Pulmonary Lymphatic Disorders

A Unique Shunt For Treatment Of A Postoperative Chylothorax

Rita Costa, Tiago Magalhaes, Gustavo Rocha, Pedro Fernandes, Elson SalgueiroUniversity Hospital Center of São João.Portugal Portuguese Journal of Cardiac Thoracic and Vascular SurgeryPort J Card Thorac Vasc Surg 2022; 29: 63-65DOI: 10.48729/pjctvs.304 AbstractPostoperative chylothorax can be a serious complication. We report on the case of a neonate who had a postoperative chylothorax immediately after esophageal surgery […]

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Management of refractory chylothorax in the neonatal intensive care unit: A 22-year experience

Yoshiaki Takahashi, Yoshiaki Kinoshita, Takashi Kobayashi, Yuhki Arai, Toshiyuki Ohyama, Naoki Yokota, Koichi Saito, Yu Sugai, Shoichi TakanoNiigata University Graduate School of Medical and Dental SciencesJapan Pediatric InternationalPediatr Int 2022; 64: DOI: 10.1111/ped.15043 AbstractBackground: The aim was to assess the therapeutic strategy of patients with chylothorax in a neonatal intensive care unit.Methods: Twenty-eight infants with chylothorax were included

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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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Pathogenic variants in MDFIC cause recessive central conducting lymphatic anomaly with lymphedema

Alicia B. Byrne, Pascal Brouillard, Drew L. Sutton, Jan Kazenwadel, Saba Montazaribarforoushi, Genevieve A. Secker, Anna Oszmiana, Milena Babic, Kelly L. Betterman, Peter J. Brautigan, Melissa White, Sandra G. Piltz, Paul Q. Thomas, Christopher N. Hahn, Matthias Rath, Ute Felbor, G. Christoph Korenke, Christopher L. Smith, Kathleen H. Wood, Sarah E. Sheppard, Denise M. Adams,

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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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Propranolol treatment for chylothorax after congenital cardiac surgery

Rozelle Corda, Sophia Chrisomalis-Dring, Sarah Crook, Carrie J. Shawber, June K. Wu, Paul J. ChaiNew York Presbyterian/Morgan Stanley Children’s Hospital, Columbia University Irving Medical Center and Vagelos College of Physicians & SurgeonsUnited States Journal of Thoracic and Cardiovascular SurgeryJ Thorac Cardiovasc Surg 2022; 163: 1630-1641DOI: 10.1016/j.jtcvs.2021.09.007 AbstractObjective: Postoperative chylothorax causes significant morbidities in pediatric patients with cardiac

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Lymphatic Phenotype of Noonan Syndrome: Innovative Diagnosis and Possible Implications for Therapy

Lotte E. R. Kleimeier, Carolinevan Schaik, Erika Leenders, Maxim Itkin, Willemijn M. Klein, Jos M. T. DraaismaRadboudumc Amalia Children’s Hospital and Radboud University Medical Center. University of Pennsylvania.Netherlands and United States Journal of Clinical MedicineJ Clin Med 2022; 11: DOI: 10.3390/jcm11113128 AbstractDysregulation of the Ras/Mitogen-activated protein kinase (MAPK) signaling pathway is suggested to play a pivotal

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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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Development of consensus recommendations for the management of post-operative chylothorax in paediatric CHD

Richard P. Lion, Melissa M. Winder, Rambod Amirnovin, Kristi Fogg, Rebecca Bertrandt, Priya Bhaskar, Cameron Kasmai, Kathryn W. Holmes, Rohin Moza, Piyagarnt Vichayavilas, Erin E. Gordon, Amiee Trauth, Megan Horsley, Deborah U. Frank, Arabela Stock, Greg Adamson, Alissa Lyman, Tia Raymond, Isaura Diaz, Alicia DeMarco, Parthak Prodhan, Michael Fundora, Alaa Aljiffry, Aaron G. Dewitt, Benjamin

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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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