor, Kate M. Peacock, Katelin B. Nickel, Jessica Zavadil, Margaret A. OlsenSaint Louis University School of Medicine. Washington University School of Medicine.United States NeurologyNeurology 2023; DOI: 10.1212/WNL.0000000000207269 AbstractObjective: Hereditary hemorrhagic telangiectasia (HHT) is an inherited disease associated with pathogenic variants in TGF-β signaling pathway-related genes, resulting in abnormal vascular development in various organs.

Brain Abscess and Stroke in Children and Adults With Hereditary Hemorrhagic Telangiectasia: Analysis of a Large National Claims Database Read More »

Systemic artery to pulmonary artery aneurysm malformations associated with variants at MCF2L

S.E. Mitchell, R. P. Martin, P. Terry, S. E. Drant, D. Valle, H. Dietz, N. SobreiraJohns Hopkins University School of Medicine. Children’s Hospital of Philadelphia. United States American Journal of Medical GeneticsAm J Med Genet 2023; 191: 1250-1260DOI: 10.1002/ajmg.a.63141 AbstractArteriovenous malformations (AVM) are characterized by abnormal vessels connecting arteries and veins resulting in a disruption of

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3D CT cinematic rendering of pediatric thoracic vascular anomalies

Hannah S. Rechta, Edmund M. Weisbergb, Elliot K. FishmanNorthwestern University Feinberg School of Medicine. Johns Hopkins University School of Medicine.United States European Journal of Radiology OpenEur J Radiol Open 2023; DOI: 10.1016/j.ejro.2023.100485 AbstractThoracic vascular anomalies in the pediatric population are a heterogeneous group of diseases, with varied clinical presentations and imaging findings. High-resolution computed tomography is

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Quantification of systemic-to-pulmonary collateral flow in univentricular physiology with 4D flow MRI

Floris-Jan S. Ridderbos, Frandics P. Chan, Joost P. van Melle, Tjark Ebels, Jeffrey A. Feinstein, Rolf M. F. Berger, Tineke P. WillemsLucile Packard Children’s Hospital and Stanford University Medical Center. Beatrix Children’s Hospital, University Medical Center Groningen, and University of Groningen. University of Amsterdam. Netherlands and United States Cardiology in the YoungCardiol Young 2022; DOI: 10.1017/S1047951122002840

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First Genotype-Phenotype Study in TBX4 Syndrome: Gain-of-Function Mutations Causative for Lung Disease

Matina Prapa, Mauro Lago-Docampo, Emilia M. Swietlik, David Montani, Melanie Eyries, Marc Humbert, Carrie L. Welch, Wendy K. Chung, Rolf M. F. Berger, Harm Jan Bogaard, Olivier Danhaive, Pilar Escribano-Subıas, Henning Gall, Barbara Girerd, Ignacio Hernandez-Gonzalez, Simon Holden, David Hunt, Samara M. A. Jansen, Wilhelmina Kerstjens-Frederikse, David G. Kiely, Pablo Lapunzina, John McDermott, Shahin Moledina,

First Genotype-Phenotype Study in TBX4 Syndrome: Gain-of-Function Mutations Causative for Lung Disease Read More »

Physiologically based pharmacokinetic modelling of treprostinil after intravenous injection and extended-release oral tablet administration in healthy volunteers: An extrapolation to other patient populations including patients with hepatic impairment

Xuemei Wu, Xiaohan Zhang, Ruichao Xu, Imam Hussain Shaik, Raman VenkataramananUniversity of Pittsburgh School of Pharmacy. Fujian Medical University Union Hospital. University of Virginia. University of Pittsburgh School of Medicine.United States and China British Journal of Clinical PharmacologyBrit J Clin Pharmacol 2022; 88: 587-599DOI: 10.1111/bcp.14966 AbstractAims: Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary arterial pressure,

Physiologically based pharmacokinetic modelling of treprostinil after intravenous injection and extended-release oral tablet administration in healthy volunteers: An extrapolation to other patient populations including patients with hepatic impairment Read More »

Construction and Evaluation of a Bio-Engineered Pump to Enable Subpulmonary Support of the Fontan Circulation: A Proof-of-Concept Study

Christopher R. Broda, P. Alex Smith, Yaxin Wang, Hamsini Sriraman, Peter S. Owuor, Luiz C. Sampaio, Iki Adachi, Doris A. Taylor, Christopher R. Broda, P. Alex Smith, Yaxin Wang, Hamsini Sriraman, Peter S. OwuorBaylor College of Medicine and Texas Children’s Hospital. Texas Medical Center. Texas Heart Institute. Pennsylvania State University. University of Texas Health Science

Construction and Evaluation of a Bio-Engineered Pump to Enable Subpulmonary Support of the Fontan Circulation: A Proof-of-Concept Study Read More »

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