PMC:7289100 / 19547-23657 JSONTXT

{"target":"https://pubannotation.org/docs/sourcedb/PMC/sourceid/7289100","sourcedb":"PMC","sourceid":"7289100","source_url":"https://www.ncbi.nlm.nih.gov/pmc/7289100","text":"Northern Italy was the first European region to be severely hit by the SARS-COV2 epidemic after the original outbreak in China [67]. Although the data on the clinical presentation of COVID-19 in children is still scarce, lower rates of infection and milder forms of disease seem to be typical of this age group [68,69]. Since the end of March 2020, we observed and treated or received information from all over Italy about an abnormally elevated number of critically ill patients with clinical characteristics consistent with Kawasaki Shock Syndrome (KSS). The common features were: “middle-aged” children (6–9 y/o) with a history of persistent high spiking fever in the previous days, abdominal pain, diarrhea, skin rash, and rapidly deteriorating clinical conditions with the onset of shock, without clear signs of dehydration. Other less common features were arthralgia, cough, meningism, conjunctivitis and reddened, cracked lips. Labworks usually showed high inflammatory markers, low lymphocyte count, low sodium, and high troponin and pro-BNP levels. Echocardiography was consistent with myocarditis in the majority of patients instead of classical coronary artery abnormalities. Patients were diagnosed as Kawasaki disease (typical or incomplete) and treated accordingly with IVIG and/or steroids. One patient refractory to such treatments responded successfully to intravenous anakinra. Althoug all the patients had family contacts consistent with COVID-19, serology and nasopharyngeal swabs were inconsistently positive both in the single patients and among all children. To date we are aware of at least 10 cases of KSS possibly related to COVID-19. Given the unprecedented cluster of patients we decided to launch a nationwide alert for pediatricians and start a national registry. Few days after the diagnosis of our first cases, news from all over Europe first, and Eastern USA thereafter, reported on the identification of patients with remarkably the same characteristics we have observed. Some children with a less severe picture, without shock at the onset, but with clinical characteristics of systemic inflammation and some features of KD have also been seen by us and others (personal communications). These observations lead to a variety of nationwide alerts in the respective countries [70,71]. On May 7, 2020, Riphagen et al. published on 8 patients treated at the South Thames Retrieval Service for a severe acute illness similar to KSS [72]. Age at presentation was between 4 and 14 years, 6 out of 8 children were of Afro-Caribbean descent and 5 were boys. All the children had a similar disease onset with persistent fever, skin rash, conjunctivitis, peripheral oedema, and extremity pain. Gastrointestinal symptoms were frequent and severe, all children developed shock requiring ICU admission and vasopressors. Another key feature described in those patients was the cardiac involvement, as testified by very elevated cardiac enzymes, and echo-bright coronary vessels. One patient developed a giant coronary aneurism. Lab works typically showed high C-reactive protein, procalcitonin, ferritin, triglycerides, and D-dimers. All children were treated with IVIG (2 g/kg) and 6 also received aspirin (50 mg/kg). Broncho-alveolar lavage or nasopharyngeal aspirates were negative for SARS-COV-2 in all children; some of the patients had histories of familial contact with the virus. The authors stated that, at the time they were writing their report, another ICU in London managed 20 patients with similar characteristics. As per the Royal College of Pediatrics and Child Health (RCPCH) alert, some of these patients resulted to be positive for SARS-CoV-2 infection, by either nasopharyngeal swab, serology, or both [69]. The New York State Department of Health reported sixty-four cases as of May 5th 2020. For the purpose of this review we will refer to this clinical entity as Pediatric COVID-19 Hyperinflammatory Syndrome (PeCOHS), while the English authorities refers to it as Pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 infection 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