Results 61 to 70 of about 1,455,846 (152)

Transcriptomic Profiling Unveils EDN3+ Meningeal Fibroblasts as Key Players in Sturge‐Weber Syndrome Pathogenesis

Advanced Science, Volume 12, Issue 17, May 8, 2025.
Sturge‐Weber syndrome (SWS) is characterized by leptomeningeal vascular malformations, leading to seizures and stroke. Analysis of 119 446 brain cells from SWS patients uncovered distinct cell heterogeneity and identified an EDN3⁺ meningeal fibroblast cluster, with WNT5A emerging as a potential key driver of SWS progression and a promising therapeutic ...
Daosheng Ai, Tianyue Ming, Xiaoli Li, Shu Wang, Zhanying Bi, Jinyi Zuo, Zizhang Cheng, Weijin Sun, Mingguo Xie, Fengzhi Li, Xiongfei Wang, Xueling Qi, Guoming Luan, Woo‐ping Ge, Yuguang Guan   +14 more
wiley   +1 more source

Image_3_Case report: Experience of a rare case of rebound of the Kasabach-Merritt phenomenon during sirolimus treatment in kaposiform hemangioendothelioma.pdf

, 2022
Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm associated with the Kasabach–Merritt phenomenon (KMP), which is a consumptive coagulopathy with associated potentially life-threatening thrombocytopenia.
Liang Wang (23021), Zhuang Liu (551357), Lei Guo (36221), Jing Li (10611), Yanli Niu (4165657), Changhua Wu (13202715), Dan Song (644313), Jie Zhou (28945)   +7 more
core   +1 more source

Advancing Precision Medicine: The Role of Genetic Testing and Sequencing Technologies in Identifying Biological Markers for Rare Cancers

Cancer Medicine, Volume 14, Issue 8, April 2025.
ABSTRACT Background Genetic testing and sequencing technologies offer a comprehensive understanding of cancer genetics, providing rapid and cost‐effective solutions. In particular, these advanced technologies play an important role in assessing the complexities of the rare cancer types affecting several systems including the bone, endocrine, digestive,
Joviana Farhat, Lara Alzyoud, Mohammad AlWahsh, Animesh Acharjee, Basem Al‐Omari   +4 more
wiley   +1 more source

Kasabach–Merritt Syndrome, an underdiagnosed swollen leg in newborn with anemia and jaundice

, 2023
Key Clinical Message Kasabach–Merritt syndrome is a rare disease. Early recognition, timely transfer, and proper management are crucial in reducing bleeding complication and mortality.
Chariya Chap, Sakviseth Bin
core   +1 more source

Kasabach Merritt Syndrome

, 2018
Kasabach-Merritt phenomenon (KMP), first described in 1940, is a rare but life-threatening coagulopathy of infancy which presents with thrombocytopenia, microangiopathic hemolytic anemia, and consumptive coagulopathy in the setting of a rapidly enlarging
Vaidya, Ruben, MD, Lewis, Deirdre, MD
core   +1 more source

Sirolimus treatment for intractable vascular anomalies (SIVA): An open‐label, single‐arm, multicenter, prospective trial

Pediatrics International, Volume 67, Issue 1, January/December 2025.
Abstract Background Intractable vascular anomalies (VAs), including vascular tumors and venous, lymphatic, and mixed malformations, often have severe symptoms and a poor prognosis, highlighting the need for new treatments. We conducted a prospective trial of sirolimus (tablet and granule forms) for the treatment of VAs.
Michio Ozeki, Saori Endo, Shiho Yasue, Ryuta Asada, Akiko M. Saito, Hiroya Hashimoto, Shigeru Ueno, Shoji Watanabe, Motoi Kato, Kyoichi Deie, Shunsuke Nosaka, Mikiko Miyasaka, Akihiro Umezawa, Kentaro Matsuoka, Mototoshi Kato, Tatsuo Kuroda, Takanobu Maekawa, Satoshi Hirakawa, Taizo Furukawa, Shigehisa Fumino, Tatsuro Tajiri, Junkichi Takemoto, Naonori Kawakubo, Akihiro Fujino   +23 more
wiley   +1 more source

Kaposiform Hemangioendothelioma of the Mediastinum With Kasabach-Merritt Phenomenon Presenting With Pericardial Effusion in a 2-Month-Old

Journal of Vascular Anomalies
Kaposiform hemangioendothelioma (KHE) is a rare vascular and lymphatic tumor of childhood that commonly presents on the skin and extremities. KHE of the mediastinum affecting the heart and great vessels is extremely rare and often locally aggressive.
Olivia A. Keane, Carolyn Taylor, Shayla Bergmann   +2 more
doaj   +1 more source

Sirolimus for Pediatric Cervicofacial Lymphatic Malformation: A Systematic Review and Meta‐Analysis

The Laryngoscope, Volume 134, Issue 5, Page 2038-2047, May 2024.
Objective This study is a systematic review and meta‐analysis of the efficacy and safety of sirolimus in the management of pediatric cervicofacial lymphatic malformations (LMs). Data Sources EMBASE, Medline, Scopus, and Cochrane databases were searched, along with the reference list of all included articles.
Yasmine Kamhieh, Robin Mitra, Thomas Burnett, Hugh Jones, Graham Roblin, Andrew Hall   +5 more
wiley   +1 more source

Diagnosis of Kasabach-Merritt phenomenon in a newborn

Kasabach-Merritt Phenomenon (KMP) is a rare and potentially life-threatening coagulopathy that has high bleeding risk and is characterized by severe thrombocytopenia and hypofibrinogenemia.
Basala, Sara, Raykhelson, Edward, McGuffin, Aaron, Ricci, Kiersten   +3 more
core   +1 more source

Case report: Subutaneous hemangiomatosis causing Kasabach-Merritt syndrome - MRI features

, 2008
Hemangiomatosis is an uncommon entity in which there is diffuse infiltration of soft tissue or bone by hemangioma. Kasabach-Merritt syndrome is an uncommon complication of large hemangiomas, in which there is thrombocytopenia and coagulopathy.
Tarun P Jain, Raju Sharma, Rohini Gupta
core   +1 more source