Kawasaki disease

What causes Kawasaki disease?

The cause of Kawasaki disease (KD) is unknown. The disease results when cells move into the tissues and buildup there, leading to vascular damage, but what causes the cell buildup in the first place is unknown. The body's response to a virus or infection combined with genetic factors may cause the disease. However, no specific virus or infection has been identified, and the role of genetics is not well understood.

Genetic factors appear to be important to this disorder, as suggested by the increased frequency of the disease in Asian and Asian-American populations and among family members of an affected child. A number of gene variants (polymorphisms) are associated with an increased risk of developing (susceptibility) KD, and some of these variants are also associated with coronary artery lesions and aneurysm formation.

Other theories suggest that the disease is caused by a response from the body's immune system.

KD is not contagious; it cannot be passed from one person to another. Other risk factors include being a male gender, being between six months and five years of age, and having a family history of KD.

Last updated on 05-01-20

How is Kawasaki disease inherited?

A susceptibility to Kawasaki disease (KD) appears to be passed through generations in families, but the inheritance pattern is unknown. Children of parents who have had KD have twice the risk of developing the disease compared to the general population. Children whose siblings have had KD are ten times more likely to develop KD than the general population, but it is still rare for more than one child in a family to develop the disease.

Last updated on 05-01-20

Can Kawasaki disease cause behavioral problems or slow a child's development?

Both generalized and localized central nervous system symptoms have been reported in Kawasaki disease. While neurologic complications or symptoms may occur in a small number of patients with Kawasaki disease, the vast majority escape serious central nervous system damage and data suggests that milder central nervous system effects, in the form of cognitive and academic difficulties are rare. In some cases, however, Kawasaki disease can be associated with significant behavioral symptoms.

A study conducted in 2000 by WJ King et al. found that cognitive development and academic performance were not significantly affected by Kawasaki disease. This study did find, however that individuals who had previous Kawasaki disease experienced significantly more behavior problems than their healthy siblings. These problems were predominantly internalizing and reflected a cluster of specific difficulties including somatic complaints, anxious- depressed behavior, and social problems. These children were also rated as having significantly more attention difficulties than their healthy siblings. A large proportion of parents in this study also perceived that the episode of Kawasaki disease had a long-lasting effect on their child, although this perception was often vague and was not related to the increased risk of behavior problems.

The reported behavioral difficulties may be due to residual central nervous system effects of the disease process, the experience of an acute illness and hospitalization, and/or continued family anxiety after the illness. Heightened parental anxieties about children who have completely recovered from an illness can lead to overprotective relationships that may contribute to difficulties in the psychological development of their children.

Last updated on 05-01-20

Are there resources where I can connect with other parents whose child has developmental or behavior issues following Kawasaki disease?

Yes. The Kawasaki Disease Foundation offers a program called KDF Bridges, where families with children who have Kawasaki Disease are matched with trained volunteers who have either had children with Kawasaki Disease or are adults who have recovered from Kawasaki Disease. There are several families listed on this web site who describe cognitive, behavioral, or developmental problems in their children and offer some suggestions for coping.

Last updated on 05-01-20

What is Kawasaki disease?

Kawasaki disease is a disease that involves inflammation of the blood vessels. It is typically diagnosed in young children, but older children and adults can also develop this condition. Kawasaki disease begins with a fever that lasts at least five days. Other classic symptoms may include red eyes, lips, and mouth; rash; 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. Kawasaki disease occurs most often in people of Asian and Pacific Island descent. The cause of Kawasaki disease is unknown. An infection along with genetic factors may be involved. Treatment includes intravenous gamma globulin and high doses of aspirin in a hospital setting. Prognosis is generally very good, but in cases of heart complications it depends on the severity of the coronary disease.

Last updated on 05-01-20

Is there a method for tracking Kawasaki disease?

Cases of Kawasaki disease are tracked by the Centers for Disease Control and Prevention (CDC) using hospital discharge data, a surveillance system, and research studies. The Kawasaki syndrome surveillance system is based on voluntary reporting by health care providers and local and state health authorities. For more information, we recommend that you contact the CDC or your local health department.

Centers for Disease Control and Prevention (CDC)
National Center for Zoonotic, Vector-Borne, & Enteric Diseases (NCZVED)
Division of High-Consequence
1600 Clifton Road, NE
Mailstop A-30
Atlanta, GA 30333
Toll-Free: 800-232-4636
TTY: 888-232-6348
Fax: fax to 404-639-3163
Website: http://www.cdc.gov/kawasaki/
Link to case report form: http://www.cdc.gov/kawasaki/about.html

Last updated on 05-01-20

Can Kawasaki disease diagnosed in childhood have later effects on adults?

Although Kawasaki disease is generally self-limiting, 10-15% of children may develop problems in their coronary arteries, even after being treated with aspirin and intravenous gamma globulin (purified antibodies--also known as IVIG). Inflammation of the heart muscle (myocardium), heart valves (endocardium) and/or sac surrounding the heart (pericardium) may occur acutely (at the onset of the disease) or many years later. The most common late complication is the persistence of coronary artery aneurysms (weakened areas of blood vessel that balloons out). Such aneurysms can lead to heart attack at a young age or later in life.

In addition, adults may present with ischemic heart disease (ischemic means that an organ, in this case the heart muscle, has not received enough blood and oxygen) as a sequela (late effects) of unrecognized Kawasaki disease in childhood.

Last updated on 05-01-20

Is my new baby at-risk to develop Kawasaki disease?

Children of parents who have had Kawasaki disease have twice the risk of developing the disorder compared to the general population. Children with affected siblings have a tenfold higher risk.

We encourage you to discuss the specific risks for your family with your healthcare provider and/or a genetics professional.

Last updated on 05-01-20

What genes are related to Kawasaki disease?

A variation in the ITPKC gene has been associated with an increased risk of developing Kawasaki disease. This gene provides instructions for making an enzyme called inositol 1,4,5-triphosphate 3-kinase C. This enzyme helps limit the activity of immune system cells called T cells, which identify foreign substances and defend the body against infection. Reducing the activity of T cells when appropriate prevents the overproduction of immune proteins called cytokines that lead to inflammation and can, when present in large quantities, can cause tissue damage. Researchers believe that variations in the ITPKC gene may interfere with the body's ability to reduce T cell activity, leading to inflammation that damages blood vessels and results in the symptoms of this disease. It is likely that other factors, including changes in additional genes, also influence the development of this complex disorder.

Last updated on 05-01-20

How might adults with a history of Kawasaki disease in childhood be managed?

Long-term follow-up in individuals with Kawasaki disease is recommended. The goal of long-term management is to prevent a block of bloodflow to the heart, caused by a blood clot in the coronary artery (coronary thrombosis) and to treat any resulting reduction of blood flow and oxygen to the heart (myocardial ischemia). There are few studies that have been published to assist in creating guidelines for management. It has been suggested that the management should vary depending on the severity of coronary artery involvement.

In general, cardiac imaging such as computerized tomographic angiography (CTA) magnetic resonance imaging (MRI), and echocardiogram are recommended with use of CTA and MRI being utilized more frequently given evidence of more accurate imaging with age. Abnormalities of the coronary arteries may require ongoing medication, interventional catheterization, and/or cardiac surgery.

Last updated on 05-01-20

How might Kawasaki disease be treated?

Intravenous gamma globulin is the standard treatment for Kawasaki disease and is administered in high doses. Children with Kawasaki disease usually improve significantly within 24 hours of treatment with IV gamma globulin (IVIG). Aspirin is often given in combination with the IV gamma globulin as part of the treatment plan in the beginning of the disease. Additional treatments include glucocorticoids, which may reduce the level of inflammatory markers and fever more rapidly, and decrease the rate of initial treatment failure in certain high-risk patients. You can read about complications, treatment, and prevention of Kawasaki disease on the American Heart Association website.

Last updated on 05-01-20

Videos/presentations

Kawasaki Disease: New Horizons

Dr. Adriana Tremoulet spoke on Kawasaki Disease (KD), in her lecture "Kawasaki Disease: New Horizons" at the NIH Clinical Center in November 2016. Dr. Tremoulet is an associate professor and associate director at the Kawasaki Disease Research Center at the University of California San Diego Rady Children's Hospital. While the lecture is aimed at medical professionals, it may be of interest to anyone who seeks detailed information on KD.

Last updated on 04-27-20

Where To Start

KidsHealth - Kawasaki

KidsHealth created by The Nemours Foundation's Center for Children's Health Media provides information in both English and Spanish about this condition. Click on the link to access the information page on Kawasaki disease.

Last updated on 04-27-20

Kawasaki syndrome - Vasculitis Foundation

The Vasculitis Foundation offers information on Kawasaki disease to assist patients, family members, and medical professionals in learning more about the condition.

Last updated on 04-27-20

Name: Kawasaki Disease Foundation 9 Cape Ann Circle
Ipswich, MA, 01938, United States
Phone: 978-356-2070 Fax : 978-356-2079 Email: info@kdfoundation.org Url: http://www.kdfoundation.org/
Name: Kawasaki Support Group Attn: Mrs. Sue Davidson 13 Norwood Grove
Potters Green Coventry CV2 2FR
United Kingdom
Phone: 024 7661 2178 Fax : 024 7661 2178 Email: helpline@kssg.org.uk Url: http://www.kssg.org.uk
Name: Vasculitis Foundation P.O. Box 28660
Kansas City, MO, 64188, United States
Phone: +1-816-436-8211 Toll Free: 1-800-277-9474 Fax : +1-816-656-3838 Email: https://www.vasculitisfoundation.org/contact-us-2/ Url: https://www.vasculitisfoundation.org/
Name: American Heart Association 7272 Greenville Avenue
Dallas, TX, 75231-4596, United States
Phone: 214-570-5978 Toll Free: 800-242-8721 Email: https://www.heart.org/en/forms/general-questions-and-latest-research-information Url: https://www.heart.org
Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: aarda@aarda.org Url: https://www.aarda.org/

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