Severe refractory Kawasaki disease in seven infants in the COVID-19 era

Vergnano, Stefania; Alders, Nele; Armstrong, Catherine; Bamber, AR; Bandi, Srini; Evans, Jennifer; Hajiani, Nadia; Kenny, Julia; Kucera, Filip; Tometzki, Andrew; Uzun, Orhan; Wilkinson, Nick; Ramanan, Athimalaipet V

doi:10.1016/s2665-9913(20)30231-9

Cited by 6 publications

(14 citation statements)

References 3 publications

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“…Fever was documented in nearly all patients (922/928; 99.4%) [ 11 – 17 , 23 , 24 , 30 – 85 ], commonly during at least 5 days (258/928; 27.0%). The majority (598/699; 85.6%) presented gastrointestinal symptoms, mostly abdominal pain (315/539; 58.4%), vomiting (306/532; 57.5%), and diarrhea (268/532; 50.4%) [ 11 – 14 , 16 , 24 , 26 , 30 – 37 , 39 , 40 , 43 – 45 , 47 , 49 – 52 , 54 – 77 , 79 , 80 , 82 , 84 – 86 ]. Cardiovascular manifestations were found in 79.3% of patients (307/387) [ 11 – 17 , 24 , 30 – 37 , 39 – 41 , 44 – 49 , 51 – 86 ].…”

Section: Resultsmentioning

confidence: 99%

“…A quarter of patients (130/557; 23.3%) fulfilled criteria for complete KD [ 12 , 13 , 15 , 30 , 35 , 37 , 41 , 45 , 46 , 50 , 53 , 56 , 59 , 61 , 63 , 64 , 68 , 69 , 72 , 73 , 77 , 79 , 81 , 83 , 85 , 86 ] (criteria in Supplementary information 1 ). A similar proportion (99/411; 24.1%) fulfilled 2 or 3 of the KD criteria in combination with prolonged fever, resembling incomplete KD [ 11 – 13 , 32 , 36 , 39 , 44 , 47 , 48 , 52 , 54 , 57 , 61 – 63 , 65 – 69 , 77 , 79 , 81 , 83 ]. Polymorphous exanthema (466/849; 54.9%) and non-purulent conjunctivitis (423/849; 49.8%) occurred the most.…”

Section: Resultsmentioning

confidence: 99%

“…Only 338/901 (37.5%) had positive respiratory RT-PCR. Positive fecal RT-PCR was rare (7/268; 2.6%) [ 16 , 39 , 40 , 45 , 58 , 59 , 62 , 64 , 79 ]. Close contacts with COVID-19 were registered in 168/598 (28.1%) [ 11 – 13 , 17 , 24 , 34 , 36 , 40 , 44 , 47 , 48 , 53 , 59 , 61 , 63 – 65 , 70 , 72 , 73 , 75 , 77 , 78 , 80 – 82 ].…”

Section: Resultsmentioning

confidence: 99%

“…Of single cases, 115/138 (83.3%) had a microbiologically confirmed SARS-CoV-2 infection (PCR and/or serology-positive). Of the 23 cases negative (or missing) for both techniques [ 11 , 12 , 24 , 35 , 50 , 54 , 56 , 62 , 73 , 77 , 79 ], 15 additionally had no known COVID-19 contact [ 11 , 12 , 35 , 50 , 54 , 56 , 62 , 79 ].…”

Section: Resultsmentioning

confidence: 99%

“…In contrast, the CDC definition comprised only 62% (8 not assessable) of single cases and neglected on 31 cases with severe course, all failing to achieve the CDC criterium concerning the multi-organ (≥2 organ systems) dysfunction. Of single cases, the WHO definition missed 1 out of 5 patients needing ECMO because of insufficient data reported to assess [ 74 ], while the CDC definition not included 2/5 ECMO cases [ 11 , 52 , 66 , 74 , 75 ] and 2/6 deaths [ 11 , 24 , 66 , 74 , 75 , 79 ].…”

Section: Resultsmentioning

confidence: 99%

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Multisystem inflammatory syndrome in children related to COVID-19: a systematic review

2021

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An association between a novel pediatric hyperinflammatory condition and SARS-CoV-2 was recently published and termed pediatric inflammatory multisystem syndrome, temporally associated with SARS-CoV-2 (PIMS-TS) or multisystem inflammatory syndrome (in children) (MIS(-C)). We performed a systematic review and describe the epidemiological, clinical, and prognostic characteristics of 953 PIMS-TS/MIS(-C) cases in 68 records. Additionally, we studied the sensitivity of different case definitions that are currently applied. PIMS-TS/MIS(-C) presents at a median age of 8 years. Epidemiological enrichment for males (58.9%) and ethnic minorities (37.0% Black) is present. Apart from obesity (25.3%), comorbidities are rare. PIMS-TS/MIS(-C) is characterized by fever (99.4%), gastrointestinal (85.6%) and cardiocirculatory manifestations (79.3%), and increased inflammatory biomarkers. Nevertheless, 50.3% present respiratory symptoms as well. Over half of patients (56.3%) present with shock. The majority of the patients (73.3%) need intensive care treatment, including extracorporal membrane oxygenation (ECMO) in 3.8%. Despite severe disease, mortality is rather low (1.9%). Of the currently used case definitions, the WHO definition is preferred, as it is more precise, while encompassing most cases. Conclusion : PIMS-TS/MIS(-C) is a severe, heterogeneous disease with epidemiological enrichment for males, adolescents, and racial and ethnic minorities. However, mortality rate is low and short-term outcome favorable. Long-term follow-up of chronic complications and additional clinical research to elucidate the underlying pathogenesis is crucial. What is Known: • A novel pediatric inflammatory syndrome with multisystem involvement has been described in association with SARS-CoV-2. • To date, the scattered reporting of cases and use of different case definitions provides insufficient insight in the full clinical spectrum, epidemiological and immunological features, and prognosis. What is New: • This systematic review illustrates the heterogeneous spectrum of PIMS-TS/MIS(-C) and its epidemiological enrichment for males, adolescents, and racial and ethnic minorities. • Despite its severe presentation, overall short-term outcome is good. • The WHO MIS definition is preferred, as it is more precise, while encompassing most cases. Supplementary Information The online version contains supplementary material available at 10.1007/s00431-021-03993-5.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Multisystem inflammatory syndrome in children related to COVID-19: a systematic review

2021

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The JANUS of chronic inflammatory and autoimmune diseases onset during COVID-19 – A systematic review of the literature

Novelli

Motta

Santis

et al. 2021

Journal of Autoimmunity

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Multisystem Inflammatory Syndrome in Children Associated with COVID-19 Infection: A Comprehensive Review

Meza-Contreras

Galdos-Bejar

Escalante-Kanashiro

2022

J Pediatr Intensive Care

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The Multisystem Inflammatory Syndrome in Children (MIS-C) is a postinfectious syndrome associated with coronavirus disease 2019 (COVID-19) disease in children. The aim of this study is to conduct a thorough review to assist health care professionals in diagnosis and management of this complication of COVID-19 disease in children. A thorough systematic review was conducted through an on-line search based on MIS-C with the primary focus on epidemiology, clinical characteristics, diagnosis, pathophysiology, management, and long-term follow-up. This syndrome is characterized by an exaggerated and uncontrolled release of proinflammatory cytokines involving dysfunction of both innate and adaptive immunity. In this review, a summary of observational studies and case reports was conducted, in which we found that MIS-C generates multiple-organ failure frequently presenting with hemodynamic instability further characterized by Kawasaki-like symptoms (such as persistent high fever, polymorphic rash, and bilateral conjunctivitis) and predominance of gastrointestinal and cardiovascular signs and symptoms. Keys to effective management involve early diagnosis, timely treatment and re-evaluation following hospital discharge. Diagnosis is marked by significant elevation of inflammatory biomarkers, laboratory evidence of COVID-19 infection or history of recent exposure, and absence of any other plausible explanation for the associated signs, symptoms, and presentation. Management includes hemodynamic stabilization, empiric antibiotic therapy (de-escalation if cultures and polymerase chain reaction studies indicate no bacterial co-infection), immunomodulatory therapy (methylprednisolone, intravenous immunoglobulin, anakinra, tocilizumab, siltuximab, Janus kinase inhibitors, tumor necrosis factor-α inhibitors), antivirals (remdesivir), and anticoagulation (acetylsalicylic acid, unfractionated or low-molecular-weight heparin or new oral anticoagulants). In addition, we identified poor prognostic risk factors to include concurrent comorbidities, blood-component consumption and marrow suppression (lymphopenia, thrombocytopenia), depletion of homeostatic components (hypoalbuminemia), and marked evidence of a hyperinflammatory response to include elevated values of ferritin, C-reactive protein, and D-dimer. MIS-C constitutes a postinfectious syndrome characterized by a marked cytokine storm, characterized by fever, bilateral conjunctivitis, and multiple organ dysfunction. Promoting future research and long-term follow-up will be essential for the development of guidelines and recommendations leading to effective identification and management of MIS-C.

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Severe refractory Kawasaki disease in seven infants in the COVID-19 era

Cited by 6 publications

References 3 publications

Multisystem inflammatory syndrome in children related to COVID-19: a systematic review

Multisystem inflammatory syndrome in children related to COVID-19: a systematic review

The JANUS of chronic inflammatory and autoimmune diseases onset during COVID-19 – A systematic review of the literature

Multisystem Inflammatory Syndrome in Children Associated with COVID-19 Infection: A Comprehensive Review

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