Because the vast majority of patients do not develop other health problems following KD, an immune defect seems highly unlikely. Our studies demonstrating an antigen-driven IgA immune response in acute KD and the presence of KD antigen in intracytoplasmic inclusion bodies in KD bronchial epithelium lead us to put forth the following model of KD pathogenesis Figure 1. The virus can result in sporadic cases of KD or in outbreaks.
The agent can remain persistent in cytoplasmic inclusion bodies, with intermittent shedding into the respiratory tract of previously infected individuals. It can enter the bloodstream via macrophages and target particularly the coronary arteries and also other sites.
Antigen-specific IgA plasma cells 17 , 19 , 20 , 22 , 23 and CD8 T cells 14 , 15 , 26 respond to the infection, but coronary arteries can be damaged. The provision of specific antibodies directed at the ubiquitous KD agent could explain the efficacy of intravenous gammaglobulin IVIG in the treatment of KD. These specific antibodies are present in IVIG because most adult donors were asymptomatically infected during young childhood, which accounts for the reduced prevalence in older children and the rarity of KD in adults.
The agent can be spread through the population either from community contacts with asymptomatic primary infection particularly in the winter-spring, or from a close contact who had been previously infected and then intermittently sheds the agent, resulting in cases during other seasons. We believe that our model, although speculative, fits clinical and epidemiologic findings in KD much better than other currently proposed speculative models.
Identification of the etiology of KD is the most important research goal in the field. With this information, a diagnostic test can be developed, therapy improved, and prevention become possible. Hopefully, in the near future, the etiology can be discovered using synthetic antibodies derived from KD patients' B cell immune response to the triggering agent.
AR and SS contributed equally to conceiving the topics covered and in authoring the work. The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Epidemiological observations of Kawasaki disease in Japan, Pediatr Int. Epidemiologic features of Kawasaki disease in South Korea: data from nationwide survey, Pediatr Infect Dis J.
Epidemiologic features of Kawasaki disease in acute stages in Taiwan, effect of different case definitions in claims data analysis. J Chin Med Assoc. Hospitalizations for Kawasaki syndrome among children in the United States, Nationwide epidemic of Kawasaki disease in Japan during winter of Lancet —9. Hawaii Med J. PubMed Abstract Google Scholar. A nationwide incidence survey of Kawasaki disease in in Japan.
J Infect Dis. Incidence rate of recurrent Kawasaki disease in Japan. Acta Paediatr. An outbreak of Kawasaki disease in Miyako Island in Okinawa prefecture. Kawasaki disease in monozygotic twins. Kawasaki disease in families.
Pediatrics —9. PubMed Abstract. Kawasaki syndrome: description of two outbreaks in the United States. The disease was first described in Japan by Tomisaku Kawasaki in , and the first cases outside of Japan were reported in Hawaii in Clinical signs include fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips, and throat. KD is a leading cause of acquired heart disease in the United States.
Serious complications include coronary artery dilatations and aneurysms. The standard treatment, intravenous immunoglobulin and aspirin, substantially decreases the development of these coronary artery abnormalities.
KD occurs worldwide, with the highest incidence in Japan, and it most often affects boys and younger children. KD may have a winter-spring seasonality, and community-wide outbreaks have been reported occasionally. In the continental United States, population-based and hospitalization studies estimate an incidence of KD ranging from about 9 to 20 per , children under 5 years of age. In the year , approximately hospitalizations with KD were reported among children under 18 years of age in the US; of these children were under 5 years of age, for a hospitalization rate of Most children with Kawasaki disease start to get much better after a single treatment with immune globulin, though sometimes more doses are needed.
Most kids with Kawasaki disease recover completely, especially when they are diagnosed and treated early. Some, especially those who develop heart problems from Kawasaki disease, might need more testing and to see a cardiologist a doctor who specializes in conditions that affect the heart. Reviewed by: Karen A. Ravin, MD. Larger text size Large text size Regular text size. What Is Kawasaki Disease?
Other symptoms include: red "bloodshot" eyes a pink rash on the back, belly, arms, legs, and genital area red, dry, cracked lips a "strawberry" tongue white coating with red bumps on the tongue a sore throat swollen palms of the hands and soles of the feet with a purple-red color swollen lymph glands in the neck The second phase usually begins 2 weeks after the fever started. Symptoms can include: peeling skin on the hands and feet joint pain diarrhea vomiting belly pain What Problems Can Happen?
If the condition isn't found until later, patients can have serious complications that affect the heart, such as: an aneurysm a bulge in the wall of the coronary arteries, which supply blood to the heart inflammation of the heart muscle, lining, valves, and the outer membrane around the heart arrhythmias , which are changes in the normal pattern of the heartbeat problems with some heart valves What Causes Kawasaki Disease?
How Is Kawasaki Disease Diagnosed? If Kawasaki disease looks likely, the doctor: will order tests to check the heart, such as an echocardiogram might test blood and urine pee samples to rule out other conditions, such as scarlet fever , measles , Rocky Mountain spotted fever , or juvenile rheumatoid arthritis How Is Kawasaki Disease Treated?
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