Results 11 to 20 of about 1,120 (144)
Resolution of Refractory Chylous Effusions With Targeted MEK Inhibition in NRAS Q61R-Driven Kaposiform Lymphangiomatosis: A Case Report [PDF]
Case Reports in PediatricsIntroduction Kaposiform lymphangiomatosis (KLA) is a rare and aggressive lymphatic anomaly characterised by chylous effusions, respiratory failure and poor prognosis. Diagnosis is often challenging and delayed due to the mosaic distribution of causative somatic mutations. Case Presentation A previously well four‐year‐old girl presented with pleural and
Georgia Brown +16 more
doaj +4 more sources
Kaposiform Lymphangiomatosis: Diagnosis, Pathogenesis, and Treatment [PDF]
Pediatric Blood and Cancer, 2023 AbstractKaposiform lymphangiomatosis (KLA) is a life‐threatening rare disease that can cause substantial morbidity, mortality, and social burdens for patients and their families. Diagnosis often occurs long after initial symptoms, and there are few centers in the world with the expertise to diagnose and care for patients with the disease.
Timothy D Le Cras, Denise M Adams
exaly +4 more sources
Vascular Health and Risk Management, 2008 Claudia S Vetter-Kauczok1, Philipp Ströbel2, Eva B Bröcker1, Jürgen C Becker11Department of Dermatology, Julius-Maximilians-University Wuerzburg, Wuerzburg, Germany; 2Department of Pathology, Ruprecht-Karls-University Mannheim, Mannheim ...
Claudia S Vetter-Kauczok +3 more
exaly +3 more sources
Detection of NRAS Mutation in Kaposiform Lymphangiomatosis Using Cell-free DNA from Plasma
Journal of Vascular Anomalies, 2023 Kaposiform lymphangiomatosis is a rare lymphatic anomaly that occurs in children and is associated with life-threatening complications. The somatic mutation NRAS Q61R (c.182A>G) has been identified in lesional tissue samples of patients with kaposiform ...
Fiona Lynch +2 more
doaj +2 more sources
Kaposiform lymphangiomatosis presenting with a Group A Streptococcus pericardial effusion. [PDF]
BMJ Case Rep, 2022 A 4-year-old child was transferred to the paediatric intensive care unit with acute respiratory failure following 4 days of fever, nausea and vomiting. Chest X-ray on admission had an enlarged cardiac silhouette and transthoracic echo confirmed a large pericardial effusion.
Koerner T +3 more
europepmc +6 more sources
Pediatric Diffuse Pulmonary Lymphangiomatosis: CT Characteristics
CT Lilun yu yingyong yanjiu, 2022 Objective: To explore the typical CT features of diffuse pulmonary lymphangiomatosis in children and improve the diagnostic accuracy. Methods: The radiologic data of 13 children with diffuse pulmonary lymphangiomatosis confirmed by clinical, imaging and ...
Jun GAO +4 more
doaj +2 more sources
Kaposiform Lymphangiomatosis: A Distinct Aggressive Lymphatic Anomaly [PDF]
Journal of Pediatrics, 2014 To describe the clinical and imaging characteristics of a new lymphatic disorder with a unique histological pattern and poor prognosis.An observational, retrospective study identified and characterized 20 patients with distinct lymphatic histopathology referred to the Vascular Anomalies Center at Boston Children's Hospital between 1995 and 2011.The ...
Stacy E Croteau +2 more
exaly +5 more sources
Cerebrospinal fluid-lymphatic fistula causing spontaneous intracranial hypotension in a child with kaposiform lymphangiomatosis. [PDF]
Pediatr Radiol, 2021 Spontaneous intracranial hypotension is an uncommon etiology of secondary headaches in children. We report a unique case of a girl with kaposiform lymphangiomatosis who developed postural headaches and imaging features of spontaneous intracranial hypotension without a spinal extradural collection.
Soderlund KA +4 more
europepmc +8 more sources
Primary Splenic Involvement in Kaposiform Lymphangiomatosis: A Case Report [PDF]
International Journal of Surgery Case ReportsABSTRACT Introduction: Kaposiform Lymphangiomatosis (KLA) is a rare, pathologically distinct lymphatic anomaly characterized by abnormal lymphatic channel malformation and a high mortality rate. Previous studies describe primary thoracic involvement.
Covil E, Gates R.
exaly +4 more sources
Trametinib normalizes angiopoietin-2 levels and successfully treats kaposiform lymphangiomatosis. [PDF]
J Vasc Anom (Phila)Kaposiform lymphangiomatosis (KLA) is a complex lymphatic anomaly with high morbidity and mortality. Angiopoietin-2 (Ang-2) levels are elevated in the blood of patients with KLA and can be used to aid in diagnosis and monitor treatment response.
McDaniel CG +6 more
europepmc +4 more sources