Rare illness may be new COVID-19 symptom for children

Only three children have been admitted to UPMC Children’s Hospital of Pittsburgh with COVID-19 infections, two being released within 24 hours and the third staying a few days but never requiring ventilation.

And yet local pediatricians remain on high alert about a potentially COVID-19-related inflammatory syndrome similar to Kawasaki disease that has reportedly affected about 100 children in New York, with three deaths.

“This is an incredibly rare condition, but we’ve done really

well here,” said Dr. Joseph Aracri, chair of Allegheny Health Network Pediatrics. “But we need to be vigilant knowing about the complications that are occurring out there” — in New York City, the United Kingdom and elsewhere.

Concern first arose in late April when the U.K. issued an alert about a condition — now known as “pediatric multi-system inflammatory syndrome” — that’s potentially associated with COVID-19. That condition was causing a small rise in the number of critically ill children in England.

The alert cited conditions of toxic-shock syndrome and an incomplete version of Kawasaki disease, “with some children experiencing gastrointestinal symptoms and cardiac inflammation.”

That alert was followed by New York City health officials raising concern initially about 15 cases that have since risen to about 100.

On May 2, the International PICU-COVID-19 Collaboration held a conference call to compare notes, according to a statement issued by Boston Children’s Hospital.

“Pediatric experts in intensive care, cardiology, rheumatology, infectious disease, and Kawasaki disease reviewed data from several dozen cases in Europe and the U.S., offered guidance for clinicians, and laid out an agenda for research,” it said.

Symptoms that were noted included persistent fever, inflammation and poor function in one or more organs, among symptoms that could not be linked to other infections.

“Many affected children were hospitalized and some required intensive care due to a clinical picture consistent with shock,” the hospital statement said.

The mysterious illness is rare, but the hospital statement advised that clinicians who suspect such a case “should consult promptly with pediatric infectious disease, rheumatology, or critical care specialists.”

“Because some children get sicker rapidly, they should be cared for in hospitals with tertiary pediatric [and] cardiac intensive care units,” it said.

Ever since the local pandemic began, about 300 children have been hospitalized at UPMC Children’s Hospital of Pittsburgh with symptoms similar to those caused by COVID-19, said Dr. Terence Dermody, physician-inchief and scientific director at Children’s. But, surprisingly only three turned out to test positive for the virus.

That rate of one COVID-19 case per 100 children seeking hospital care compares to the experience of Seattle Children’s, he said, that had four COVID-19 positives among 700 children treated there by mid-March for various infections.

“At [UPMC] Children’s we haven’t seen a case of COVID-19 in three weeks,” he said. “That’s absolutely amazing. Our worry is less about COVID-19 and more about children who have deferred care and need to come in to see us” for such conditions as asthma, diabetes and cancer, among others.

For now, the hospital has seen no cases of pediatric multi-system inflammatory syndrome, or PMIS. In other cities, such cases seem to be associated with COVID-19 with no proof, to date, that coronavirus is causing them.

Dr. Dermody said he’s proud that local leadership set guidelines “ahead of the curve” to maintain physical distancing and decrease person-to-person contact. It’s also helped reduce cases among children.

“That’s helped us to keep COVID-19 at a modest rate in Western Pennsylvania,” he said. “Take away the high number of cases in skilled nursing facilities and we’ve had one of the mildest case loads in the United States.”

Dr. Aracri also said cases of the inflammatory syndrome are “incredibly, incredibly rare,” but the New

York City totals mean pediatricians “need to be vigilant, knowing the complications that are out there.”

The syndrome associated with COVID-19 is being compared with Kawasaki disease, but there are clear differences.

The National Institutes of Health describes Kawasaki disease as an “inflammation of the blood vessels” affecting children but with no known cause. Symptoms begin with a fever that can last five days along with red eyes, lips and mouth, rashes, swollen and red hands and feet, and swollen lymph nodes.

“Sometimes the disease affects the coronary arteries, which carry oxygen-rich blood to the heart, which can lead to serious heart problems.” More severe cases can result in weakness in blood vessels around the heart.

Resulting low blood pressure caused by inflammation can lead to toxic shock.

Treatments for Kawasaki disease include antibody proteins known as gamma globulins that are given intravenously along with high doses of aspirin, all within a hospital setting, the NIH said. The same holds true for treating PMIS.

“We don’t even know if this is caused by COVID-19,” Dr. Aracri said. “All we know is that a new inflammatory reaction is happening of which some are associated with COVID-19. It’s similar to Kawasaki disease and toxic shock syndrome but we don’t have a full context of what the inflammatory process is that leads to the full syndrome. This is all new and needs to be studied more because it is so recent.”

Among those children who test positive for COVID19, 90% range from being asymptomatic to having mild to moderate symptoms. Only about 5% or fewer — typically children with preexisting health problems — require medical treatment, he said.

“This is an incredibly rare condition, but we’ve done really well here,” he said. “But we need to be vigilant knowing about the complications that are occurring out there” — in New York City, the U.K. and elsewhere.

Dr. Dermody said PMIS generally affects children from 1 to 2 years of age up to 15 and 16, “so it’s not a particular age range within pediatrics,” while Kawasaki disease predominantly affects toddlers.

Both, however, can target coronary blood vessels.

“In this field we have searched for the cause of Kawasaki disease for decades and came up empty,” Dr. Dermody said. “Maybe there are multiple causes or an elusive agent that’s causing it.”

But he said those who develop such conditions may have more exuberant inflammatory responses to infections that target and damage the linings of coronary vessels. “If we could learn more about that, we could design a treatment to target it,” he said.

Still, current treatments to suppress the inflammatory response are effective in the vast majority of patients, he said.

“Parents should be vigilant when a child has a fever greater than 100.4 degrees for more than a day or two, with a rash, a sore throat, nausea, vomiting, diarrhea or abdominal pain,” Dr. Dermody said.

He recommended that parents or guardians first call the child’s primary care physician, who might recommend emergency room care. At Children’s, a team of specialists coordinates treatments.

“We haven’t had a case [of PMIS] here but we are exceedingly vigilant in watching for these children and keeping in contact with colleagues nationwide who are involved with a registry of such cases,” he said. “Last week, we received an alert from the state Department of Health about this and continue to watch for COVID-19 and its various manifestations.”