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Lyve1-Driven Nras Causes Edema, Enlarged Lymphatic Vessels, and Hepatic Vascular Defects in Embryonic Mice.
Pediatr Blood Cancer
December 2024
Division of Pulmonary Biology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
Background: Kaposiform lymphangiomatosis (KLA) is a complex lymphatic anomaly associated with a somatic activating NRAS p.Q61R (NRAS) mutation. KLA is characterized by malformed lymphatic vessels that can lead to effusions and coagulopathy.
View Article and Find Full Text PDFCase report of solitary giant hepatic lymphangioma.
Korean J Hepatobiliary Pancreat Surg
May 2016
Department of Radiology, Gimpo Woori Hospital, Gimpo, Korea.
A hepatic lymphangioma is a rare benign neoplasm that is usually associated with systemic lymphangiomatosis. A solitary hepatic lymphangioma is extremely rare. Therefore, we present a rare case of a female patient who underwent right hepatectomy for solitary giant hepatic lymphangioma.
View Article and Find Full Text PDFHepatobiliary and pancreatic: liver transplantation for massive hepatic lymphangiomatosis.
J Gastroenterol Hepatol
February 2014
Department of Paediatrics, Toowoomba Base Hospital, Brisbane, Queensland, Australia.
[A case of isolated hepatic lymphangioma].
Korean J Gastroenterol
February 2012
Department of Internal Medicine, Maryknoll Medical Center, Busan, Korea.
Hepatic lymphangioma is a rare benign neoplasm. It usually occurs as a part of systemic lymphangiomatosis. Isolated hepatic lymphangioma is extremely rare.
View Article and Find Full Text PDFRenal lymphangiomatosis, interrupted IVC with persistent primitive hepatic venous plexus and multiple anomalous venous channels: parts of an overlap syndrome?
Pediatr Radiol
February 2012
Clarendon Wing Radiology Department, Leeds General Infirmary, Leeds, LS2 9NS, UK.
A 5-year-old girl with cutis marmorata telangiectasia congenita (CMTC) and congenital glaucoma, who had previously presented with seizures, transient hemiplegia, upper gastrointestinal bleeding and hemihypertrophy, developed a large pleural effusion. Subsequent imaging revealed renal lymphangiomatosis, multiple anomalous intra-abdominal venous channels, an interrupted inferior vena cava with a persistent primitive hepatic venous plexus (PPHVP) and meningeal angiomas. To the best of our knowledge, the CT findings of PPHVP and the combination of the demonstrated abnormalities have not been previously reported.
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