Results 21 to 30 of about 1,120 (144)

Human Lymphatic Endothelial Cells Expressing NRAS^Q61R Model Characteristics of Kaposiform Lymphangiomatosis. [PDF]

Pediatr Blood Cancer
ABSTRACTKaposiform lymphangiomatosis (KLA) is a complex lymphatic anomaly characterized by lymphatic overgrowth and dysfunction. The goal of this study was to determine how the NRASQ61R variant found in the tissue of KLA patients impacts key characteristics of lymphatic endothelial cells (LEC).
Fox DA, McDaniel CG, Pastura P, Le Cras TD.   +3 more
europepmc   +5 more sources

Significant improvement of a nevus spilus‐type congenital melanocytic nevus with oral selumetinib [PDF]

Pediatric Dermatology, Volume 41, Issue 5, Page 936-937, September/October 2024.
Abstract Giant congenital melanocytic nevi (GCMN) can be cosmetically significant and can lead to melanoma. There is no standard pharmacologic treatment for GCMN. We present the case of an 8‐year‐old female with kaposiform lymphangiomatosis caused by an NRAS mutation whose nevus spilus‐type GCMN improved on oral selumetinib.
R. Berna, T. Hasbun, D. Adams, A. T. Moon, J. R. Treat   +4 more
wiley   +2 more sources

Intestinal failure in kaposiform hemangioendothelioma successfully resolved with medical therapy and nearly fat-free oral feeding: A novel case report [PDF]

Intestinal Failure
Background: Kaposiform hemangioendothelioma and kaposiform lymphangiomatosis (KHE/KLA) are rare, non-malignant lymphovascular anomalies often complicated by Kasabach-Merritt phenomenon (KMP). Morbidity and mortality are high.
Tierra L. Mosher, Eduardo J. Contijoch, Jordan Bui, Joyce Teng, Michael R. Jeng, Shweta S. Namjoshi   +5 more
doaj   +2 more sources

Case Report: Life-threatening Kasabach–Merritt phenomenon in a 2-month-old child [PDF]

Frontiers in Pediatrics
We present a rare case of a 2-month-old girl, born at term, who was admitted to the pediatric intensive care unit for fussiness, increasing abdominal distension, and intermittent diarrhea for one week. She was found to be hypertensive, coagulopathic, and
Ashley V. Wong Grossman, Juan Pablo Forero, Jeffrey S. Yu, Megan M. Gilbert, Kanwaljeet J. S. Anand   +4 more
doaj   +2 more sources

Kaposiform Lymphangiomatosis: Pathologic Aspects in 43 Patients. [PDF]

Am J Surg Pathol, 2022
Kaposiform lymphangiomatosis is an uncommon generalized lymphatic anomaly with distinctive clinical, radiologic, histopathologic, and molecular findings. Herein, we document the pathology in 43 patients evaluated by the Boston Children’s Hospital Vascular Anomalies Center from 1999 to 2020.
Perez-Atayde AR, Debelenko L, Al-Ibraheemi A, Eng W, Ruiz-Gutierrez M, O'Hare M, Croteau SE, Trenor CC, Boyer D, Balkin DM, Barclay SF, Hsi Dickie B, Liang MG, Chaudry G, Alomari AI, Mulliken JB, Adams DM, Kurek KC, Fishman SJ, Kozakewich HPW.   +19 more
europepmc   +3 more sources

Lymphatic malformations involving the thorax in children: a retrospective cohort study [PDF]

BMC Pulmonary Medicine
Background Lymphatic malformations (LMs) involving the thorax are rare, with limited clinical understanding. We aimed to summarize the classification, clinical features, treatment, and prognosis of thoracic LMs, and to improve disease management and ...
Wenting Han, Haiming Yang, Jinrong Liu, Huimin Li, Hui Xu, Hui Liu, Xiaolei Tang, Shunying Zhao   +7 more
doaj   +2 more sources

Comment on: Potential biomarkers of kaposiform lymphangiomatosis. [PDF]

Pediatr Blood Cancer, 2020
Le Cras TD, Adams DM.
europepmc   +4 more sources

Treatment of severe Kaposiform lymphangiomatosis positive for NRAS mutation by MEK inhibition. [PDF]

Pediatr Res, 2023
Kaposiform lymphangiomatosis (KLA) is a complex lymphatic anomaly involving most commonly the mediastinum, lung, skin and bones with few effective treatments. In recent years, RAS-MAPK pathway mutations were shown to underlie the pathogenesis of several complex lymphatic anomalies.
Chowers G, Abebe-Campino G, Golan H, Vivante A, Greenberger S, Soudack M, Barkai G, Fox-Fisher I, Li D, March M, Battig MR, Hakonarson H, Adams D, Dori Y, Dagan A.   +14 more
europepmc   +3 more sources

Recurrent haemoptysis: a rare diagnosis of kaposiform lymphangiomatosis and review of literature. [PDF]

BMJ Case Rep, 2022
Kaposiform lymphangiomatosis (KLA) is a rare clinicopathological entity among lymphatic anomalies. The main involved sites are the mediastinum and the lungs but the disease can also affect multiple extrathoracic organs. Little is known about the pathophysiology, the natural history, the treatment response and the long-term outcome of this disorder. KLA
Fedidat R, Elia A, Hershko A, Fridlender Z.   +3 more
europepmc   +3 more sources

A somatic activating NRAS variant associated with kaposiform lymphangiomatosis. [PDF]

Genet Med, 2019
Kaposiform lymphangiomatosis (KLA) is a rare, frequently aggressive, systemic disorder of the lymphatic vasculature, occurring primarily in children. Even with multimodal treatments, KLA has a poor prognosis and high mortality rate secondary to coagulopathy, effusions, and systemic involvement. We hypothesized that, as has recently been found for other
Barclay SF, Inman KW, Luks VL, McIntyre JB, Al-Ibraheemi A, Church AJ, Perez-Atayde AR, Mangray S, Jeng M, Kreimer SR, Walker L, Fishman SJ, Alomari AI, Chaudry G, Trenor Iii CC, Adams D, Kozakewich HPW, Kurek KC.   +17 more
europepmc   +4 more sources