伴有卡萨巴赫-梅里特现象的卡波西样血管内皮瘤新生儿：6例报告
NEWBORNS WITH KASABACH-MERRITT PHENOMENON-ASSOCIATED KAPOSIFORM HEMANGIOENDOTHELIOMA: A REPORT OF 6 CASES.
作者信息
Benzar Iryna M, Rebenkov Stanislav O, Levytskyi Anatolii F, Fedoniuk Larysa Ya, Fomina Lyudmyla V, Sas Lesia M, Stravskyy Taras Ya
机构信息
BOGOMOLETS NATIONAL MEDICAL UNIVERSITY, KYIV, UKRAINE.
NATIONAL SPECIALIZED CHILDREN HOSPITAL OHMATDYT, KYIV, UKRAINE.
出版信息
Wiad Lek. 2020;73(8):1785-1789.
Combination of kaposiform hemangioendothelioma (KHE) and Kasabach-Merritt phenomenon (KMP) in newborn children is a life-threatening constellation. The purpose of the study is the choice of the diagnostic and treatment methods in these patients and evaluating the effectiveness of treatment using radiological methods of investigation. The study enrolled 6 newborn patients with KHE within a period 2013 - 2018. MRI (CT) performed to make the diagnosis and evaluate treatment response. Hypervascular mass accompanied by reticular lymphedema, hyper intensive in T2 WI; isointensive in T1 WI, intense contrast enhancement, heterogeneous diffusion restriction were unique MRI characteristics of KHE. The sustained remission was achieved with treatment by propranolol (n=2), vincristine (n=1), and their combination (n=3).
新生儿卡波西样血管内皮瘤（KHE）合并卡萨巴赫-梅里特现象（KMP）是一种危及生命的综合征。本研究的目的是选择这些患者的诊断和治疗方法，并使用放射学检查方法评估治疗效果。该研究在2013年至2018年期间纳入了6例患有KHE的新生儿患者。通过磁共振成像（MRI）（计算机断层扫描[CT]）进行诊断并评估治疗反应。伴有网状淋巴水肿的高血管性肿块，在T2加权成像（T2 WI）中呈高信号强度；在T1加权成像（T1 WI）中呈等信号强度，强化明显，弥散受限不均一是KHE独特的MRI特征。通过普萘洛尔（n = 2）、长春新碱（n = 1）及其联合用药（n = 3）治疗实现了持续缓解。
Zotero 插件