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Case definition: Inflammatory syndrome in children linked to Covid-19

How to identify paediatric multisystem inflammatory syndrome temporally associated with Covid-19

This RCPCH document for clinicians, which has been developed after expert review of the cases, includes a case definition and approach to clinical management.

Most children are asymptomatic or exhibit mild symptoms from Covid-19 infection. However, in the last two months a small number of children have been identified who develop a significant systemic inflammatory response.

All children have been diagnosed and managed appropriately along standard referral pathways. Affected children may require paediatric intensive care and input from paediatric infectious diseases, cardiology, and rheumatology.

This rare syndrome shares common features with other paediatric inflammatory conditions including: Kawasaki disease, staphylococcal and streptococcal toxic shock syndromes, bacterial sepsis and macrophage activation syndromes.

It can also present with unusual abdominal symptoms with excessive inflammatory markers. Early recognition by paediatricians and specialist referral including to critical care is essential. Advice currently given to families and carers of children and young people (for example RCPCH parent advice during Covid-19 leaflet) supports appropriate referral to health services. This document is to raise awareness and gives management advice to clinicians and has been developed after expert review of the cases.

Case definition:

1. A child presenting with persistent fever, inflammation (neutrophilia, elevated CRP and lymphopaenia) and evidence of single or multi-organ dysfunction (shock, cardiac, respiratory, renal, gastrointestinal or neurological disorder) with additional features (see listed in Appendix 1 ). This may include children fulfilling full or partial criteria for Kawasaki disease.

2. Exclusion of any other microbial cause, including bacterial sepsis, staphylococcal or streptococcal shock syndromes, infections associated with myocarditis such as enterovirus (waiting for results of these investigations should not delay seeking expert advice).

3. SARS-CoV-2 PCR testing may be positive or negative

All stable children should be discussed as soon as possible with specialist services to ensure prompt treatment (paediatric infectious disease / cardiology / rheumatology*). There should be a low threshold for referral to Paediatric Intensive Care using normal pathways.

Source: Royal College of Paediatrics and Child Health, Guidance: Paediatric multisystem inflammatory syndrome temporally associated with Covid-19 [publlished 1 May]