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Multisystem inflammatory syndrome in children is a new childhood inflammatory disorder associated with respiratory syndrome coronavirus 2 (SARS-CoV-2). This illness of elevated inflammatory markers and multiple organ involvement similar to Kawasaki disease is not commonly reported from Asia. A 17-month-old boy presented with acute onset fever, rash, non-exudative conjunctivitis and swellings of hands and legs. In x-ray chest there was infiltration on the right lower lobe and echocardiography showed evidence of coronary arteritis. The diagnosis of multisystem inflammatory syndrome in children was confirmed on the basis of characteristic clinical features and laboratory parameters fulfilling standard case definition for multisystem inflammatory syndrome in children. The child responded to treatment with intravenous immunoglobulin and high dose aspirin. Hence, amidst SARS-CoV-2 pandemic, multisystem inflammatory syndrome in children should be suspected and effectively treated even in a country like Nepal.Keywords: Kawasaki disease; multiple inflammatory syndrome in children; Nepal; respiratory syndrome coronavirus 2

ABSTRACT This study describes epidemiological aspects of the Multisystemic Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 and mortality by COVID-19 in children (0-9 years old) and adolescents (10-19 years old). The data sources, for 2020-2021, were the Epidemiological Surveillance System for MIS-C and Mortality Information System for COVID-19, both managed by the Ministry of Health. There were 1,503 cases, more frequent in children (77%) than in adolescents (23%), and 93 reported deaths due to MIS-C in 26 of the 27 States of the Country. The highest number of cases in children was reported in São Paulo (268), but the highest incidence took place in the Federal District (7.8 per 100,000 inhabitants). The rate of deaths due to MIS-C was 5.4% in children and 8.7% in adolescents. There were 2,329 deaths due to COVID-19 in the population under 20 years old, with a higher rate in adolescents (4.4 per 100,000 inhabitants) than in children (2.7); the highest rate occurred in Roraima. We recommend intensifying immunization against COVID-19 in such population, increasing protection against the negative effects of COVID-19 and MIS-C, which may have short, medium and/or long-term consequences, so as not to compromise the full integration of these citizens into society.

Ewa WiÄ™sik-Szewczyk,1 Anna Felis-Giemza,2 MirosÅ'aw Dziuk,3 Karina Jahnz-Różyk1 1Department of Internal Medicine, Pulmonology, Allergy and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine in Warsaw, Warsaw, Poland; 2Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland; 3Department of Nuclear Medicine, Military Institute of Medicine in Warsaw, Warsaw, PolandCorrespondence: Ewa WiÄ™sik-SzewczykDepartment of Internal Medicine, Pulmonology, Allergy and Clinical Immunology, Military Institute of Medicine in Warsaw, Szaserów 128 01-141, Warsaw, PolandTel/Fax +48 261 816 581Email ewa.w.szewczyk@gmail.comAbstract: A 32-year-old-man, with a history of chronic urticaria from the age of 27, diagnosed with an adult-onset Still's disease and received a low dose of glucocorticoids, methotrexate and tocilizumab. Despite the long-term combined treatments, he suffered from chronic urticaria, low-grade fever and bone pain. He was found to have high inflammatory markers, hypogammaglobulinemia, monoclonal IgM – kappa light chain in serum and increased radiotracer uptake in the whole bone scintigraphy. No pathological variants for monogenic autoinflammatory diseases were present in the genome exome sequencing. These investigations confirmed the diagnosis of Schnitzler syndrome, which is an exception before the age of 35. Switching from tocilizumab to interleukin 1 receptor inhibitor, anakinra led to a full clinical response and normalisation of inflammatory markers. Patients with a history of fever and chronic urticaria are routinely tested for monoclonal gammopathy in the context of malignancy, but it should also be considered as a sign of the autoinflammatory syndrome. The Schnitzler syndrome and the adult-onset Still's disease share common features, so the diagnosis requires a thorough investigation to establish an optimal treatment. In the diagnostic algorithm, monoclonal gammopathy is usually considered red flag for malignancy but might be overlooked as a criterion of Schnitzler syndrome, particularly in young adults. We confirm that the interleukin 1 inhibitor should be the first line of therapy in Schnitzler syndrome, and in the presented case we found it more effective than the interleukin 6 blockade. The main goal of this paper is to increase awareness of Schnitzler syndrome among health care professionals. We aim to present features which can be helpful in differential diagnosis.Keywords: chronic urticaria, monoclonal gammopathy, tocilizumab, anakinra, autoinflammation

Paradoxical tuberculosis-associated immune reconstitution inflammatory syndrome (TB-IRIS) is an aberrant inflammatory response occurring in a subset of TB-HIV co-infected patients initiating anti-retroviral therapy (ART). Here, we examined monocyte activation by prospectively quantitating pro-inflammatory plasma markers and monocyte subsets in TB-HIV coinfected patients from a South Indian cohort at baseline and following ART initiation at the time of IRIS, or at equivalent time points in non-IRIS controls. Pro-inflammatory biomarkers of innate and myeloid cell activation were increased in plasma of IRIS patients pre-ART and at the time of IRIS; this association was confirmed in a second cohort in South Africa. Increased expression of these markers correlated with elevated antigen load as measured by higher sputum culture grade and shorter duration of anti-TB therapy. Phenotypic analysis revealed the frequency of CD14++CD162 monocytes was an independent predictor of TB-IRIS, and was closely associated with plasma levels of CRP, TNF, IL-6 and tissue factor during IRIS. In addition, production of inflammatory cytokines by monocytes was higher in IRIS patients compared to controls pre- ART. These data point to a major role of mycobacterial antigen load and myeloid cell hyperactivation in the pathogenesis of TB-IRIS, and implicate monocytes and monocyte-derived cytokines as potential targets for TB-IRIS prevention or treatment. ; European Union (PIRSES-GA-2011-295214) ; Medical Research Council (U1175.02.002.00014.01) ; Wellcome Trust (084323, 088316) ; National Research Foundation (NRF) of South Africa (UID: 85858). ; Wellcome Trust fellowship (098316) ; South African Department of Science and Technology co-funding (DST/CON 0257/2012) ; EDCTP Strategic Primer Grant (SP.2011.41304.074) ; National Institute of Allergy and Infectious Diseases grant (U01AI089244)

Background: Children comprise only 1–5% of COVID-19 cases. Recent studies have shown that COVID-19 associated multisystem inflammatory syndrome in children (MIS-C) can present with neurological signs and symptoms. In this systematic review and meta-analysis, we have reviewed neurological involvement in these patients. Methods: A comprehensive electronic literature search was done on PubMed, Google Scholar, Embase, Cochrane database, and SCOPUS for the published English language articles from December 1, 2019, to February 28, 2021. A meta-analysis of the proportion was expressed as a pooled proportion with a 95% confidence interval (CI). Representative forest plots showing individual studies and the combined effect size were generated to provide an overview of the results.Results: This systematic review and meta-analysis analyzed 15 published MIS-C studies with a total of 785 patients. Neurological manifestations in patients with MIS-C was found in 27.1%. We found that 27% developed headaches, 17.1% developed meningism/meningitis and 7.6 % developed encephalopathy. Other uncommon neurological manifestations of MIS-C includes anosmia, seizures, cerebellar ataxia, global proximal muscle weakness and bulbar palsy. In MIS-C patients with neurological feature, neuroimaging showed signal changes in the splenium of the corpus callosum. Electroencephalography showed slow wave pattern and nerve conduction studies and electromyography showed mild myopathic and neuropathic changes. Conclusions: Our study revealed that neurological manifestations are not uncommon in patients with MIS-C. Further large prospective studies are needed to better explore the disease spectrum and to unravel the underlying pathophysiology.Keywords: Children; COVID-19; kawasaki disease; MIS-C, neurology

AbstractAimsMultisystemic inflammatory syndrome in children (MIS‐C) is a condition noted in some children asymptomatic but positive to Sars‐cov‐2 antibody and it presents clinical and laboratory changes similar to Kawasaki disease (KD). Oral changes have also been observed. This systematic review evaluated oral manifestations detected in children with MIS‐C and KD associated to COVID‐19.Methods and ResultsThis work was registered at PROSPERO (#CRD42020225909), following PRISMA guidelines. A comprehensive research was conducted in MEDLINE, Web of Science, EMBASE, LILACS, Scopus, and Grey Literature through August 2021, based on original research evaluating children diagnosed with MIS‐C or KD related to COVID‐19. Two authors independently screened all retrieved references. Twenty five selected studies evaluated 624 children, mean age 8.78 years. The assessment of the risk of bias (ROB) showed that most of them presented low ROB. Oral manifestations were erythematous mucous membrane, oral ulcers lesions, dry, swollen and cracked lips, and strawberry tongue.ConclusionMIS‐C and KD share the same oral manifestations and their identification may lead to an early diagnosis.

Diagnostic Markers for Early Sepsis Diagnosis in Children With Systemic Inflammatory Response Syndrome
Sepsis caused by infection remains a major cause of mortality among children. One of the main reasons for high sepsis mortality rates is the inability to obtain early diagnosis. Sensitive and specific biomarkers are greatly needed in rapid diagnosis of sepsis. The main aim of study was to investigate the ability of high-mobility group box-1 protein (HMGB1), lipopolysaccharide-binding protein (LBP), Interleukin-6 (IL-6), procalcitonin (PCT) and C reactive protein (CRP) to differentiate sepsis patients. Eighty-four children with Systemic Inflammatory Response Syndrome (SIRS) were included in the prospective study. Sepsis was recognised in 23% (n = 19) of them. LBP, IL-6, CRP and PCT levels were significantly higher among the sepsis group (P < 0.05). HMGB1 levels in the sepsis patients did not significantly differ from SIRS patients. In ROC analysis in sepsis patients, identification markers LBP, IL6 and CRP performed quite similarly (P < 0.001), with the best result being for IL6. Our data suggest that in early sepsis diagnosis in children, LBP, IL-6, PCT and CRP are probably the superior diagnostic markers, with the best performance by IL6. LBP and IL-6 are superior markers for sepsis patients' disease process monitoring. HMGB1 does not have a diagnostic value for sepsis patient identification.

Traumatic CNS injury triggers a systemic inflammatory response syndrome (SIRS), in which circulating inflammatory cells invade body organs causing local inflammation and tissue damage. We have shown that the SIRS caused by spinal cord injury is greatly reduced by acute intravenous treatment with an antibody against the CD11d subunit of the CD11d/CD18 integrin expressed by neutrophils and monocyte/macrophages, a treatment that reduces their efflux from the circulation. Traumatic brain injury (TBI) is a frequently occurring injury after motor vehicle accidents, sporting and military injuries, and falls. Our studies have shown that the anti-CD11d treatment diminishes brain inflammation and oxidative injury after moderate or mild TBI, improving neurological outcomes. Accordingly, we examined the impact of this treatment on the SIRS triggered by TBI.