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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.

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.

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.

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.

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.

We describe a 46-year-old male with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection who presented as a Kawasaki-like syndrome with features including prolonged fever, bilateral conjunctivitis, oral mucosal swelling, diffuse erythematous rash, cervical and hilar lymphadenopathy, as well as cardiovascular complications and multi-organ failure. There are several reports of a similar clinical entity mimicking Kawasaki disease (KD) in the pediatric population, which has been termed Pediatric Inflammatory Multisystem Syndrome Temporally Associated with SARS-CoV-2 (PIMS-TS) by the Royal College of Pediatric and Child Health. To our knowledge, to date, there has been only one case report of COVID-19 presenting as KD in an adult patient.

A hyperinflammatory syndrome has been described in times of COVID-19 in children. In the setting of uncertainty due to a new virus, the so-called hyperinflammatory syndrome has been coined as a novel entity by some and is being referred to as pediatric inflammatory multisystem syndrome (PIMS). However, the characteristics of the syndrome resemble those of Kawasaki disease (KD), an inflammatory syndrome in children that can lead to coronary artery abnormalities due to a subsequent vasculitis. Furthermore, Kawasaki disease may occasionally trigger macrophage activation syndrome (MAS), a condition in which there is uncontrolled activation and proliferation of macrophages and other cell types, and could lead to multiorgan system dysfunction. This study provides a review of the data regarding COVID-19, Kawasaki disease, and macrophage activation syndrome to demonstrate the similarities and differences between the inflammatory syndrome seen with COVID-19 and KD. In addition, a framework for diagnosis and evaluation is provided that focuses on the pathway previously established for KD and MAS. The authors believe that based on current knowledge, KD treatment delays may carry deleterious effects in the near future for children with COVID-19-related Kawasaki disease.

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 juvenile idiopathic arthritis.