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With the increased spread of severe acute respiratory syndrome coronavirus 2 infection, more patients with multisystem inflammatory syndrome in children (MIS-C) are being reported worldwide. This systematic review with meta-analysis aims to analyse the clinical features, proposed pathogenesis and current treatment options for effective management of children with this novel entity. Electronic databases (Medline, Google Scholar, WHO, CDC, UK National Health Service, LitCovid, and other databases with unpublished preprints) were extensively searched, and all articles on MIS-C published from January 1, 2020, to October 10, 2020, were retrieved. English language studies were included. This systematic review analysed 17 studies with 992 MIS-C patients from low-income and middle-income countries (LMICs) and developed countries (France, the UK, Italy, Spain, Chile and the US CDC data). Fever (95%) was the most common clinical manifestation followed by gastrointestinal (78%), cardiovascular (75.5%), and respiratory system (55.3%) involvement. Laboratory or epidemiologic evidence of inflammation and SARS-CoV-2 infection was present. Though the exact pathogenesis remains elusive, virus-induced post-infective immune dysregulation appears to play a predominant role. Features resembling Kawasaki disease, toxic shock syndrome or macrophage activation syndrome were present; 49% had shock; 32% had myocarditis; 18% had coronary vessel abnormalities and 9% had congestive cardiac failure. Sixty-three percent of the patients were admitted in paediatric intensive care unit (PICU); 63% received intravenous immunoglobulin, 58% received corticosteroids and 19% received alternate agents like tocilizumab; there were 22 (2.2%) deaths. Only 9/144 children in LMICs received tocilizumab that was significantly less than children in developed countries (p < 0.0001). This systematic review delineates and summarises recently published data on MIS-C from LMICs and developed countries. Although most needed PICU admission and received treatment with IVIG and steroids, most of the patients survived. Significantly fewer patients in developing countries received tocilizumab therapy than those in developed countries. It is crucial for clinician to recognise MIS-C, to differentiate it from other defined inflammatory conditions and initiate early treatment. Further studies are needed for long-term prognosis, especially relating to cardiac complications of MIS-C. Keywords COVID-19. Hemophagocytic lympho-histiocytosis. Kawasaki disease. SARS-CoV-2. Macrophage activation syndrome. Multisystem inflammatory syndrome in children (MIS-C). Toxic shock syndrome This article is part of the Topical Collection on Covid-19
With the increased spread of severe acute respiratory syndrome coronavirus 2 infection, more patients with multisystem inflammatory syndrome in children (MIS-C) are being reported worldwide. This systematic review with meta-analysis aims to analyse the clinical features, proposed pathogenesis and current treatment options for effective management of children with this novel entity. Electronic databases (Medline, Google Scholar, WHO, CDC, UK National Health Service, LitCovid, and other databases with unpublished preprints) were extensively searched, and all articles on MIS-C published from January 1, 2020, to October 10, 2020, were retrieved. English language studies were included. This systematic review analysed 17 studies with 992 MIS-C patients from low-income and middle-income countries (LMICs) and developed countries (France, the UK, Italy, Spain, Chile and the US CDC data). Fever (95%) was the most common clinical manifestation followed by gastrointestinal (78%), cardiovascular (75.5%), and respiratory system (55.3%) involvement. Laboratory or epidemiologic evidence of inflammation and SARS-CoV-2 infection was present. Though the exact pathogenesis remains elusive, virus-induced post-infective immune dysregulation appears to play a predominant role. Features resembling Kawasaki disease, toxic shock syndrome or macrophage activation syndrome were present; 49% had shock; 32% had myocarditis; 18% had coronary vessel abnormalities and 9% had congestive cardiac failure. Sixty-three percent of the patients were admitted in paediatric intensive care unit (PICU); 63% received intravenous immunoglobulin, 58% received corticosteroids and 19% received alternate agents like tocilizumab; there were 22 (2.2%) deaths. Only 9/144 children in LMICs received tocilizumab that was significantly less than children in developed countries (p < 0.0001). This systematic review delineates and summarises recently published data on MIS-C from LMICs and developed countries. Although most needed PICU admission and received treatment with IVIG and steroids, most of the patients survived. Significantly fewer patients in developing countries received tocilizumab therapy than those in developed countries. It is crucial for clinician to recognise MIS-C, to differentiate it from other defined inflammatory conditions and initiate early treatment. Further studies are needed for long-term prognosis, especially relating to cardiac complications of MIS-C. Keywords COVID-19. Hemophagocytic lympho-histiocytosis. Kawasaki disease. SARS-CoV-2. Macrophage activation syndrome. Multisystem inflammatory syndrome in children (MIS-C). Toxic shock syndrome This article is part of the Topical Collection on Covid-19
Pediatric inflammatory multisystem syndrome temporally associated with coronavirus disease 2019 (COVID-19) is an emerging rare disease reported in children 4 to 6 weeks after a usually asymptomatic COVID-19 infection. Though it usually presents as a Kawasaki-like illness or toxic shock syndrome, other multisystem presentations have been reported. Presentation as hemiplegia, however, is rare. Here, we describe a child with acute hemiplegia and rapidly progressive shock, who responded dramatically to steroids and intravenous immunoglobulin and experienced a full recovery. By reporting this case, we wish to add to the literature this atypical presentation of this novel disease, and highlight the importance of quickly diagnosing and treating this life-threatening disease.
BACKGROUND Several studies have reported pediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS) cases with their cardiac manifestations, but only few studies synthesize the cardiovascular characteristics in children with PIMS-TS. However, detecting cardiac abnormalities is crucial in improving patients' outcomes and reducing mortality. This review aimed to summarize the overall symptoms, laboratory, and workup findings in PIMS-TS patients, focusing on cardiovascular manifestations. METHODS We searched 4 medical databases (PubMed, Science Direct, Medline, and Scielo) and 4 preprint databases (Medrxiv, Research Square, SSRN, and Biorxiv). The literature search was done on November 8, 2021. All case reports, case series, cross-sectional studies, cohort studies, and possible clinical trials published from December 2020 onward that studied PIMS-TS on cardiac manifestation (aged 0–18 years) were included. Studies on multisystem inflammatory syndrome in children, animal studies, and studies without full-text availability were excluded. This review was registered in PROSPERO (CRD42021194468). RESULTS 59 studies were included with a total of 698 patients. The most common cardiovascular findings were the presence of cardiogenic shock (37%) and hypotension (8.5%). Almost all laboratory values were deranged. Cardiac computed tomography scan mostly showed normal results (56%), followed by cardiomegaly with pericardial effusion (14%). Electrocardiography showed normal findings (46%), ST-segment abnormalities (32%), and abnormal T wave (12%). Echocardiography findings showed left ventricle dysfunction (40.6%), which can be considered most significant, followed by pericardial effusion together with pericarditis (11.4%) and tricuspid regurgitation (6.9%). CONCLUSIONS This review found various cardiac abnormalities that may develop during PIMS-TS. Due to these findings, we should be more vigilant and not underestimate the consequences in pediatric COVID-19 patients.
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