Primary Pulmonary Lymphatic Disease

Severe Lymphatic Disorder and Multifocal Atrial Tachycardia Treated with Trametinib in a Patient with Noonan Syndrome and SOS1 Mutation

Michele Lioncino, Adelaide Fusco, Emanuele Monda, Diego Colonna, Martina Caiazza, Michelina Sibilio, Daniela Magri, Angela Carla Borrelli, Barbara D’Onofrio, Maria Luisa Mazzella, Rossella Colantuono, Maria Rosaria Arienzo, Berardo Sarubbi, Maria Giovanna Russo, Giovanni Chello, Giuseppe LimongelliUniversity of Campania “Luigi Vanvitelli” and Monaldi Hospital. University College of London and St. Bartholomew’s Hospital.Italy and United Kingdom GenesGenes […]

Severe Lymphatic Disorder and Multifocal Atrial Tachycardia Treated with Trametinib in a Patient with Noonan Syndrome and SOS1 Mutation Read More »

Refractory chylothorax after severe vomiting and coughing in a 4-year-old child

Vincent De Pauw, Siel Daelemans, Leontien Depoorter, Carola Brussaard, Dirk SmetsUZ Brussel.Belgium Journal of Surgical Case ReportsJ Surg Case Rep 2023; DOI: 10.1093/jscr/rjad466 AbstractChylothorax is the accumulation of lymphatic fluid in the pleural space. It is a rare condition with potentially life-threatening disorders. In children, the etiology of chylothorax can be mainly attributed to idiopathic factors,

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Cough-induced chylothorax in a two-year-old boy – case report and review of the literature

Melanie Anger, Julian Hofmann, Bettina Ruf, Marc Steinborn, Daniela Reber, Katharina Warncke, Nikolaus RieberKinderklinik München Schwabing, Munich Klinik and School of Medicine and Technical University of Munich. Germany BioMed Central PediatricsBMC Pediatr 2023; 23:DOI: 10.1186/s12887-023-04221-9 AbstractBackground: Chylothorax is a very rare form of pleural effusion in children, especially after the neonatal period, and predominantly occurs secondary to cardiothoracic

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Case report: Blotchy skin in a puffy neonate: is there a new association?

Chacko J. Joseph, Arijit Lodha, Soumya R. Thomas, Essa Al Awad, Nicola A. M. Wright, Cora Constantinescu, Doan Le, Majeeda KamaluddeenUniversity of Calgary Cumming School of Medicine. University of Alberta. Canada Frontiers in PediatricsFront Pediatr 2023; 11:DOI: 10.3389/fped.2023.1247343 AbstractIntroduction: Purpura fulminans in the neonatal population is a rare but potentially life-threatening condition complicated by thrombosis, resultant vital

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Non-Contrast MR Lymphography and Intranodal Dynamic Contrast MR Lymphangiography in Children with Congenital Heart Disease-Imaging Findings as well as Impact on Patient Management and Outcome

Christoph Bauer, Mario Scala, Pavel Sekyra,Franz Fellner, Gerald TulzerKepler University Hospital GmbH. Johannes Kepler University Linz. Austria International Journal of Molecular SciencesInt J Mol Sci 2023; 24:DOI: 10.3390/ijms241914827 AbstractLymphatic flow disorders are rare but devastating complications in children with congenital heart disease. T2-weighted magnetic resonance lymphography and intranodal dynamic contrast magnetic resonance lymphangiography are imaging modalities

Non-Contrast MR Lymphography and Intranodal Dynamic Contrast MR Lymphangiography in Children with Congenital Heart Disease-Imaging Findings as well as Impact on Patient Management and Outcome Read More »

Chylothorax related to acute SARS-CoV-2 infection in a patient with Noonan syndrome with prior uncomplicated cardiac surgeries

Lubaina Ehsan, Jessica A. Thoe, John J. Parent, Joseph D. FakhouryWestern Michigan University Homer Stryker, M.D. School of Medicine and Bronson Children’s Hospital. Indiana University School of Medicine.United States Cardiology in the YoungCardiol Young 2024; 34: 448-451DOI: 10.1017/S1047951123004171 AbstractSARS-CoV-2 is a novel coronavirus that has rarely been associated with chylothorax. Patients with Noonan syndrome are at

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PKD1L1 Is Involved in Congenital Chylothorax

Jonathan B. Whitchurch, Sophia Schneider, Alina C. Hilger, Ricarda Köllges, Jil D. Stegmann, Lea Waffenschmidt, Laura Dyer, Holger Thiele, Bhanupriya Dhabhai, Tikam Chand Dakal, Andreas Müller, Dominic P. Norris, Heiko M. ReutterHarwell Campus. University Hospital Bonn. University Hospital Erlangen. University of Cologne. Mohanlal Sukhadia University.United Kingdom, Germany and India CellsCells 2024; 13:DOI: 10.3390/cells13020149 AbstractBesides visceral heterotaxia, Pkd1l1 null

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Chylothorax Associated with the Syndrome of Idiopathic Osteolysis Including Gorham-Stout Disease: Spontaneous Remission Without Treatment

Sota Monma, Michihiro Yano, Masazumi Matsuda, Hiroyuki Tsuchie, Naoko MoriAkita University Graduate School of Medicine. Japan Turkish Archives of PediatricsTurk Arch Pediatr 2024; 59: 230-231DOI: 10.5152/TurkArchPediatr.2024.23208 AbstractAbstract Not Available CategoryPrimary Pulmonary Lymphatic DiseaseGenetic Factors Associated with Pulmonary Vascular Disease Age Focus: Pediatric Pulmonary Vascular Disease Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since

Chylothorax Associated with the Syndrome of Idiopathic Osteolysis Including Gorham-Stout Disease: Spontaneous Remission Without Treatment Read More »

Pulmonary vascular changes induced by congenital obstruction of pulmonary venous return

Masato Endo, Shigeo Yamaki, Mikio Ohmi, Koichi TabayashiTohoku University School of Medicine.Japan Annals of Thoracic SurgeryAnn Thorac Surg 2000; 69: 193-197DOI: 10.1016/s0003-4975(99)01079-6 AbstractBackground: Pulmonary venous obstruction (PVO) induces pulmonary arterial hypertension, as well as pulmonary venous hypertension, and jeopardizes the repair of cardiac lesions.Methods: Four cases of congenital mitral stenosis and 4 cases of cor triatriatum (Lucas type

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The Hamman-Rich syndrome in childhood; report of a case with unilateral pulmonary arterial and venous stenosis and atriovenous occlusion

Israel DiamondChildren’s Hospital and University of Louisville School of MedicineUnited States PediatricsPediatrics 1958; 22: 279–288https://doi.org/10.1542/peds.22.2.279 AbstractThe Hamman-Rich syndrome is described in a 4-year-old Negro male. The clinical picture was that of persistent cough and progressive dyspnea beginning at 4 months of age. Diagnosis was made ante mortem by lung biopsy. The fibrotic process and arteriolosclerosis

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