Filed (PHiled). Greater than 1-2 years since publication

Sirolimus efficacy in the treatment of critically ill infants with congenital primary chylous effusions

Shreva Agarwal, Berkley Kingman Anderson, Priya Mahajan, Caraciolo J. Fernandes, Judith F. Margolin, Ionela IacobasTexas Children’s Hospital and Baylor College of Medicine.United States Pediatric Blood and CancerPediatr Blood Cancer 2022; 69: DOI: 10.1002/pbc.29510 AbstractBackground: Chylothorax can be a presenting symptom of complex lymphatic anomaly in children and is associated with significant respiratory morbidity. Historically, the traditional pharmacological […]

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Intranodal lipiodol injection for the treatment of chyle leak in children – a preliminary experience

Amit Gupta, Priyanka Naranje, Zainab Vora, Raju Sharma, Manisha Jana, Ashu Seith Bhalla, Pallavi Sinha, Rohan Malik, Prabudh Goel, Anjan Dhua, Devasenathipathy KandasamyAll India Institute of Medical SciencesIndia British Journal of RadiologyBr J Radiol 2022; 95: DOI: 10.1259/bjr.20211270 AbstractObjective: To assess the effectiveness of intranodal lymphangiography using ethiodised oil (Lipiodol; Guerbet Japan, Tokyo, Japan) for the treatment

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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,

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

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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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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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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Neonatal lymphatic flow disorders: central lymphatic flow disorder and isolated chylothorax, diagnosis and treatment using novel lymphatic imaging and interventions technique

Deborah Rabinowitz, Kevin Dysart, Maxim ItkinNemours Children’s Hospital. Sidney Kimmel Medical College at Thomas Jefferson University. Perelman School of Medicine at the University of Pennsylvania. United States Current Opinion in PediatricsCurr Opin Pediatr 2022; 34: 191-196DOI: 10.1097/MOP.0000000000001109 AbstractPurpose of review: Neonatal lymphatic disorders (NLDs) are conditions that are relatively rare and difficult to treat. The recent development

Neonatal lymphatic flow disorders: central lymphatic flow disorder and isolated chylothorax, diagnosis and treatment using novel lymphatic imaging and interventions technique Read More »

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