The dangerous "Kawasaki disease"

This is the 4496th article of Da Yi Xiao Hu

Dangerous Moment

At the end of September 2016, a two-month-old baby boy named Xiao Kai (pseudonym) from Fuzhou had a high fever for 10 consecutive days. His eyeballs were red like rabbit eyes and his tongue was swollen like bayberry. The baby's parents thought he had a cold and went to many hospitals to see a doctor without success. Finally, he was sent to Fujian Provincial Maternal and Child Health Hospital for emergency treatment. The doctor finally diagnosed that Xiao Kai had a serious disease.

Kawasaki disease has led to severe pneumonia, respiratory failure, sepsis, and aseptic meningoencephalitis. If not treated promptly, it may cause sudden death.

What is Kawasaki disease?

Kawasaki disease, also known as pediatric mucocutaneous lymph node syndrome, was first reported by Japanese physician Tomisaku Kawasaki in 1967 and named after him. This disease is an acute febrile rash disease in children with systemic vasculitis as an important lesion. Usually infants and young children under 5 years old are the high-risk population, with more boys than girls. It is relatively rare in adults and children under 3 months old. The clinical manifestations are generally fever, non-purulent lymphadenopathy in the neck, rash, conjunctival congestion, diffuse congestion of the oral mucosa, bayberry tongue, palmoplantar erythema, and hard edema of the hands and feet. Because this disease can induce serious cardiovascular complications, it has gradually attracted people's attention. The incidence rate of untreated children is as high as 20% to 25%.

What are the symptoms of Kawasaki disease?

Clinically, high fever (above 39°C) is often the initial manifestation. The fever usually lasts for more than 5 days, usually one or two weeks. In some cases, the fever will rise again after one or two days. The fever can last for three or four weeks. Antipyretics can only temporarily lower the body temperature. After a few days of fever, the palms and soles become red, swollen and painful. Maculopapular rashes of varying sizes appear on the trunk with no abnormalities in shape. They may also appear on the face and limbs. They are not itchy and there are no herpes or scabs. After a few days of fever, the conjunctivae on both sides will be congested, with the bulbar conjunctiva being the most serious. Only a few may develop purulent conjunctivitis. Anterior iridocyclitis may be detected with a slit lamp. The lips and face become red, swollen, dry and cracked, and may even cause bleeding;

The tongue is often bayberry-like, with congestion of the oral mucosa but no ulcers.

In addition, some patients have unilateral or bilateral lymphadenopathy in the early stages, which is non-suppurative and subsides after a few days. Sometimes the swelling spreads to the submandibular area and may even be misdiagnosed as mumps. The lymphadenopathy is limited to the anterior triangle of the neck, is painless, and rarely affects other parts.

In the second week of the disease, some children may experience peeling of the hands and feet, usually starting from the transition area of ​​the nail bed, and some may first show perianal desquamation.

Kawasaki disease treatment and prognosis

Treatment in the acute phase includes oral aspirin, intravenous immunoglobulin, and the use of hormones.

The prognosis of this disease is generally good, and most children can recover on their own, but about 5% to 9% of children with Kawasaki disease may develop coronary artery complications. They may die from coronary artery aneurysm rupture, thrombotic occlusion, myocarditis, or suspected myocardial infarction. The mortality rate has now dropped to less than 1%, and about 2% may relapse.

This project is funded by Shanghai Science Education Development Foundation (Project No.: B202117)

Author: Shanghai Jing'an District Central Hospital

Dr. Xia Lemin