肝细胞衰竭揭示免疫功能低下儿童骨肉瘤与sars - cov -2相关的多系统炎症综合征

Ryouni K, Chahid I, Atrassi M, Bensabbahia D, Abkari A
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摘要

儿童多系统炎症综合征(multi - system Inflammatory Syndrome in Children, MIS-C)是全球范围内报道的一种新现象,与COVID-19存在时间相关性。目的:我们的病例报告的目的是澄清皮姆斯综合征在骨肉瘤领域的表现,这可能与转移混淆。患者和方法:我们报告一例12岁的男性右肱骨骨肉瘤患者,因肝细胞衰竭合并皮肤黏膜黄疸显示PIMS综合征而入院。结果:患者最初接受3个疗程的化疗,随后进行肿瘤整体切除和骨整合,符合PIMS-TS的病例定义,表现为发热,多器官和皮肤粘膜受累,心血管和胃肠道症状。他的生物学检查显示明显的炎症综合征,铁蛋白水平非常高,d -二聚体和bnp前水平高,伴有淋巴细胞减少和肝细胞溶解。超声心动图显示扩张性心肌病,LVEF为13%,伴有中度心包积液。当血清学结果为阳性确认PIMS-TS时,通过PCR检测完成了检查,结果为阴性。在治疗上,他接受了一个疗程的免疫球蛋白、皮质类固醇,鉴于心肌损伤,在血压恢复正常后,他又服用了阿司匹林、ACE抑制剂、受体阻滞剂和螺内酯。进化是惊人的,以明显的临床改善为标志,生物参数正常化,超声心动图显示他的LVEF改善。结论:虽然发病机制尚不清楚,但可能与免疫介导的损伤有关。我们在此提供有关这种情况的最新信息,以提高儿科医生的认识。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
SARS-CoV-2-Related Multisystem Inflammatory Syndrome in an Immunocompromised Child with Osteosarcoma Revealed by Hepatocellular Failure
Introduction: Multisystem Inflammatory Syndrome in Children (MIS-C) is a new phenomenon reported worldwide with temporal association with COVID-19. Objective: The aim of our case report is to clarify the manifestations of a pims syndrome in a osteosarcoma field, which can be confused with metastases. Patients and methods: We report a case of a 12-year-old male patient followed for osteosarcoma of the right humerus who was admitted for hepatocellular failure with cutaneous-mucosal jaundice revealing a PIMS syndrome. Results: The patient had initially received 3 courses of chemotherapy, followed by monobloc resection of the tumour mass and osteosynthesis, who met the case definition of PIMS-TS presented with fever, multi organ and mucocutaneous involvement, cardiovascular and gastrointestinal symptoms. His biological work-up showed a significant inflammatory syndrome with very high ferritin levels, high D-dimer and pro-BNP levels, associated with lymphopenia and liver cytolysis. An echocardiogram revealed a dilated cardiomyopathy with an LVEF of 13% associated with a moderate pericardial effusion. The work-up was completed by a PCR covid which came back negative, when the serology came back positive confirming a PIMS-TS. Therapeutically, he received a course of immunoglobulin, corticosteroid, and in view of the myocardial damage, he was put on Aspirin, ACE inhibitor, beta-blocker and spironolactone after normalization of his blood pressure. The evolution was spectacular, marked by a clear clinical improvement, the normalisation of biological parameters and the improvement of his LVEF on echocardiography. Conclusion: Although the pathogenesis is not clearly known, immune-mediated injury has been implicated. We herein provide current information on this condition, in order to raise awareness amongst pediatricians.
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