Pediatric Pulmonary Vascular Disease

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

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Management of congenital and postoperative chylothorax: Use of thoracoscopic lymphatic leak ligations with intraoperative ICG lymphangiography

Shunya Takada, Chiyoe Sirota, Hiroo Uchida, Hizuru Amano, Akinari Hinoki, Wataru Sumida, Satoshi Makita, Masamune Okamoto, Aitaro, Takimoto, Akihiro Yasui, Youichi Nakagawa, Daiki Kato, Yaohui Guo, Takahisa TainakaNagoya University Graduate School of Medicine. Japan Journal of Pediatric SurgeryJ Pediatr Surg 2022; DOI: 10.1016/j.jpedsurg.2022.11.018 AbstractBackground: Congenital chylothorax (CCT) and postoperative chylothorax (POCT) are rare and difficult to treat.

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Treatment and outcomes of chylothorax in children: 20-year experience of a single institute

Kanokpan Ruangnapa, Wanaporn Anuntaseree, Kantara Saelim, Pharsai Prasertsan, Maneerat Puwanant, Supaporn DissanevatePrince of Songkla UniversityThailand Journal of Thoracic DiseaseJ Thorac Dis 2022; 14: 3719-3726DOI: 10.21037/jtd-22-474 AbstractBackground: Chylothorax is an uncommon cause of pleural effusion in children. This study aimed to determine the characteristics, treatment strategies, and outcomes of chylothorax in children from a single institute.Methods: The 65 episodes

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Congenital Chylothorax of the Newborn: A Systematic Analysis of Published Cases between 1990 and 2018

Bernhard Resch, Gülsen Sever Yildiz, Friedrich ReitererMedical University of GrazAustria RespirationRespiration 2022; 101: 84-96DOI: 10.1159/000518217 AbstractBackground: Congenital chylothorax (CCT) of the newborn is a rare entity but the most common cause of pleural effusion in this age-group. We aimed to find the optimal treatment strategy.Material and methods: A PubMed search was performed according to the PRISMA criteria. All

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Impaired PIEZO1 function in patients with a novel autosomal recessive congenital lymphatic dysplasia

Viktor Lukacs, Jayanti Mathur, Rong Mao, Pinar Bayrak-Toydemir, Melinda Procter, Stuart M. Cahalan, Helen J. Kim, Michael Bandell, Nicola Longo, Ronald W. Day, David A. Stevenson, Ardem Patapoutian, Bryan L. KrockThe Scripps Research Institute. Genomics Institute of the Novartis Research Foundation. ARUP Institute for Clinical and Experimental Pathology. University of Utah. Stanford University. Children’s Hospital

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Intranodal cone-beam computed tomographic lymphangiography with water-soluble iodinated contrast agent for evaluating chylothorax in infants – preliminary experience at a single institution

Dicken Wong, Kin Fen Kevin Fung, Hay-Son Robin Chen, Kin Sing Lun, Yee Ling Elaine KanKwong Wah Hospital. Hong Kong Children’s Hospital. Hong Kong Pediatric RadiologyPediatr Radiol 2023; 53: 179-183DOI: 10.1007/s00247-022-05441-4 AbstractThis brief report demonstrates the diagnostic utility of cone-beam CT lymphangiography (CBCTL) with intranodal injection of water-soluble iodinated contrast agent for assessing lymphatic disorders in

Intranodal cone-beam computed tomographic lymphangiography with water-soluble iodinated contrast agent for evaluating chylothorax in infants – preliminary experience at a single institution Read More »

Investigation into the genetics of fetal congenital lymphatic anomalies

Daniella Rogerson, Anna Alkelai, Jessica Giordano, Madhulatha Pantrangi, Meng-Chang Hsiao, Chia-Ling Nhan-Chang, Joshua E. Motelow, Vimla Aggarwal, David Goldstein, Ron Wapner, Carrie J. ShawberColumbia University Vagelos College of Physicians and Surgeons. Columbia University Irving Medical Center.United States Prenatal DiagnosisPrenat Diagn 2023; DOI: 10.1002/pd.6345 AbstractObjective: Congenital lymphatic anomalies (LAs) arise due to defects in lymphatic development and often

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