scholarly journals Perspective of Immunopathogenesis and Immunotherapies for Kawasaki Disease

2021 ◽  
Vol 9 ◽  
Author(s):  
Lung Chang ◽  
Horng-Woei Yang ◽  
Tang-Yu Lin ◽  
Kuender D. Yang
Keyword(s):  
Kawasaki Disease ◽  
Macrophage Activation ◽  
Clinical Symptoms ◽  
Human Leukocyte ◽  
T Helper 17 ◽  
Leukocyte Antigen ◽  
Ivig Resistance ◽  
Risk Patients ◽  
Phenotypic Variations ◽  
Inflammatory Syndrome

Kawasaki Disease (KD) is an acute inflammatory illness that mostly occurs in children below 5 years of age, with intractable fever, mucocutaneous lesions, lymphadenopathy, and lesions of the coronary artery (CAL). KD is sharing clinical symptoms with systemic inflammatory syndrome in children (MIS-C) which is related to COVID-19. Certain genes are identified to be associated with KD, but the findings usually differ between countries and races. Human Leukocyte Antigen (HLA) allele types and toll-like receptor (TLR) expression are also correlated to KD. The acute hyperinflammation in KD is mediated by an imbalance between augmented T helper 17 (Th17)/Th1 responses with high levels of interleukin (IL)-6, IL-10, IL-17A, IFN-γ, and IP-10, in contrast to reduced Th2/Treg responses with lower IL-4, IL-5, FoxP3, and TGF-β expression. KD has varying phenotypic variations regarding age, gender, intravenous immunoglobulin (IVIG) resistance, macrophage activation and shock syndrome. The signs of macrophage activation syndrome (MAS) can be interpreted as hyperferritinemia and thrombocytopenia contradictory to thrombocytosis in typical KD; the signs of KD with shock syndrome (KDSS) can be interpreted as overproduction of nitric oxide (NO) and coagulopathy. For over five decades, IVIG and aspirin are the standard treatment for KD. However, some KD patients are refractory to IVIG required additional medications against inflammation. Further studies are proposed to delineate the immunopathogenesis of IVIG-resistance and KDSS, to identify high risk patients with genetic susceptibility, and to develop an ideal treatment regimen, such as by providing idiotypic immunoglobulins to curb cytokine storms, NO overproduction, and the epigenetic induction of Treg function.

scholarly journals Phenotype, Susceptibility, Autoimmunity, and Immunotherapy Between Kawasaki Disease and Coronavirus Disease-19 Associated Multisystem Inflammatory Syndrome in Children

2021 ◽  
Vol 12 ◽  
Author(s):  
Men-Ren Chen ◽  
Ho-Chang Kuo ◽  
Yann-Jinn Lee ◽  
Hsin Chi ◽  
Sung Chou Li ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Genetic Susceptibility ◽  
Protective Factor ◽  
Transforming Growth Factor ◽  
Gastrointestinal Symptoms ◽  
Treatment Resistance ◽  
Human Leukocyte ◽  
T Helper 17 ◽  
Inducible Protein ◽  
Inflammatory Syndrome

Coronavirus disease-19 (COVID-19) in children is usually mild but some are susceptible to a Kawasaki disease (KD)-like multisystem inflammatory syndrome in children (MIS-C) in the convalescent stage, posing a need to differentiate the phenotype, susceptibility, autoimmunity, and immunotherapy between KD and MIS-C, particularly in the upcoming mass vaccination of COVID-19. Patients with MIS-C are prone to gastrointestinal symptoms, coagulopathy, and shock in addition to atypical KD syndrome with fever, mucocutaneous lesions, lymphadenopathy, and/or cardiovascular events. MIS-C manifests KD-like symptoms that alert physicians to early recognize and adopt the KD treatment regimen for patients with MIS-C. MIS-C linked to COVID-19 teaches us infection-associated autoimmune vasculitis and vice versa. Studies on genetic susceptibility have identified certain human leukocyte antigen (HLA) locus and toll-like receptor (TLR) associated with KD and/or COVID-19. Certain HLA subtypes, such as HLA-DRB1 and HLA-MICA A4 are associated with KD. HLA-B*46:01 is proposed to be the risk allele of severe COVID-19 infection, and blood group O type is a protective factor of COVID-19. The autoimmune vasculitis of KD, KD shock syndrome (KDSS), or MIS-C is mediated by a genetic variant of HLA, FcγR, and/or antibody-dependent enhancement (ADE) resulting in hyperinflammation with T helper 17 (Th17)/Treg imbalance with augmented Th17/Th1 mediators: interleukin-6 (IL-6), IL-10, inducible protein-10 (IP-10), Interferon (IFNγ), and IL-17A, and lower expression of Treg-signaling molecules, FoxP3, and transforming growth factor (TGF-β). There are certain similarities and differences in phenotypes, susceptibility, and pathogenesis of KD, KDSS, and MIS-C, by which a physician can make early protection, prevention, and precision treatment of the diseases. The evolution of immunotherapies for the diseases has shown that intravenous immunoglobulin (IVIG) alone or combined with corticosteroids is the standard treatment for KD, KDSS, and MIS-C. However, a certain portion of patients who revealed a treatment resistance to IVIG or IVIG plus corticosteroids, posing a need to early identify the immunopathogenesis, to protect hosts with genetic susceptibility, and to combat Th17/Treg imbalance by anti-cytokine or pro-Treg for reversal of the hyperinflammation and IVIG resistance. Based on physiological and pathological immunity of the diseases under genetic susceptibility and host milieu conditions, a series of sequential regimens are provided to develop a so-called “Know thyself, enemy (pathogen), and ever-victorious” strategy for the prevention and immunotherapy of KD and/or MIS-C.

Audiological Patterns in Patients with Autoimmune Hearing Loss

2021 ◽  
pp. 1-10
Author(s):  
George Psillas ◽  
Grigorios G. Dimas ◽  
Michalis Daniilidis ◽  
Paris Binos ◽  
Thomas Tegos ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Clinical Symptoms ◽  
Human Leukocyte ◽  
Progressive Hearing Loss ◽  
Leukocyte Antigen ◽  
Statistical Effect ◽  
Hearing Recovery ◽  
Negative Effect ◽  
Vestibular Symptoms ◽  
Autoimmune Hearing Loss

<b><i>Introduction:</i></b> The aim of this study was to illustrate clinical and audiological patterns of hearing impairment in patients with autoimmune hearing loss (AIHL). <b><i>Methods:</i></b> Fifty-three patients with AIHL were retrospectively recruited, and a tapering schema of steroid treatment was administered in all these patients. The diagnosis of AIHL was essentially based on clinical symptoms, such as recurrent, sudden (sensorineural hearing loss [SSHL]), fluctuating, or quickly progressing (&#x3c;12 months) SSHL (uni-/bilateral), in association with the coexistence of autoimmune diseases, high antinuclear antibodies (ANA) and the presence of human leukocyte antigen (HLA) B27, B35, B51, C04, and C07. Logistic regression analysis was applied to correlate the clinical data and laboratory features of AIHL with final outcomes. <b><i>Results:</i></b> The onset of AIHL was mainly progressive (49%), followed by SSHL (39.6%) or fluctuating (11.3%). The pure-tone audiogram showed more commonly a downsloping pattern (42.6% of ears), but also an upsloping, flat, cookie-bite, or inverse cookie-bite shape. Bilateral progressive AIHL was more frequently simultaneous (23 patients) than heterochronous (4 patients). Nineteen patients (35.8%) showed a favorable response to steroid therapy. The presence of recurrent, bilateral SSHL versus recurrent, unilateral SSHL had statistically negative effect on hearing recovery (OR = 0.042, <i>p</i> &#x3c; 0.05). The heterochronous bilateral SSHL may have better prognosis than simultaneous bilateral SSHL (OR = 10.000, <i>p</i> = 0.099). The gender, age, concomitant autoimmune disease, high ANA, HLA alleles, tinnitus, and vestibular symptoms had no statistical effect on a favorable outcome of AIHL. <b><i>Conclusions:</i></b> A bilateral, simultaneous, and progressive hearing loss combined with downsloping audiogram occurred more often in patients with AIHL. Bilateral simultaneous SSHL with recurrences represents the worse prognostic form of AIHL.

scholarly journals Monocyte human leukocyte antigen-DR but not β-d-glucan may help early diagnosing invasive Candida infection in critically ill patients

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Boris Jung ◽  
Clément Le Bihan ◽  
Pierre Portales ◽  
Nathalie Bourgeois ◽  
Thierry Vincent ◽  
...  
Keyword(s):  
Septic Shock ◽  
Human Leukocyte Antigen ◽  
Critically Ill ◽  
Human Leukocyte ◽  
Candida Infection ◽  
Added Value ◽  
Leukocyte Antigen ◽  
Candida Sp ◽  
Invasive Candida Infection ◽  
Risk Patients

Abstract Background Precision medicine risk stratification is desperately needed to both avoid systemic antifungals treatment delay and over prescription in the critically ill with risk factors. The aim of the present study was to explore the combination of host immunoparalysis biomarker (monocyte human leukocyte antigen-DR expression (mHLA-DR)) and Candida sp wall biomarker β-d-glucan in risk stratifying patients for secondary invasive Candida infection (IC). Methods Prospective observational study. Two intensive care units (ICU). All consecutive non-immunocompromised septic shock patients. Serial blood samples (n = 286) were collected at day 0, 2 and 7 and mHLA-DR and β-d-glucan were then retrospectively assayed after discharge. Secondary invasive Candida sp infection occurrence was then followed at clinicians’ discretion. Results Fifty patients were included, 42 (84%) had a Candida score equal or greater than 3 and 10 patients developed a secondary invasive Candida sp infection. ICU admission mHLA-DR expression and β-d-glucan (BDG) failed to predict secondary invasive Candida sp infection. Time-dependent cause-specific hazard ratio of IC was 6.56 [1.24–34.61] for mHLA-DR < 5000 Ab/c and 5.25 [0.47–58.9] for BDG > 350 pg/mL. Predictive negative value of mHLA-DR > 5000 Ab/c and BDG > 350 pg/mL combination at day 7 was 81% [95% CI 70–92]. Conclusions This study suggests that mHLA-DR may help predicting IC in high-risk patients with septic shock. The added value of BDG and other fungal tests should be regarded according to the host immune function markers.

scholarly journals The Trilogy of SARS-CoV-2 in Pediatrics (Part 2): Multisystem Inflammatory Syndrome in Children

2021 ◽  
Vol 26 (4) ◽  
pp. 318-338
Author(s):  
Van L. Tran ◽  
Sarah Parsons ◽  
Andrew Nuibe
Keyword(s):  
Kawasaki Disease ◽  
Clinical Symptoms ◽  
Gastrointestinal Symptoms ◽  
Febrile Illness ◽  
Current Treatment ◽  
Published Data ◽  
Treatment Considerations ◽  
Cardiac Manifestations ◽  
Effectiveness Studies ◽  
Inflammatory Syndrome

Multisystem Inflammatory Syndrome in Children (MIS-C) was first recognized as a novel illness in 2020 with manifestations similar to other hyperinflammatory syndromes, such as Kawasaki disease or macrophage activation syndrome. Severity varies from a self-limited febrile illness to shock requiring inotropes and mechanical ventilation. Gastrointestinal symptoms and persistent fevers are the most common clinical symptoms, with the addition of cardiac manifestations inclusive of ventricular dysfunction and coronary artery aneurysms. With no controlled trials or comparative effectiveness studies evaluating treatment of MIS-C to date, current treatment with immunomodulatory agents has mainly been derived from previous experience treating Kawasaki disease. This article provides a comprehensive review summarizing published data for the evaluation and management of MIS-C, with a focus on pharmacotherapy treatment considerations.

Effect of initial immunosuppression on long-term kidney transplant outcome in immunological low-risk patients

2018 ◽  
Vol 34 (8) ◽  
pp. 1417-1422 ◽  
Author(s):  
Laura A Michielsen ◽  
Arjan D van Zuilen ◽  
Marianne C Verhaar ◽  
Bram W Wisse ◽  
Elena G Kamburova ◽  
...  
Keyword(s):  
Kidney Transplant ◽  
Graft Survival ◽  
Human Leukocyte ◽  
Low Risk ◽  
Kidney Graft ◽  
Free Survival ◽  
Leukocyte Antigen ◽  
Single Antigen ◽  
Risk Patients

Abstract Background Few studies have evaluated the effect of different immunosuppressive strategies on long-term kidney transplant outcomes. Moreover, as they were usually based on historical data, it was not possible to account for the presence of pretransplant donor-specific human-leukocyte antigen antibodies (DSA), a currently recognized risk marker for impaired graft survival. The aim of this study was to evaluate to what extent frequently used initial immunosuppressive therapies increase graft survival in immunological low-risk patients. Methods We performed an analysis on the PROCARE cohort, a Dutch multicentre study including all transplantations performed in the Netherlands between 1995 and 2005 with available pretransplant serum (n = 4724). All sera were assessed for the presence of DSA by a luminex single-antigen bead assay. Patients with a previous kidney transplantation, pretransplant DSA or receiving induction therapy were excluded from the analysis. Results Three regimes were used in over 200 patients: cyclosporine (CsA)/prednisolone (Pred) (n = 542), CsA/mycophenolate mofetil (MMF)/Pred (n = 857) and tacrolimus (TAC)/MMF/Pred (n = 811). Covariate-adjusted analysis revealed no significant differences in 10-year death-censored graft survival between patients on TAC/MMF/Pred therapy (79%) compared with patients on CsA/MMF/Pred (82%, P = 0.88) or CsA/Pred (79%, P = 0.21). However, 1-year rejection-free survival censored for death and failure unrelated to rejection was significantly higher for TAC/MMF/Pred (81%) when compared with CsA/MMF/Pred (67%, P < 0.0001) and CsA/Pred (64%, P < 0.0001). Conclusion These results suggest that in immunological low-risk patients excellent long-term kidney graft survival can be achieved irrespective of the type of initial immunosuppressive therapy (CsA or TAC; with or without MMF), despite differences in 1-year rejection-free survival.

scholarly journals Human Leukocyte Antigen (HLA) Influence on Prognosis of Autoimmune Hearing Loss

2021 ◽  
Vol 11 (1) ◽  
pp. 31-37
Author(s):  
George Psillas ◽  
Paris Binos ◽  
Grigorios G Dimas ◽  
Michalis Daniilidis ◽  
Jiannis Constantinidis
Keyword(s):  
Hearing Loss ◽  
Human Leukocyte Antigen ◽  
Clinical Symptoms ◽  
Human Leukocyte ◽  
Hla Typing ◽  
Response To Treatment ◽  
Hla B27 ◽  
Hla Alleles ◽  
Leukocyte Antigen ◽  
Autoimmune Hearing Loss

Background: To evaluate the effect of human leukocyte antigen (HLA) on hearing outcome in patients suffering from autoimmune hearing loss (AIHL). Materials and Methods: The diagnosis of AIHL was essentially based on clinical symptoms, such as recurrent, sudden, fluctuating, or quickly progressing (<12 months) sensorineural hearing loss (uni-/bilateral). The molecular typing of HLA alleles was achieved by using polymerase chain reaction procedures. Patients underwent a tapering schema of steroid treatment and audiometric features were recorded. A logistic regression model was used to identify which HLA typing alleles were statistically significant in patients’ response to treatment. Results: Forty patients with AIHL were found to be carriers of HLA B27, B35, B51, C4, C7, and DRB1*04 alleles. No statistically significant influence of HLA B27, B35, B51, C4, C7, DRB1*04 HLA alleles typing was detected for the prognosis of AIHL. In these patients, the onset of AIHL was mainly progressive (53.8%), 29.2% of them had moderate hearing loss, and most of the cases had both bilateral hearing loss (62.5%) and downsloping audiogram (40%). Conclusion: The presence of HLA B27, B35, B51, C4, C7, and DRB1*04 alleles had no significant effect on a favorable outcome of AIHL. However, larger samples of patients are necessary in order to improve the knowledge about the HLA influence on the clinical course of AIHL.

scholarly journals Laboratory parameters between multisystem inflammatory syndrome in children and Kawasaki disease

2021 ◽  
Author(s):  
Chunling Zhou ◽  
Yan Zhao ◽  
Xia Wang ◽  
Ying Huang ◽  
Xuewen Tang ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Inflammatory Markers ◽  
Clinical Symptoms ◽  
Meta Analysis ◽  
Absolute Lymphocyte Count ◽  
Cardiac Markers ◽  
C Reactive Protein ◽  
Laboratory Parameters ◽  
Reactive Protein ◽  
Inflammatory Syndrome

Multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 (COVID-19) has been described to partially overlap with Kawasaki disease (KD) with regard to clinical symptoms, but they are unlikely to share the same disease entity. We conducted a systematic review and meta-analysis to characterize the laboratory parameters of MIS-C compared with those of KD and Kawasaki disease shock syndrome (KDSS). Databases were searched for studies on laboratory parameters of MIS-C (hematology, inflammatory markers, cardiac markers and biochemistry) through May 31, 2021. Twelve studies with 3073 participants yielded 969 MIS-C patients. In terms of hematology, MIS-C patients had lower levels of leukocytes, absolute lymphocyte count and platelet count (PLT) than KD patients and had similar absolute neutrophil count (ANC) and hemoglobin (Hb) levels. In terms of inflammatory markers, MIS-C patients had higher levels of C-reactive protein, D-dimer and ferritin than KD patients and had similar levels of procalcitonin and ESR. In terms of cardiac markers, MIS-C patients had higher CPK levels than KD patients. The levels of NT-proBNP, troponin and AST were not significantly different between MIS-C and KD patients. In terms of biochemistry, MIS-C patients had lower levels of albumin, sodium and ALT and higher levels of creatinine than KD patients. In addition, MIS-C patients had lower levels of PLT, Hb and ESR and higher levels of ANC than KDSS patients. Measurement of laboratory parameters might assist clinicians with accurate evaluation of MIS-C and further mechanistic research.

scholarly journals Coronavirus Disease 2019-Related Multisystem Inflammatory Syndrome in Children: A Systematic Review and Meta-Analysis

2021 ◽  
Vol 2021 ◽  
pp. 1-11
Author(s):  
Ji-Gan Wang ◽  
Zhi-Juan Zhong ◽  
Meng Li ◽  
Jun Fu ◽  
Yu-Heng Su ◽  
...  
Keyword(s):  
Mortality Rate ◽  
Kawasaki Disease ◽  
Fixed Effects ◽  
Clinical Symptoms ◽  
Myocardial Dysfunction ◽  
Clinical Manifestations ◽  
Gastrointestinal Symptoms ◽  
Musculoskeletal Symptoms ◽  
Hands And Feet ◽  
Inflammatory Syndrome

Background. This study aimed to describe the clinical symptoms, laboratory findings, treatment, and outcomes of coronavirus disease 2019-related multisystem inflammatory syndrome in children to provide a reference for clinical practice. Methods. We employed a literature search of databases such as PubMed, Web of Science, EMBASE, and Johns Hopkins University for articles on COVID-19-related multisystem inflammatory syndrome in children published between April 1, 2020, and January 15, 2021. High-quality articles were selected for analysis on the basis of their quality standard scores. Using R3.6.3 software, meta-analyses of random- or fixed-effects models were used to determine the prevalence of comorbidities. Subgroup analysis was also performed to determine heterogeneity. Results. A total of 57 articles (2,290 pediatric patients) were included in the study. Clinical Manifestations. :ncidences of fever, gastrointestinal symptoms, respiratory symptoms, and musculoskeletal symptoms (myalgias or arthralgias) were 99.91% (95% CI: 99.67–100%), 82.72% (95% CI: 78.19–86.81%), 53.02% (45.28–60.68%), and 14.16% (95% CI: 8.4–21.12%), respectively. The incidences of rash, conjunctival injection, lymphadenopathy, dry cracked lips, neurologic symptoms (headache, altered mental status, or confusion), swollen hands and feet, typical Kawasaki disease, and atypical Kawasaki disease were 59.34% (95% CI: 54.73–63.87%), 55.23% (95% CI: 50.22–60.19%), 27.07% (95% CI: 19.87–34.93%), 46.37% (95% CI: 39.97–52.83%), 28.87% (95% CI: 22.76–35.40%), 28.75% (95% CI: 21.46–36.64%), 17.32% (95% CI: 15.44–19.29%), and 36.19% (95% CI: 21.90–51.86%), respectively. The incidences of coronary artery dilation, aneurysm, pericardial effusion, myocarditis, myocardial dysfunction, high troponin, and N-terminal pro-B-type natriuretic peptide were 17.83%, 6.85%, 20.97%, 35.97%, 56.32%, 76.34%, and 86.65%, respectively. The incidences of reduced lymphocytes, thrombocytopenia, hypoalbuminemia, elevated C-reactive protein, ferritin, LDH, interleukin-6, PCT, and FIB were 61.51%, 26.42%, 77.92%, 98.5%, 86.79%, 80.59%, 89.30%, 85.10%, and 87.01%, respectively. PICU Hospitalization Rate and Mortality. The incidences of PICU hospitalization or with shock were 72.79% and 55.68%, respectively. The mortality rate was 1.00%. Conclusion and Relevance. PICU hospitalization and shock rates of multisystem inflammatory syndrome in children associated with COVID-19 were high, and its cumulative multiorgans and inflammatory indicators are increased, but if treated in time, the mortality rate was low.

scholarly journals COVID-19 and Multisystem Inflammatory Syndrome in Children: A Systematic Review and Meta-analysis

2020 ◽  
Author(s):  
Jun Yasuhara ◽  
Kae Watanabe ◽  
Hisato Takagi ◽  
Naokata Sumitomo ◽  
Toshiki Kuno
Keyword(s):  
Kawasaki Disease ◽  
Clinical Symptoms ◽  
Myocardial Dysfunction ◽  
Meta Analysis ◽  
Gastrointestinal Symptoms ◽  
Multiple Organ ◽  
Coronary Abnormalities ◽  
Cardiovascular Involvement ◽  
Distinct Features ◽  
Inflammatory Syndrome

Background: Multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 has been increasingly recognized. However, the clinical features of MIS-C and the differences from Kawasaki disease remain unknown. The study aims to investigate the epidemiology and clinical course of MIS-C. Methods: PubMed and EMBASE were searched through August 30, 2020. Observational studies describing MIS-C were included. Data regarding demographic features, clinical symptoms, laboratory, echocardiography and radiology findings, treatments, and outcomes were extracted. Study-specific estimates were combined using one-group meta-analysis in a random-effects model. Results: A total of 27 studies were identified including 917 MIS-C patients. The mean age was 9.3 (95% confidence interval [CI], 8.4-10.1). The pooled proportions of Hispanic and Black cases were 34.6% (95% CI, 28.3-40.9) and 31.5% (95% CI, 24.8-38.1), respectively. The common manifestations were gastrointestinal symptoms (87.3%; 95% CI, 82.9-91.6) and cardiovascular involvement such as myocardial dysfunction (55.3%; 95% CI, 42.4-68.2), coronary artery aneurysms (21.7%; 95% CI, 12.8-30.1) and shock (65.8%; 95% CI, 51.1-80.4), with marked elevated inflammatory and cardiac markers. The majority of patients received intravenous immunoglobulin (81.0%; 95% CI, 75.0-86.9), aspirin (67.3%; 95% CI, 48.8-85.7), and corticosteroids (63.6%; 95% CI, 53.4-73.8) with a variety of anti-inflammatory agents. Although myocardial dysfunction improved in 55.1% (95% CI, 33.4-76.8) at discharge, the rate of extracorporeal membrane oxygenation use was 6.3% (95% CI, 2.8-9.8) and the mortality was 1.9% (95% CI, 1.0-2.8). Conclusion: Our findings suggest that MIS-C leads to multiple organ failure, including gastrointestinal manifestations, myocardial dysfunction and coronary abnormalities, and has distinct features from Kawasaki disease.

scholarly journals Multi-System Inflammatory Syndrome in a Child Mimicking Kawasaki Disease

2020 ◽  
Author(s):  
Aman Gupta ◽  
Arpinder Gill ◽  
Manu Sharma ◽  
Megha Garg
Keyword(s):  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Early Recognition ◽  
Clinical Manifestations ◽  
Case Definitions ◽  
Laboratory Parameters ◽  
Life Saving ◽  
Inflammatory Syndrome ◽  
Activation Syndrome

Abstract There have been recent reports of children presenting with severe multi-system hyperinflammatory syndrome resembling Kawasaki disease (KD) during current COVID-19 pandemic. Exact pathophysiology is unknown, however, most of the children have multi-organ dysfunction and respiratory system involvement is less common compared to adults. These patients have certain characteristic laboratory parameters different from those seen in children with KD. However, only limited literature is available at present for identification and management of such patients. We report a young girl who presented with fever, rash and other manifestations mimicking classic KD and fulfilling the case definitions for pediatric multi-system inflammatory syndrome. She had lymphopenia, thrombocytopenia and hyponatremia in the absence of macrophage activation syndrome, similar to that seen in patients reported from UK and Italy. Clinical manifestations resolved and laboratory parameters improved with intravenous immunoglobulin and corticosteroids. Early recognition is important to administer immunomodulatory therapy which may be life saving for these patients.

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