Dallas, TX—The American Heart Association recently sought to reassure parents that it remains rare for children to experience Kawasaki-like disease tied to the COVID-19 pandemic.

A press release points out that, while most children infected with the novel coronavirus are asymptomatic or exhibit only mild symptoms, a small number of children in the United States and elsewhere developed a more serious inflammatory syndrome with COVID-19 in the past 2 months. Most were hospitalized, some required intensive care, and a few died.

Children with the condition have a persistent fever, inflammation, and evidence of single or multi-organ dysfunction—shock, cardiac, respiratory, renal, gastrointestinal or neurological disorder—and might or might not test positive for COVID-19, according to the AHA.

“We want to reassure parents—this appears to be uncommon. While Kawasaki disease can damage the heart or blood vessels, the heart problems usually go away in five or six weeks, and most children fully recover,” explained Jane Newburger, MD, MPH, of the Kawasaki Program at Boston Children’s Hospital; and Commonwealth Professor of Pediatrics at Harvard Medical School. “Rarely, but sometimes, the coronary artery damage persists. Because of this, Kawasaki disease is the most common cause of acquired heart disease in children in developed countries. Prompt treatment is critical to prevent significant heart problems.”

Earlier in May, New York notified other states of the apparent COVID-related illness in children. New York public health officials said they were investigating more than 80 reported cases where children—predominantly school-aged—were experiencing symptoms similar to an atypical Kawasaki disease or a toxic shock-like syndrome possibly due to COVID-19. As of May 12, three young people had died, and two additional deaths were under investigation.

In addition, more than 50 cases were reported in European countries and the United Kingdom.

Boston Children’s Hospital recently convened a Zoom conference for clinicians to compare notes on what is being called “Pediatric Multi-System Inflammatory Syndrome Potentially Associated with COVID-19.” On May 2, the International PICU-COVID-19 panelists reported that some children presented with some or all features seen in Kawasaki disease, which can result in enlargement or aneurysms of the coronary arteries. Symptoms included fever, rash, conjunctivitis; red, swollen hands; and red, cracked lips. In addition, some children had clinical and laboratory signs of cytokine storm syndrome, an exaggerated systemic immune response that has caused organ damage in adults with COVID-19.

Many of the children also had coagulopathies, cardiac dysfunction, diarrhea, abdominal distension, and other GI symptoms—with some children having positive stool tests for SARS-CoV-2—or acute kidney injury. Respiratory symptoms were not always a prominent feature, according to the report.

“We could be dealing with several phenotypes on a spectrum,” explained Boston Children’s Hospital rheumatologist Mary Beth Son, MD, who was one of the conference panelists. “In some cases, children present with shock and some have features of Kawasaki disease, whereas others may present with signs of cytokine storm. In some geographic areas, there has been an uptick in Kawasaki disease cases in children who don’t have shock.”

Cases in the U.S. so far have been mainly in East Coast cities, with some in the Midwest and South. According to conference attendees, most children affected have done well so far, with treatments including anticoagulation, IV immunoglobulin, IL-1 or IL-6 blockade, and corticosteroids, as well as supportive care.

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