WHO develops preliminary case definition for inflammatory syndrome in children

Evelyn Lewin

19/05/2020 3:04:42 PM

The COVID-19-associated syndrome seems to be its own unique entity.

Parent putting face mask on child.
The individual risk to children in Australia at the moment is low, but this may change.

Concern is growing regarding an inflammatory syndrome affecting children and adolescents with COVID-19.
Now known as multisystem inflammatory syndrome in children (MIS-C), the condition has also been referred to as Paediatric Inflammatory Multisystem Syndrome Temporally associated with SARS-CoV-2 (PIMS-TS).
It was reported on 16 May that medical professionals in the US are investigating more than 200 confirmed or suspected cases of MIS-C, and that three children in New York had died as a result of the syndrome.
Reports of the condition have also emerged elsewhere, with the BBC reporting on 14 May that up to 100 children in the UK had been affected, with studies in Europe suggesting the same reaction is occurring too.
‘The CDC [Centers for Disease Control and Prevention] has confirmed a link to COVID-19,’ New York City Mayor Bill de Blasio said on 18 May.
‘This is important. We assumed it, but they have done additional research to 100% confirm it and released a national standard definition.’
The World Health Organization (WHO) has now developed a preliminary case definition for this condition, announced via a scientific brief on 15 May.
The case definition is children and adolescents aged 0–19 with fever for more than three days who have two of the following: 

  • Rash or bilateral non-purulent conjunctivitis or muco-cutaneous inflammation signs (oral, hands or feet)
  • Hypotension or shock
  • Features of myocardial dysfunction, pericarditis, valvulitis, or coronary abnormalities (including ECHO findings or elevated Troponin/NT-proBNP)
  • Evidence of coagulopathy (by PT, PTT, elevated d-Dimers)
  • Acute gastrointestinal problems (diarrhoea, vomiting, or abdominal pain)
  • Elevated markers of inflammation such as erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), or procalcitonin 
  • No other obvious microbial cause of inflammation, including bacterial sepsis, staphylococcal or streptococcal shock syndromes 
  • Evidence of COVID-19 (RT-PCR, antigen test or serology positive), or likely contact with patients with COVID-19 
The WHO stated the case definition will be revised as more data becomes available.
According to the CDC, MIS-C may begin weeks after SARS-CoV-2 infection.
‘The child may have been asymptomatically infected and, in some cases, the child and their caregivers may not even know they had been infected,’ the CDC stated.
Professor David Burgner is a paediatric infectious diseases clinician scientist at the Murdoch Children’s Research Institute (MCRI) and medical advisor to the Kawasaki Disease Foundation Australia.
He told newsGP this syndrome seems to only occur in populations that have high levels of community transmission, and that currently there have been no confirmed cases of this in Australia.
‘The US – particularly New York – Italy, France and the UK are the places that I’m aware it’s been formally reported from, but it may be occurring in more places with high background rates of community transmission,’ he said.

Paediatric infectious diseases clinician scientist Professor David Burgner. 

Professor Burgner therefore believes there is no need for undue alarm over the syndrome in Australia.
‘The level of concern at the moment should be low,’ he said.
‘We have to keep it in perspective; that’s a message we’re very keen to get across – that the individual risk to children in Australia at the moment must be very low.
‘If we do get a significant second wave of community transmission then we may see some cases, but the estimate is that there’ll be relatively few.’
Professor Burgner said this syndrome has been likened to both Kawasaki disease (KD) and toxic shock syndrome (TSS), but he believes the COVID-19-related syndrome ‘sits somewhere between’ the two and is its own unique syndrome.
‘The trouble with all these syndromes is there’s no diagnostic test; they’re just a series of clinical features,’ he said.
‘So it’s really hard to say where one ends and one begins. It does look like there are features that certainly fit with KD, but there are features there that are quite distinct and that we don’t see commonly with KD.
‘We’ve never really seen something like this, with these exact features with another respiratory virus.
‘Although the ground shifts under our feet with COVID-19 all the time, it looks like it’s related to a distinct entity in [terms of] its epidemiology, clinical presentation and laboratory characteristics.’
In terms of which symptoms predominate in MIS-C, Professor Burgner said all of the studies on the syndrome have been via ‘very small’ case series.
‘So it’s very hard to generalise,’ he said.
Features that differentiate MIS-C from other known syndromes include the predominance of abdominal symptoms at presentation, the increased presence of myocardial dysfunction, and the increased likelihood for such patients to go into shock.
While the syndrome is rare, Professor Burgner said it may ‘disproportionately’ affect children of African and Afro-Caribbean background.
‘I suspect this is a unique gene environment interaction,’ he said.
‘It’s possible there’s a genetic variant in these children and that, together with this new trigger, is giving this syndrome that looks phenotypically like, but not exactly like, other syndromes.’
As concern grows, Professor Burgner is keen to note there is ongoing surveillance in major emergency departments for KD, and that the PAEDS network is actively looking for MIS-C cases.
While Professor Burgner believes it is unlikely we will see many, if any, such cases in Australia, he would like to convey a message to GPs.
‘Be alert, but not alarmed,’ he said.
‘So if you see a child who’s got fever, marked abdominal symptoms and maybe has a rash and looks unwell, send them to a paediatric centre urgently because this is something we do need to bear in mind now.
‘It’s pretty unlikely that any given GP will see something like this, but doctors do need to be aware of it.’
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