Northern Italy is the second region hit by the SARS-COV2 infection worldwide. Data onCOVID-19 clinical presentation in children is still scarce, but fewer rate of infectionand milder disease seem typical of this age group. In the last three weeks it has beenreported an abnormal number of critically ill patients with clinical characteristicsconsistent with Kawasaki Shock Syndrome (KSS). The common manifestations are: "middleaged" children (6-9 y/o) with a history of persistent high spiking fever in the lastdays, abdominal pain, diarrhea, skin rash and rapidly deteriorating clinical conditionwith the onset of shock, without clear signs of dehydration. Other less common featuresare arthralgia, cough, meningism, conjunctivitis and reddened, cracked lips. Labworksusually show high inflammatory markers, low lymphocyte counts, low sodium, and hightroponin levels. Echocardiography have been consistent with myocarditis in the majorityof patient instead of classical coronary artery abnormalities. Patients have beendiagnosed as Kawasaki disease (typical or incomplete) and treated accordingly withIntraVenous ImmunoGlobulin (IVIG) and/or steroids. One patient refractory to suchtreatments responded successfully to intravenous Anakinra. All the patients reported afamily history consistent with COVID-19, serology and naso-pharyngeal swabs wereinconsistently positive. To date we are aware of at least 10 such cases. KSS is a rareand dreadful complication, with an estimated prevalence of 5% of patients with KawasakiDisease (KD). Given the extreme rarity of this condition, the occurrence of so many casesin the last weeks points to a possible causative agent. As our hospitals are in highendemic area, SARS-COV2 seems the most obvious, although testing for such infection inpatients returned conflicting results. It is not clear, at this moment, if this clinicalentity is a proper KD triggered by SARS-COV2, or a systemic vasculitis with similarfeatures of KD, secondary to SARS-COV2 infection. The aim of this nationwide study is tobetter define this clinical entity.
Not Provided
Other: Clinical evaluation
Evaluation of clinical manifestation, laboratory ad imaging testing results, disease
course, treatment and outcome
Inclusion Criteria:
- All children with clinical diagnosis of Kawasaki Disease
- age<18 years
- Absence of other underlying chronic diseases
Exclusion Criteria:
- Presence of underlying chronic diseases
- Not fulfilling clinical criteria for Kawasaki Disease diagnosis
Ospedale Pediatrico Meyer
Florence 3176959, Italy
IRCCS Istituto Giannina Gaslini
Genova 8969657, Italy
Ospedale Pediatrico Bambino Gesù
Roma 8957247, Italy
IRCCS Burlo Garofolo
Trieste 3165185, Italy
Andrea Taddio, MD, Study Director
IRCCS materno infantile Burlo Garofolo