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Multisystemic inflammatory syndrome in children (MIS-C) is a rare, severe, post-infectious hyperinflammatory condition that occurs after COVID-19 infection. In this study, we aimed to demonstrate the risk reduction of MIS-C and severe MIS-C after Pfizer–BioNTech BNT162b2 mRNA COVID-19 vaccination. This nationwide cohort study included 526,685 PCR-confirmed COVID-19 cases (age < 19 years), of whom 14,118 were fully vaccinated prior to COVID-19 infection. MIS-C cases were collected from all hospitals in Israel from April 2020 through November 2021. The MIS-C rates were calculated among two COVID-19 populations: positive PCR confirmed cases and estimated COVID-19 cases (PCR confirmed and presumed). Vaccination status was determined from Ministry of Health (MoH) records. The MIS-C risk difference (RD) and 95% confidence intervals (95%CI) between vaccinated and unvaccinated patients are presented. Overall, 233 MIS-C cases under the age of 19 years were diagnosed and hospitalized in Israel during the study period. Among the estimated COVID-19 cases, MIS-C RD realistically ranged between 2.1 [95%CI 0.7–3.4] and 1.0 [95%CI 0.4–1.7] per 10,000 COVID-19 cases. For severe MIS-C, RD realistically ranged between 1.6 [95%CI 1.3–1.9] and 0.8 [95%CI 0.7–1.0], per 10,000 COVID-19 cases. Sensitivity analysis was performed on a wide range of presumed COVID-19 rates, demonstrating significant RD for each of these rates. Conclusion: This research demonstrates that vaccinating children and adolescents against COVID-19 has reduced the risk of MIS-C during the study period. What is Known: • Most of the published literature regarding vaccine effectiveness is based on case-control studies, which are limited due to small sample sizes and the inability to fully estimate the risk of MIS-C among vaccinated and unvaccinated children and adolescents. • The known underestimation of COVID-19 diagnosis among children and adolescents is challenging, as they often have few to no symptoms. What is New: • Significant risk difference was found in favor of the vaccinated group, even after including extreme assumptions regarding the underdiagnosed COVID-19 rate. • During this nationwide study period, it was found that vaccinating children and adolescents reduced the risk of MIS-C and its complications. [ABSTRACT FROM AUTHOR]

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Children with recent or acute coronavirus disease 2019 (COVID-19) infections are susceptible to a number of neurological complications, including encephalopathy and seizures. Within the phenomenon of multisystem inflammatory syndrome in children (MIS-C), patients may be encephalopathic or have other nervous system sequelae. The electroencephalographic (EEG) patterns accompanying neurological complications of COVID-19 infection have been reported but primarily in case reports or small case series. In this study, we reviewed all reports of EEG patterns seen in pediatric patients with presentations attributed to COVID-19 infection. Fifty patient reports were identified, drawn from 27 articles. We separately analyzed patients whose primary neurological concern was (1) encephalopathy, (2) seizures, or (3) other neurological abnormalities. Patients with acute encephalopathy tend to have EEG showing diffusely slow background, often in the delta range; however, the pattern of slowing is sometimes anterior or posterior predominant and may evolve over the course of illness. Patients with COVID-19 infection presenting with seizures may have focal or bilateral semiologies, but postictal EEG rarely shows interictal epileptiform discharges and is more likely to also show diffuse slowing. However, subclinical seizures and nonconvulsive status epilepticus have been reported, so prolonged EEG monitoring may still be indicated. [ABSTRACT FROM AUTHOR]

Introduction: Despite the existing data on the Multisystem Inflammatory Syndrome in Children (MIS-C), the factors that determine these patients evolution remain elusive. Answers may lie, at least in part, in genetics. It is currently under investigation that MIS-C patients may have an underlying innate error of immunity (IEI), whether of monogenic, digenic, or even oligogenic origin. Methods: To further investigate this hypothesis, 30 patients with MIS-C were submitted to whole exome sequencing. Results: Analyses of genes associated with MIS-C, MIS-A, severe covid-19, and Kawasaki disease identified twenty-nine patients with rare potentially damaging variants (50 variants were identified in 38 different genes), including those previously described in IFNA21 and IFIH1 genes, new variants in genes previously described in MIS-C patients (KMT2D, CFB, and PRF1), and variants in genes newly associated to MIS-C such as APOL1, TNFRSF13B, and G6PD. In addition, gene ontology enrichment pointed tothe involvement of thirteen major pathways, including complement system, hematopoiesis, immune system development, and type II interferon signaling, that were not yet reported in MIS-C. Discussion: These data strongly indicate that different gene families may favor MIS- C development. Larger cohort studies with healthy controls and other omics approaches, such as proteomics and RNAseq, will be precious to better understanding the disease dynamics. [ABSTRACT FROM AUTHOR]

Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

IntroductionDespite the existing data on the Multisystem Inflammatory Syndrome in Children (MIS-C), the factors that determine these patients evolution remain elusive. Answers may lie, at least in part, in genetics. It is currently under investigation that MIS-C patients may have an underlying innate error of immunity (IEI), whether of monogenic, digenic, or even oligogenic origin.MethodsTo further investigate this hypothesis, 30 patients with MIS-C were submitted to whole exome sequencing. ResultsAnalyses of genes associated with MIS-C, MIS-A, severe covid-19, and Kawasaki disease identified twenty-nine patients with rare potentially damaging variants (50 variants were identified in 38 different genes), including those previously described in IFNA21 and IFIH1 genes, new variants in genes previously described in MIS-C patients (KMT2D, CFB, and PRF1), and variants in genes newly associated to MIS-C such as APOL1, TNFRSF13B, and G6PD. In addition, gene ontology enrichment pointed to the involvement of thirteen major pathways, including complement system, hematopoiesis, immune system development, and type II interferon signaling, that were not yet reported in MIS-C.DiscussionThese data strongly indicate that different gene families may favor MIS- C development. Larger cohort studies with healthy controls and other omics approaches, such as proteomics and RNAseq, will be precious to better understanding the disease dynamics.

Introduction. Pediatric Inflammatory Multisystem Syndrome (MIS-C) is treated by the administration of intravenous polyvalent immunoglobulins and corticosteroids, as recommended by the French National Authority for Health (Haute Autorité de Santé) and the WHO (World Health Organization). However, no corticosteroids tapering schedule has been validated and patients returning home are not properly supervised by a pharmacist. Aims. Identify the occurrence of relapses according to the corticosteroid tapering schedules prescribed on return home. Analyze patients' reported compliance to these decreases. Identify possible links between poor compliance and relapse. Patients and method. This retrospective study analyzes the digital medical records on Orbis® software of patients who have been hospitalized for a MIS-C between April 2020 and June 2021 in a French pediatric hospital. Results. 66 MIS-C patients were included. 54 were treated by intravenous corticotherapy 2 mg/kg/day, 2 with 1 mg/kg/day, 10 have not received any. Five different tapering schedules were prescribed, 3 patients relapsed. Recurrence of relapse is not significantly related to the tapering schedule followed (p = 0,759). 6/54 (11 %) patients wrongly -followed their tapering schedules. Among them, 2 relapsed, versus 1/48 (2 %) among compliers (p = 0.029 ; OR = 0.04). Discussion – Conclusion. This study emphasizes the difficulty for a patient to comply with corticosteroids tapering schedule without supervision, as well as the subsequent rebound risks. Pharmaceutical counseling for patients returning home after hospitalization will be promoted to ensure better communication and patients' understanding and compliance. [ABSTRACT FROM AUTHOR]

BackgroundFollowing the Coronavirus Disease-19 (COVID-19) pandemic outbreaks, the hyperinflammatory condition termed Multisystem Inflammatory Syndrome in Children (MIS-C) became a healthcare issue worldwide. Since December 2020 the mRNA vaccine against SARS-CoV-2 has become available with a good safety profile. However, evidence regarding safety and vaccination strategies in children with previous MIS-C is still lacking. The aim of our study was to investigate the current approach of international centers to anti-SARS-CoV-2 and other vaccinations in children with a history of MIS-C.MethodsPhysicians who care for patients with MIS-C were invited to anonymously complete a 15-question, web-based survey. The survey was open from October 6 to December 31, 2021.ResultsA total of 290 replies from 236 centers in 61 countries were collected. Most respondents (86%) were pediatric rheumatologists. The anti-SARS-CoV-2 vaccine was available in 85% of the countries. Sixty-seven centers (28%) in 22 countries already vaccinated MIS-C patients without adverse reactions in most cases (89%). Six reported complications: 2 not specified, 3 mild symptoms and 1 reported a MIS-C-like reaction. Most centers (84%) favored vaccinating MIS-C patients against SARS-CoV-2, after 3–6 months (40%), 6–12 months (52%) or >12 months (8%). The survey revealed broad heterogeneity of responses among healthcare providers within the same country and within the same center. The variable with the greatest impact on the decision not to vaccinate MIS-C patients was the current lack of evidence (51%), followed by patient/parent objection (40%). The most relevant parameters in the vaccination strategy were time from MIS-C episode (78%), immunosuppressive treatment (35%), SARS-CoV-2 serologic status (32%), and MIS-C features (31%). Almost all centers favored continuing regular vaccination with non-live (99%) and live (93%) vaccines; however, with high variability in suggested timelines.ConclusionTo date, the experience of the international pediatric rheumatology community in vaccinating MIS-C patients against SARS-CoV-2 is overall reassuring. However, lack of evidence causes broad heterogeneity in vaccination strategy worldwide.

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Purpose of Review: The novel coronavirus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and has developed into a pandemic. A unique challenge of this pandemic has been the emergence of multisystem inflammatory syndrome in children (MIS-C), a rare post-infectious hyperinflammatory disorder associated with SARS-CoV-2. This syndrome is characterized by overwhelming systemic inflammation, fever, hypotension, and cardiac dysfunction. This disorder may also have features overlapping with Kawasaki disease (KD), macrophage activation syndrome (MAS), and toxic shock syndrome (TSS). The goal of this review is to outline the presenting features, presumed immunopathogenesis, management, and outcomes of patients with MIS-C. Recent Findings: Patients with MIS-C present with characteristics that fall within a wide clinical spectrum. Main features include fever, gastrointestinal symptoms such as abdominal pain and diarrhea, and cardiac complications such as myocarditis and coronary artery aneurysms, although various other features have been reported. Younger children may present with features of Kawasaki-like disease, and older children are often admitted to the intensive care unit with cardiogenic shock. Current treatment guidelines recommend intravenous immunoglobulins (IVIG) and glucocorticoids, with utilization of biologics in refractory cases. Fortunately, the majority of patients recover, with resolution of the systemic inflammation and cardiac abnormalities. Mortality from MIS-C is rare. Summary: This review provides an overview of the presenting features, proposed pathogenesis, suggested therapies, and outcomes of MIS-C. Clinicians must have a high clinical suspicion for this disorder in children who have had recent COVID-19 infection or exposure and present with a significant inflammatory response. Understanding of this disorder continues to evolve, and prompt diagnosis and treatment allow for the best possible outcome for patients with MIS-C. [ABSTRACT FROM AUTHOR]

The coronavirus disease 2019 (COVID-19) pandemic has caused widespread mortality and morbidity. Though children are largely spared from severe illness, a novel childhood hyperinflammatory syndrome presumed to be associated with and subsequent to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has emerged with potentially severe outcomes. Multisystem inflammatory disorder in children (MIS-C) most commonly affects young, school-aged children and is characterized by persistent fever, systemic hyperinflammation, and multisystem organ dysfunction. While uncommon and generally treatable, MIS-C presents potentially life-altering medical sequelae, complicated by a dearth of information regarding its etiology, pathophysiology, and long-term outcomes. The severity of MIS-C may warrant the need for increased awareness and continued COVID-19 mitigation efforts, particularly until potential factors conferring a predisposition to MIS-C can be clarified through additional research. Well-informed guidelines will be critical as the school year progresses. In this article, current knowledge on MIS-C is reviewed and the potential implications of this novel syndrome are discussed from a public health perspective.

Introduction and objectivesPediatric inflammatory multisystem syndrome (PIMS) is a life-threatening complication in pediatric patients with SARS-CoV-2 infection. An increase in the association of gastrointestinal symptoms and the presence of PIMS has been observed. The objective of this study was to analyze whether pediatric patients with COVID-19, who debut with gastrointestinal symptoms, have a higher risk of developing PIMS.Material and methodsAn observational, analytical and retrolective study was carried out with a review of the records of patients diagnosed with COVID-19. Demographic, clinical and laboratory variables were recorded.ResultsA total of 248 patients who met the selection criteria were included. Of Those 40% were female, with a mean age of 7 +/- 5.8 years. Gastrointestinal symptoms were the initial presentation in 103 patients, with vomiting being the most frequent symptom, followed by abdominal pain and diarrhea. In total 52 patients developed PIMS, 30 of whom presented with gastrointestinal symptoms. A RR of 1.57 (97% CI of 1.17–2.11) was found for the presentation of PIMS in patients positive for SARS-CoV-2 who present with gastrointestinal symptoms.ConclusionsThere is an increased risk of developing pediatric multisystem inflammatory syndrome when there are gastrointestinal symptoms in pediatric patients with COVID-19.

Abstract Background Although symptoms and signs of COVID-19 (Coronavirus disease 2019) in children are milder than adults, there are reports of more severe cases which were defined as pediatric inflammatory multisystem syndrome (PIMS). The purpose of this report was to describe the possible association between COVID-19 and PIMS in children. Methods From 28 March to 24 June 2020, 10 febrile children were admitted with COVID-19 infection showing characteristics of PIMS in Buali tertiary hospital of Sari, in Mazandaran province, northern Iran. Demographic and clinical characteristics, laboratory and imaging findings, and therapeutic modalities were recorded and analyzed. Results The mean age of the patients was 5.37 ± 3.9 years (13 months to 12 years). Six of them were boys. Kawasaki disease, myocarditis, toxic shock syndrome, appendicitis, sepsis, urosepsis, prolonged febrile seizure, acute hemorrhagic edema of infancy, and COVID-19-related pneumonia were their first presentation. All of them had increased C-reactive protein levels, and most of them had elevated erythrocyte sedimentation rate, lymphopenia, anemia, and hypoalbuminemia. Three of them had thrombocytopenia(PLT

The pandemic of COVID-19 initially appeared to cause only a mild illness in children. However, it is now apparent that a small percentage of children can develop a hyperinflammatory syndrome labeled as Pediatric inflammatory multisystem syndrome—temporally associated with SARS-CoV-2 (PIMS-TS) with a phenotype resembling Kawasaki disease (KD) ('Kawa-COVID-19′). Features of this newly recognized condition may include fever, hypotension, severe abdominal pain and cardiac dysfunction, evidence of inflammation, and single or multi organ dysfunction in the absence of other known infections. Children emerge to have mild symptoms compared to adults, perhaps due to reduced expression of the angiotensin converting enzyme (ACE)-2 receptor (the target of SARS-CoV-2) gene, trained innate immunity, and a young and fit immune system. Some of these children may share features of Kawasaki disease, toxic shock syndrome or cytokine storm syndrome. They can deteriorate rapidly and may need intensive care support as well. The PCR test is more often negative although most of the children have antibodies to SARS-CoV-2. Although the pathogenesis is not clearly known, immune-mediated injury has been implicated. [ABSTRACT FROM AUTHOR]

The cutaneous manifestations of COVID-19 infection are evolving rapidly as new data continue to be published in the literature. Considering that majority of these manifestations resemble other viral exanthemas, a high degree of suspicion and clinical acumen is needed for early identification of COVID-19 infections and its prompt management. The most common manifestations in adults include maculopapular rash, purpura, vesicular rash, urticarial rash, and pseudo-chilblains, while in children, the manifestations include Kawasaki disease-like inflammatory syndrome (pediatric inflammatory multisystem syndrome) and erythema multiforme. This review presents a comprehensive guide to the cutaneous manifestations of COVID-19 infections in adults and children. Also included are the dermatological manifestations observed due to prolonged use of personal protective equipment in healthcare workers.

The first cases of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection were identified at the end of 2019 and, in the next few months, coronavirus disease (COVID-19) spread throughout the world. Initially, it was believed that this disease mainly affected elderly individuals with comorbidities, in whom respiratory failure often occurs. It was believed that children fell ill from the infection more often, although the course of infection in the vast majority of pediatric cases has been asymptomatic or mildly symptomatic. In April and May 2020, the first report of a rapidly progressing disease, similar to Kawasaki syndrome, was found in children who had been infected with SARS-CoV-2. Shortly thereafter, children with symptoms of pediatric inflammatory multisystem syndrome (PIMS-ST [temporally associated with SARS-CoV-2 infection]) began presenting to pediatric hospitals around the world. The syndrome has a mortality rate of up to 2%. Symptoms of PIMS-TS include those that may suggest the need for surgical treatment (severe abdominal pain with the presence of peritoneal symptoms, ascites, high levels of inflammatory markers, intestinal inflammation, and appendages revealed on ultrasound examination). However, there are few reports addressing surgical cases associated with this condition. The authors present a case involving an 11-year-old boy who was admitted to hospital with severe abdominal pain and underwent surgery for symptoms of peritonitis and was diagnosed with PIMS in the post-operative period. Due to the large number of illnesses caused by SARS-CoV-2 infection in recent months, the diagnosis of PIMS-TS/MISC should be considered in the differential diagnosis of acute abdominal symptoms, especially in atypical courses and interviews indicating exposure to SARS-CoV-2.

Multisystem inflammatory syndrome in children (MIS-C) or pediatric inflammatory multisystem syndrome (PIMS / PIMS-TS), is a rare systemic illness causing persistent fever and extreme inflammation following exposure to SARS-CoV-2. In children who go on to develop MIS-C, some organs and tissues such as the heart, lungs, blood vessels, kidneys, digestive system, brain, skin or eyes become severely inflamed. Signs and symptoms depend on which areas of the body are affected.

Background: Following the spread of the coronavirus disease 2019 (COVID-19) pandemic a new disease entity emerged, defined as Pediatric Inflammatory Multisystem Syndrome temporally associated with COVID-19 (PIMS-TS), or Multisystem Inflammatory Syndrome in Children (MIS-C). In the absence of trials, evidence for treatment remains scarce.Purpose: To develop best practice recommendations for the diagnosis and treatment of children with PIMS-TS in Switzerland. It is acknowledged that the field is changing rapidly, and regular revisions in the coming months are pre-planned as evidence is increasing.Methods: Consensus guidelines for best practice were established by a multidisciplinary group of Swiss pediatric clinicians with expertise in intensive care, immunology/rheumatology, infectious diseases, hematology, and cardiology. Subsequent to literature review, four working groups established draft recommendations which were subsequently adapted in a modified Delphi process. Recommendations had to reach >80% agreement for acceptance.Results: The group achieved agreement on 26 recommendations, which specify diagnostic approaches and interventions across anti-inflammatory, anti-infectious, and support therapies, and follow-up for children with suspected PIMS-TS. A management algorithm was derived to guide treatment depending on the phenotype of presentation, categorized into PIMS-TS with (a) shock, (b) Kawasaki-disease like, and (c) undifferentiated inflammatory presentation.Conclusion: Available literature on PIMS-TS is limited to retrospective or prospective observational studies. Informed by these cohort studies and indirect evidence from other inflammatory conditions in children and adults, as well as guidelines from international health authorities, the Swiss PIMS-TS recommendations represent best practice guidelines based on currently available knowledge to standardize treatment of children with suspected PIMS-TS. Given the absence of high-grade evidence, regular updates of the recommendations will be warranted, and participation of patients in trials should be encouraged.

Although the pattern of proinflammatory cytokines induced in COVID-2019 is similar to that of rheumatoid arthritis, the association of arthritis with SARS-CoV-2 infection is extremely rare and the symptoms are generally acute and self-limited. Herein we present the clinical case of a child who developed chronic arthritis after SARS-CoV-2 infection. An 11-year-old girl started with symptoms of multisystem inflammatory syndrome temporally associated with COVID-19 infection and subsequently developed chronic arthritis. After six weeks of arthritis, corticosteroids were started which resulted in clinical improvement after two weeks of use. Serology for SARS-CoV-2 was positive in the fifth week after symptom onset. Currently, the patient has no clinical complaints but continues to experience morning stiffness, high erythrocyte sedimentation rate, and synovial hypertrophy with no power Doppler signal on ultrasound. We alert to the possibility that SARS-CoV-2 may be a trigger of chronic arthritis.

The well-documented systemic inflammation associated to pediatric obesity might act as an augmenting factor for other inflammatory conditions, such as pediatric inflammatory multisystem syndrome (PIMS) associated to COVID-19. We report the case of 9-year-old boy admitted in our clinic for fever, anorexia, and fatigability. The clinical exam revealed influenced general status, palpebral edema, non-exudative conjunctivitis, and abdominal tenderness. The patient weighed 45 kg. The laboratory tests at the time of admission pointed out anemia, lymphopenia; elevated inflammatory biomarkers, NT-proBNP, D-dimers, and troponin; high liver enzymes and lactate dehydrogenase levels, as well as hypoalbuminemia. The patient tested positive for both RT-PCR and serology for SARS-CoV-2 infection. We initiated intravenous immunoglobulin and methylprednisolone, associated with empirical antibiotic, anticoagulation therapy, and symptomatic treatment. The patient was discharged on the 7th day of admission with the recommendation to continue enoxaparin and methylprednisolone at home tapering the dose for the next week. The subclinical inflammatory status associated to obesity might serve as an unfortunate trigger factor for the development of COVID-19 severe forms in children. Therefore, clinicians should be aware that children with obesity and COVID-19 represent a peculiar group that should be closely monitored and thoroughly assessed in order to preempt life-threatening complications, such as PIMS.

Renal infarction is a rare finding in children. Associations between SARS-CoV-2 infections and thromboembolic events including renal infarcts have been described in adults. Although a similar association in children has not yet been described with this pandemic, the pediatric literature is still evolving with the recognition of new manifestations including the post-infectious Multisystem Inflammatory Syndrome in Children (MIS-C). We report the rare event of multiple renal infarcts in a 6-year-old boy manifesting several features of MIS-C 9 weeks following a self-limiting febrile illness characteristic of COVID-19. An underlying Factor V Leiden mutation was identified in this child but felt to be insufficient on its own to explain his clinical presentation. As SARS-CoV-2 testing was delayed, the failure to identify viral RNA or antibodies may not exclude the virus' potential role in precipitating the infarct in this host. Given that renal infarcts have been described in adult patients with COVID-19, reporting this perplexing case where SARS-CoV-2 may have played a role, may help identify this potential complication.

The coronavirus disease 2019 (COVID-19) pandemic has caused widespread mortality and morbidity. Though children are largely spared from severe illness, a novel childhood hyperinflammatory syndrome presumed to be associated with and subsequent to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has emerged with potentially severe outcomes. Multisystem inflammatory disorder in children (MIS-C) most commonly affects young, school-aged children and is characterized by persistent fever, systemic hyperinflammation, and multisystem organ dysfunction. While uncommon and generally treatable, MIS-C presents potentially life-altering medical sequelae, complicated by a dearth of information regarding its etiology, pathophysiology, and long-term outcomes. The severity of MIS-C may warrant the need for increased awareness and continued COVID-19 mitigation efforts, particularly until potential factors conferring a predisposition to MIS-C can be clarified through additional research. Well-informed guidelines will be critical as the school year progresses. In this article, current knowledge on MIS-C is reviewed and the potential implications of this novel syndrome are discussed from a public health perspective. [ABSTRACT FROM AUTHOR]

Globally, the coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), appeared to have a milder clinical course in children compared to adults. As severe forms of COVID-19 in adults included an aberrant systemic immune response, children with chronic systemic inflammatory diseases were cautiously followed. No evidence for a specific susceptibility was identified in this pediatric population. European and US Pediatricians started to notice cases of myocarditis, sharing some features with toxic shock syndrome, Kawasaki disease, and macrophage activation syndrome in otherwise healthy patients. Multisystem Inflammatory Syndrome in Children (MIS-C) and Pediatric Inflammatory Multisystem Syndrome (PIMS) have designated this new entity in the US and Europe, respectively. The spectrum of severity ranged from standard hospitalization to pediatric intensive care unit management. Most patients had a clinical history of exposure to COVID-19 patients and/or SARS-COV2 biological diagnosis. Clinical presentations include fever, cardiac involvement, gastro-intestinal symptoms, mucocutaneous manifestations, hematological features, or other organ dysfunctions. The temporal association between the pandemic peaks and outbreaks of PIMS seems to be in favor of a post-infectious, immune-mediated mechanism. Thus, SARS-CoV2 can rarely be associated with severe systemic inflammatory manifestations in previously healthy children differently from adults highlighting the specific need for COVID-19 research in the pediatric population.

Numerous recent studies have shown that patients with underlying cardiovascular disease (CVD) are at increased risk of more severe clinical course as well as mortality of COVID-19. Also, the available data suggests that COVID-19 is related to numerous de novo cardiovascular complications especially in the older population and those with pre-existing chronic cardiometabolic conditions. SARS-CoV-2 virus can cause acute cardiovascular injury, as well as increase the risk of chronic cardiovascular damage. As CVD seem to be the major comorbidity in critically unwell patients with COVID-19 and patients often die of cardiovascular complications, we review the literature and discuss the possible pathophysiology and molecular pathways driving these disease processes: cytokine release syndrome, RAAS system dysregulation, plaque destabilization and coagulation disorders with the aim to identify novel treatment targets. In addition, we review the pediatric population, the major cause of the cardiovascular complications is pediatric inflammatory multisystem syndrome that is believed to be associated with COVID-19 infection. Due to the increasingly recognized CVD damage in COVID-19, there is a need to establish clear clinical and follow-up protocols and to identify and treat possible comorbidities that may be risk factors for the development of cardiovascular complications.

Background: A small subset of pediatric patients develop a rare syndrome associated with Coronavirus Disease 2019 (COVID-19) infection called multisystem inflammatory syndrome in children (MIS-C). This syndrome shares characteristics with Kawasaki disease.Case Report: A 15-year-old girl presented to our Emergency Department (ED) with fevers and malaise. She was diagnosed on her initial visit with an acute viral syndrome and discharged with a COVID polymerase chain reaction test pending, which was subsequently negative. She returned 3 days later with persistent fever, conjunctivitis, and a symmetric targetoid rash over her palms. She had no adenopathy, but her erythrocyte sedimentation rate and C-reactive protein were both significantly elevated at 90 mm/h and 19.61 mg/dL, respectively. The patient was then transferred to the regional children's hospital due to a clinical suspicion for MIS-C, and subsequent COVID-19 immunoglobulin G testing was positive. She had been empirically started on intravenous immunoglobulin in addition to 81 mg aspirin daily. Initial echocardiograms showed mild dilatation of the left main coronary artery, and on repeat echocardiogram, a right coronary artery aneurysm was also identified. Oral prednisone therapy (5 mg) was initiated and the patient was discharged on a continued prednisone taper. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: We present a case of a 15-year-old girl who presented to the ED with MIS-C who developed coronary aneurysms despite early therapy, to increase awareness among emergency physicians of this emerging condition. [ABSTRACT FROM AUTHOR]

Numerous recent studies have shown that patients with underlying cardiovascular disease (CVD) are at increased risk of more severe clinical course as well as mortality of COVID-19. Also, the available data suggests that COVID-19 is related to numerous de novo cardiovascular complications especially in the older population and those with pre-existing chronic cardiometabolic conditions. SARS-CoV-2 virus can cause acute cardiovascular injury, as well as increase the risk of chronic cardiovascular damage. As CVD seem to be the major comorbidity in critically unwell patients with COVID-19 and patients often die of cardiovascular complications, we review the literature and discuss the possible pathophysiology and molecular pathways driving these disease processes: cytokine release syndrome, RAAS system dysregulation, plaque destabilization and coagulation disorders with the aim to identify novel treatment targets. In addition, we review the pediatric population, the major cause of the cardiovascular complications is pediatric inflammatory multisystem syndrome that is believed to be associated with COVID-19 infection. Due to the increasingly recognized CVD damage in COVID-19, there is a need to establish clear clinical and follow-up protocols and to identify and treat possible comorbidities that may be risk factors for the development of cardiovascular complications. [ABSTRACT FROM AUTHOR]

Copyright of Der Pneumologe is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Polyarteritis nodosa (PAN) and Kawasaki syndrome (KS) are rare forms of primary vasculitis with heterogeneous manifestations and courses of the disease. According to the Chapel Hill Consensus Conference 2012 they belong to the vasculitis of medium size vessels. In contrast to microscopic polyangiitis (MPA), PAN and KS do not affect microscopic vessels such as arterioles, venules or capillaries and are not associated with antineutrophil cytoplasmic antibodies (ANCA). The diagnostics are based on the typical constellation of clinical symptoms, on angiographic findings, the exclusion of other differential diagnoses and, in the case of PAN, in the histopathological confirmation. The therapeutic options of KS in childhood and PAN in adults and children, which are dependent on the severity and the prognosis, are presented., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

The article presents a case study of a 21-month-old, male, Japanese patient presented with a fever and cough, and the index patient was his mother, who previously received the diagnosis of COVID-19. The article discusses that he also had mild diarrhea but denied conjunctivitis, erythema at the Bacillus Calmette-Guerin inoculation site; and laboratory data showed elevated inflammatory and cytokine marker.

Introduction: Despite the existing data on the Multisystem Inflammatory Syndrome in Children (MIS-C), the factors that determine these patients evolution remain elusive. Answers may lie, at least in part, in genetics. It is currently under investigation that MIS-C patients may have an underlying innate error of immunity (IEI), whether of monogenic, digenic, or even oligogenic origin., Methods: To further investigate this hypothesis, 30 patients with MIS-C were submitted to whole exome sequencing., Results: Analyses of genes associated with MIS-C, MIS-A, severe covid-19, and Kawasaki disease identified twenty-nine patients with rare potentially damaging variants (50 variants were identified in 38 different genes), including those previously described in IFNA21 and IFIH1 genes, new variants in genes previously described in MIS-C patients (KMT2D, CFB, and PRF1), and variants in genes newly associated to MIS-C such as APOL1, TNFRSF13B, and G6PD. In addition, gene ontology enrichment pointed to the involvement of thirteen major pathways, including complement system, hematopoiesis, immune system development, and type II interferon signaling, that were not yet reported in MIS-C., Discussion: These data strongly indicate that different gene families may favor MIS- C development. Larger cohort studies with healthy controls and other omics approaches, such as proteomics and RNAseq, will be precious to better understanding the disease dynamics., Competing Interests: The authors declare that this research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Reis, Soares Faccion, de Carvalho, Moore, Zuma, Plaça, Salerno Filgueiras, Leandro Mathias Fonseca, Cabral-Marques, Bonomo, Savino, Freitas, Faoro, Passetti, Robaina, de Oliveira, Novaes Bellinat, Zeitel, Salú, de Oliveira, Rodrigues-Santos, Prata-Barbosa and Vasconcelos.)

Background: Following the Coronavirus Disease-19 (COVID-19) pandemic outbreaks, the hyperinflammatory condition termed Multisystem Inflammatory Syndrome in Children (MIS-C) became a healthcare issue worldwide. Since December 2020 the mRNA vaccine against SARS-CoV-2 has become available with a good safety profile. However, evidence regarding safety and vaccination strategies in children with previous MIS-C is still lacking. The aim of our study was to investigate the current approach of international centers to anti-SARS-CoV-2 and other vaccinations in children with a history of MIS-C., Methods: Physicians who care for patients with MIS-C were invited to anonymously complete a 15-question, web-based survey. The survey was open from October 6 to December 31, 2021., Results: A total of 290 replies from 236 centers in 61 countries were collected. Most respondents (86%) were pediatric rheumatologists. The anti-SARS-CoV-2 vaccine was available in 85% of the countries. Sixty-seven centers (28%) in 22 countries already vaccinated MIS-C patients without adverse reactions in most cases (89%). Six reported complications: 2 not specified, 3 mild symptoms and 1 reported a MIS-C-like reaction. Most centers (84%) favored vaccinating MIS-C patients against SARS-CoV-2, after 3-6 months (40%), 6-12 months (52%) or >12 months (8%). The survey revealed broad heterogeneity of responses among healthcare providers within the same country and within the same center. The variable with the greatest impact on the decision not to vaccinate MIS-C patients was the current lack of evidence (51%), followed by patient/parent objection (40%). The most relevant parameters in the vaccination strategy were time from MIS-C episode (78%), immunosuppressive treatment (35%), SARS-CoV-2 serologic status (32%), and MIS-C features (31%). Almost all centers favored continuing regular vaccination with non-live (99%) and live (93%) vaccines; however, with high variability in suggested timelines., Conclusion: To date, the experience of the international pediatric rheumatology community in vaccinating MIS-C patients against SARS-CoV-2 is overall reassuring. However, lack of evidence causes broad heterogeneity in vaccination strategy worldwide., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2022 Minoia, Lucioni, Heshin-Bekenstein, Vastert, Kessel, Uziel, Lamot, Ruperto, Gattorno, Bracaglia and Toplak.)

Introduction and Objectives: Pediatric inflammatory multisystem syndrome (PIMS) is a life-threatening complication in pediatric patients with SARS-CoV-2 infection. An increase in the association of gastrointestinal symptoms and the presence of PIMS has been observed. The objective of this study was to analyze whether pediatric patients with COVID-19, who debut with gastrointestinal symptoms, have a higher risk of developing PIMS., Material and Methods: An observational, analytical and retrolective study was carried out with a review of the records of patients diagnosed with COVID-19. Demographic, clinical and laboratory variables were recorded., Results: A total of 248 patients who met the selection criteria were included. Of Those 40% were female, with a mean age of 7 +/- 5.8 years. Gastrointestinal symptoms were the initial presentation in 103 patients, with vomiting being the most frequent symptom, followed by abdominal pain and diarrhea. In total 52 patients developed PIMS, 30 of whom presented with gastrointestinal symptoms. A RR of 1.57 (97% CI of 1.17-2.11) was found for the presentation of PIMS in patients positive for SARS-CoV-2 who present with gastrointestinal symptoms., Conclusions: There is an increased risk of developing pediatric multisystem inflammatory syndrome when there are gastrointestinal symptoms in pediatric patients with COVID-19., (Copyright © 2022 Jaramillo-Esparza and Vázquez-Frias.)

Viral and paraviral exanthems are the most common exanthems in children and are often the reason for a medical evaluation, especially in pediatric primary care and emergency rooms. Familiarity with the various eruptions is important for early diagnosis and patient management as well as minimizing the risk of infection. In this review, we present the newly described entities reactive infectious mucocutaneous eruptions (RIME) and the pediatric inflammatory multisystem syndrome (PIMS). In addition, atypical manifestations of Gianotti-Crosti syndrome and hand, foot, and mouth disease are discussed., (© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)