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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 7  |  Issue : 6  |  Page : 349-351

Multisystem inflammatory syndrome in children: A case report from India


1 Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India
2 Department of Laboratory Oncology, All India Institute of Medical Sciences, New Delhi, India

Date of Submission08-Aug-2020
Date of Decision26-Aug-2020
Date of Acceptance05-Sep-2020
Date of Web Publication11-Nov-2020

Correspondence Address:
Dr. Jhuma Sankar
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JPCC.JPCC_126_20

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  Abstract 


With reports of a multisystem inflammatory syndrome in children (MIS-C) related to COVID-19 infection resembling Kawasaki disease, there is an increasing need for awareness of this condition, especially in resource-limited settings. Herein, we report a 6-year-old previously healthy boy, who presented with a short history of fever, rash, and difficulty in breathing. At admission, he had bilateral nonexudative conjunctival congestion, oral erythema, maculopapular rash, and hemodynamic instability. Investigations showed neutrophilic leukocytosis, elevated inflammatory markers, and cardiac dysfunction. The child was managed with invasive ventilatory support, vasoactive agents, and broad-spectrum antibiotics and was given intravenous immunoglobulins and steroids. Reverse transcription-polymerase chain reaction for SARS-CoV-2 was negative; however, antibody test for SARS-CoV-2 was positive, confirming the diagnosis of MIS-C. The child improved clinically, inflammatory markers decreased, and the child could be discharged after 10 days of hospital stay.

Keywords: COVID-19, Kawasaki disease, multisystem inflammatory syndrome


How to cite this article:
Prakash S, Kumar BK, Dwivedi T, Sankar J. Multisystem inflammatory syndrome in children: A case report from India. J Pediatr Crit Care 2020;7:349-51

How to cite this URL:
Prakash S, Kumar BK, Dwivedi T, Sankar J. Multisystem inflammatory syndrome in children: A case report from India. J Pediatr Crit Care [serial online] 2020 [cited 2020 Nov 25];7:349-51. Available from: http://www.jpcc.org.in/text.asp?2020/7/6/349/300578




  Introduction Top


COVID-19 pandemic has had a devastating global impact. Nonetheless, children have been relatively spared, both in terms of incidence and severity.[1] However, numerous reports from Europe and the USA have described a novel syndrome in children with some similarities to Kawasaki disease (KD), toxic shock syndrome (TSS), and other hyperinflammatory conditions.[2],[3],[4] This syndrome, commonly termed as a multisystem inflammatory syndrome in children (MIS-C), is characterized by fever, myocardial dysfunction with or without shock, prominent gastrointestinal involvement, and elevated inflammatory markers with an epidemiological and serological association with COVID-19. We report a case of a 6-year-old boy with fever, shock, and multisystem involvement, with positive antibodies against SARS CoV-2 from New Delhi, India.


  Case Report Top


A previously healthy 6-year-old boy presented to the emergency department (ED) with fever for 5 days, rash for 3 days, lethargy and poor feeding for 2 days, and shortness of breath for a day. At presentation to ED, the child had tachypnea (respiratory rate: 35/min) with increased respiratory efforts, tachycardia (150/min), and signs of poor peripheral perfusion. Initially, the child was in compensated shock and progressed rapidly to hypotensive shock. On examination, the child had bilateral nonexudative conjunctival congestion; oral mucosal erythema; diffuse blanchable erythema over the face, trunk, and limbs; and bilateral cervical lymphadenopathy (1.5 cm). The child was managed with a fluid bolus followed by adrenaline infusion. The child was ventilated because of worsening respiratory distress and shock and was transferred to the pediatric intensive care unit (PICU). Possibilities considered were KD shock syndrome, MIS-C, TSS, and septic shock.

Laboratory investigations revealed anemia (hemoglobin: 9 g/dL), neutrophilic leukocytosis (total leukocyte count: 27,000/mm3, N82% L14% M4%), thrombocytopenia (1.4 lakh/mm3), normal renal parameters, hypoalbuminemia (1.8 g/dL), and mildly deranged liver enzymes (aspartate transaminase: 75 IU/L and alanine transaminase: 36 IU/L). Inflammatory markers were significantly elevated (erythrocyte sedimentation rate: 35 mm/h [0–15 mm/h], C-reactive protein: 258 mg/L [0–6 mg/dl], procalcitonin >100 ng/ml [0–0.15 ng/ml], and ferritin: 1873 ng/ml [21.8–274.6 ng/ml]). Echocardiography was suggestive of mild left ventricular dysfunction (ejection fraction: 45%–50%) with normal coronaries. Troponin I level was 15.7 pg/ml (0–20 pg/ml). Chest X-ray showed cardiomegaly (cardiothoracic ratio: 54%) [Figure 1], and ultrasonography abdomen revealed gallbladder edema and mild ascites. Endotracheal aspirate for reverse transcription-polymerase chain reaction (RT-PCR) for SARS-CoV-2 was negative. Workup for other infectious etiologies (blood culture, malaria workup, and dengue IgM) was not contributory.
Figure 1: Chest X ray showing cardiomegaly (cardiothoracic ratio: 54%)

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In view of the clinical constellation of symptoms consistent with KD, intravenous immunoglobulin (IVIG) was given at a dose of 2 g/kg over 24 h. The child was started on broad-spectrum intravenous antibiotics (ceftriaxone and vancomycin). There was an initial worsening of shock over the first 24 h requiring vasoactive support of adrenaline, noradrenaline, and milrinone. Hydrocortisone was added in view of refractory shock (100 mg/m2/day). Hemodynamics gradually improved, and inotropes could be tapered and stopped by 72 h of hospital stay. High-grade fever was noted initially but resolved within 48 h. The child had abdominal distension with bilious aspirates during the initial 3 days of hospital stay, which was managed conservatively. The child could be extubated to the high-flow nasal cannula by day 4, and oxygen was stopped by day 7. Repeat echocardiography done on day 7 showed normal cardiac function and normal coronaries. There was a concomitant decrease in the inflammatory markers.

Antibody testing for SARS-CoV-2 (IgM and IgG) done on day 13 of illness using chemiluminescent immunoassay was positive (SARS COV2T [Siemens]: >10 index; >1 index is reactive). With the clinical picture compatible with MIS-C, residence from a COVID hotspot area, and serology positive for the virus, the final diagnosis of MIS-C was established. The child remained clinically stable subsequently and was discharged on aspirin and oral steroids (1 mg/kg/day with a plan to taper over 2 weeks) after 10 days of hospital stay.


  Discussion Top


Since the first description of MIS-C, much interest has been generated in understanding the epidemiology and pathophysiology of this condition. Our case fulfills the case definition proposed for MIS-C by the WHO[5] and the Centers for Disease Control and Prevention, as well as the diagnostic criteria for KD.

Previous reports have shown that around 1/3 to 1/2 of such children with MIS-C have fulfilled diagnostic criteria for KD.[6],[7] MIS-C differs from KD by various features including older age of presentation, predominant gastrointestinal involvement and cardiovascular involvement, and more severe inflammation. Furthermore, previous reports have highlighted the poor response of MIS-C to IVIG and the need for adjunct steroids to improve outcomes. In the index case, the child responded well to IVIG and steroids.

As in KD, several hypotheses have been proposed to explain the cytokine storm seen in MIS-C. The most likely explanation based on the current understanding is that it is a postinfectious process, an aberrant immune-mediated delayed cytokine response to an already cleared SARS-CoV-2 from the body. This is supported by the observation that antibodies against SARS-CoV-2 are detected in a majority of patients, but only a minority have a positive RT-PCR. Other possible hypotheses suggest that it is due to delayed interferon-induced cytokine storm in patients with a high viral load or some genetic factors due to an initial blockage of Type I and Type III interferon responses.[8]

Our case highlights the favorable outcome seen in children with MIS-C, provided it is recognized early, and appropriate interventions are instituted. Very few cases of MIS-C have been reported from outside of Europe and the USA.[9],[10] Whether this is due to an actual difference in the incidence of this syndrome due to genetic/environmental factors or a failure to detect enough cases or due to a delayed peak of SARS-CoV-2 is not clear. As COVID-19 pandemic has peaked later in the South East Asian region, a greater number of cases may be seen in the near future. In addition, the natural history of MIS-C in the Indian subcontinent needs to be established, given the genetic and environmental differences.


  Conclusion Top


With the increasing number of SARS-CoV-2 cases in the Indian subcontinent, clinicians should be aware of this increasingly recognized syndrome, and appropriate, timely referral to a center with adequate expertise will possibly improve outcomes in these children.

Acknowledgment

We acknowledge the contributions of PICU residents and nursing staff for their valuable support in managing the case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ludvigsson JF. Systematic review of COVID-19 in children shows milder cases and a better prognosis than adults. Acta Paediatr 2020;109:1088-95.  Back to cited text no. 1
    
2.
Kaushik S, Aydin SI, Derespina KR, Bansal PB, Kowalsky S, Trachtman R, et al. Multisystem Inflammatory Syndrome in Children (MIS-C) associated with SARS-CoV-2 infection: A multi-institutional study from New York City. J Pediatr 2020;224:24-9.  Back to cited text no. 2
    
3.
Belhadjer Z, Méot M, Bajolle F, Khraiche D, Legendre A, Abakka S, et al. Acute Heart Failure in Multisystem Inflammatory Syndrome in Children in the Context of Global SARS-CoV-2 Pandemic. Circulation 2020;142:429-36.  Back to cited text no. 3
    
4.
Whittaker E, Bamford A, Kenny J, Kaforou M, Jones CE, Shah P,et al. Clinical Characteristics of 58 Children with a Pediatric Inflammatory Multisystem Syndrome Temporally Associated with SARS-CoV-2. JAMA. 2020;324:259-69. Doi:10.1001/jama.2020.10369.  Back to cited text no. 4
    
5.
Multisystem Inflammatory Syndrome in Children and Adolescents Temporally Related to COVID-19. Available from: https://www.who.int/news-room/commentaries/detail/multisystem-inflammatory-syndrome-in-children-and-adolescents-with-covid-19. [Last accessed on 2020 Jun 27].  Back to cited text no. 5
    
6.
Cheung EW, Zachariah P, Gorelik M, Boneparth A, Kernie SG, Orange JS, et al. Multisystem Inflammatory Syndrome Related to COVID-19 in Previously Healthy Children and Adolescents in New York City. JAMA. 2020;324:294-96. Doi:10.1001/jama.2020.10374.  Back to cited text no. 6
    
7.
Verdoni L, Mazza A, Gervasoni A, Martelli L, Ruggeri M, Ciuffreda M, et al. An outbreak of severe Kawasaki-like disease at the Italian epicentre of the SARS-CoV-2 epidemic: An observational cohort study. Lancet 2020;395:1771-8.  Back to cited text no. 7
    
8.
Rowley AH. Understanding SARS-CoV-2-related multisystem inflammatory syndrome in children. Nat Rev Immunol 2020;20:1-2.  Back to cited text no. 8
    
9.
Rauf A, Vijayan A, John ST, Krishnan R, Latheef A. Multisystem Inflammatory Syndrome with Features of Atypical Kawasaki Disease during COVID-19 Pandemic. Indian J Pediatr 2020; 87:745-7. https://doi.org/10.1007/s12098-020-03357-1.  Back to cited text no. 9
    
10.
Balasubramanian S, Nagendran TM, Ramachandran B, Ramanan AV. Hyper-inflammatory syndrome in a child with COVID-19 treated successfully with intravenous immunoglobulin and tocilizumab. Indian Pediatr 2020;57:681-3.  Back to cited text no. 10
    


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