Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing

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 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 »

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 »

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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Prediction Model with External Validation for Early Detection of Postoperative Pediatric Chylothorax

Rohin Moza, Melissa Winder, Gregory T. Adamson, Zhining Ou, Angela P. Presson, Senthuran Vijayarajah, Stephanie A. Goldstein, David K. BaillyPrimary Children’s Hospital and University of Utah.United States Pediatric CardiologyPediatr Cardiol 2023; DOI: 10.1007/s00246-022-03034-4 AbstractEarlier diagnosis of chylothorax following pediatric cardiac surgery is associated with decreased duration of chylothorax. Pleural fluid testing is used to diagnosis chylothorax

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Pearls & Oy-sters: Cerebral Abscess Secondary to Pulmonary Arteriovenous Malformation in Hereditary Hemorrhagic Telangiectasia

Jodie I. Roberts, Kristine Woodward, Adam Kirton, Michael J. EsserUniversity of Calgary; Alberta Children’s Hospital Research Institute.Canada NeurologyNeurology 2022; 98: 292-295DOI: 10.1212/WNL.0000000000013181 AbstractHereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant condition that is linked to a myriad of neurologic complications arising from vascular malformations of the brain, spinal cord, and lungs. Our case describes a previously

Pearls & Oy-sters: Cerebral Abscess Secondary to Pulmonary Arteriovenous Malformation in Hereditary Hemorrhagic Telangiectasia Read More »

Hypoxia and Polycythemia: A Pediatric Emergency Department Point of Care Ultrasound Diagnosis of Pulmonary Arteriovenous Malformation

Shachar Oren, Shachar, Galit Brenner, Yael Garty, Eric ScheierKaplan Medical Center. Hebrew University of Jerusalem.Israel Pediatric Emergency CarePediatr Emerg Car 2022; 38: 633-635DOI: 10.1097/PEC.0000000000002721 AbstractPoint of care ultrasound (POCUS) can be useful in the differentiation between cardiac and pulmonary etiologies of hypoxia. Here, we present a child with signs of chronic hypoxia, found on POCUS to

Hypoxia and Polycythemia: A Pediatric Emergency Department Point of Care Ultrasound Diagnosis of Pulmonary Arteriovenous Malformation Read More »

Cystic fibrosis with multiple pulmonary arteriovenous malformations: A case report

Orito Ikeda, Kunihiko Shimizu, Yoshitake Yamada, Hiroaki Sugiura, Hideaki Suzuki, Syuichiro Umetsu, Kozo Sato, Masahiro JinzakiSaiseikai Yokohama-shi Tobu Hospital. Keio University School of Medicine. National Defense Medical College Hospital. Japan Radiology Case ReportsRadiol Case Rep 2023; 18: 1033-1036DOI: 10.1016/j.radcr.2022.12.024 AbstractCystic fibrosis is an autosomal recessive genetic disorder that damages the exocrine function of the body, resulting

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