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  3. Kawasaki disease
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Kawasaki disease


Información en español Title


Other Names:
Mucocutaneous lymph node syndrome; Kawasaki syndrome
Categories:
Blood Diseases; Heart Diseases; Immune System Diseases; Blood Diseases; Heart Diseases; Immune System Diseases; Kidney and Urinary Diseases See More

Summary Summary


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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.[1] 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.[2] The cause of Kawasaki disease is unknown.[1][2] An infection along with genetic factors may be involved.[2][3] Treatment includes intravenous gamma globulin and high doses of aspirin in a hospital setting.[1][2] Prognosis is generally very good, but in cases of heart complications it depends on the severity of the coronary disease.[3] 
Last updated: 3/9/2017

Symptoms Symptoms


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This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 36 |
Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Cheilitis
Inflammation of the lips
0100825
Conjunctivitis 0000509
Erythema 0010783
Lymphadenopathy
Swollen lymph nodes
0002716
Proteinuria
High urine protein levels
Protein in urine
[ more ]
0000093
Recurrent pharyngitis
Recurrent sore throat
0100776
Skin rash 0000988
Vasculitis 0002633
30%-79% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ]
0002027
Abnormal heart valve morphology 0001654
Abnormality of nail color
Abnormality of nail colour
0100643
Arthritis
Joint inflammation
0001369
Diarrhea
Watery stool
0002014
Edema
Fluid retention
Water retention
[ more ]
0000969
Fatigue
Tired
Tiredness
[ more ]
0012378
Fever 0001945
Glossitis
Inflammation of the tongue
Smooth swollen tongue
[ more ]
0000206
Leukocytosis
Elevated white blood count
High white blood count
Increased blood leukocyte number
[ more ]
0001974
Pericarditis 0001701
5%-29% of people have these symptoms
Arrhythmia
Abnormal heart rate
Heart rhythm disorders
Irregular heart beat
Irregular heartbeat
[ more ]
0011675
Arthralgia
Joint pain
0002829
Ascending tubular aorta aneurysm 0004970
Aseptic leukocyturia 0100586
Cholecystitis
Gallbladder inflammation
0001082
Congestive heart failure
Cardiac failure
Cardiac failures
Heart failure
[ more ]
0001635
Cranial nerve paralysis 0006824
Double outlet right ventricle with subpulmonary ventricular septal defect without pulmonary stenosis 0011658
Hepatitis
Liver inflammation
0012115
Interstitial pulmonary abnormality 0006530
Irritability
Irritable
0000737
Jaundice
Yellow skin
Yellowing of the skin
[ more ]
0000952
Meningitis 0001287
Migraine
Intermittent migraine headaches
Migraine headache
Migraine headaches
[ more ]
0002076
Myocarditis 0012819
Nausea and vomiting 0002017
Ptosis
Drooping upper eyelid
0000508
Showing of 36 |
Do you have more information about symptoms of this disease? We want to hear from you.
Last updated: 4/1/2018

Cause Cause


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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.[2][4]

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.[12298]

Other theories suggest that the disease is caused by a response from the body's immune system.[5]

KD is not contagious; it cannot be passed from one person to another.[2] Other risk factors include being a male gender, being between six months and five years of age, and having a family history of KD.[5]
Last updated: 3/9/2017

Inheritance Inheritance


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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[4], but it is still rare for more than one child in a family to develop the disease.[6]
Last updated: 3/9/2017

Treatment Treatment


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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.[1] 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.[3] You can read about the Guidelines for risk stratification of myocardial infarction in the long-term follow-up of children with Kawasaki disease, developed by the American Heart Association.
Last updated: 3/9/2017

Management Guidelines

  • Freeman AF, Shulman ST. Kawasaki Disease: Summary of the American Heart Association Guidelines. Am Fam Physician 2006;74:1141-50.
Do you have updated information on this disease? We want to hear from you.

Find a Specialist Find a Specialist


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If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

  • To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.
  • If your physician would like to review your case with a vasculitis specialist, the Vasculitis Foundation lists vasculitis medical consultants that may be able to speak with your healthcare provider. Click on the link above to learn more.
  • The Vasculitis Foundation has a "Find a Physician" map on its website.

Research Research


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Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Kawasaki disease. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Organizations Organizations


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Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

  • American Heart Association
    7272 Greenville Avenue
    Dallas, TX 75231-4596
    Toll-free: 800-242-8721
    Telephone: 214-373-6300
    Fax: 214-373-0268
    E-mail: Review.personal.info@heart.org
    Website: http://www.heart.org/
  • Kawasaki Disease Foundation
    9 Cape Ann Circle
    Ipswich, MA 01938
    Telephone: 978-356-2070
    Fax: 978-356-2079
    E-mail: info@kdfoundation.org
    Website: http://www.kdfoundation.org/
  • Kawasaki Support Group
    Attn: Mrs. Sue Davidson
    13 Norwood Grove
    Potters Green
    Coventry
    CV2 2FR
    United Kingdom
    Telephone: 024 7661 2178
    Fax: 024 7661 2178
    E-mail: helpline@kssg.org.uk
    Website: http://www.kssg.org.uk
  • Vasculitis Foundation
    P.O. Box 28660
    Kansas City, MO 64188
    Toll-free: 800-277-9474
    Telephone: 816-436-8211
    TTY: (816) 656-3838
    E-mail: http://www.vasculitisfoundation.org/contact/
    Website: http://www.vasculitisfoundation.org/

Organizations Providing General Support

  • American Autoimmune Related Diseases Association (AARDA)
    22100 Gratiot Avenue
    Eastpointe, MI 48021
    Toll-free: 800-598-4668
    Telephone: 586-776-3900
    Fax: 586-776-3903
    E-mail: aarda@aarda.org
    Website: https://www.aarda.org/
Do you know of an organization? We want to hear from you.

Learn More Learn More


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These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • You can obtain information on this topic from the Centers for Disease Control and Prevention (CDC). The CDC is recognized as the lead federal agency for developing and applying disease prevention and control, environmental health, and health promotion and education activities designed to improve the health of the people of the United States.
  • The Centers for Disease Control and Prevention (CDC) provide more information on reporting cases of Kawasaki syndrome.  Click on the CDC link to view this information.

    National Center for Zoonotic, Vector-Borne, Enteric Diseases (NCZVED)
    1600 Clifton Road, NE
    MS D-76
    Atlanta, GA 30329-4018
  • Genetics Home Reference (GHR) contains information on Kawasaki disease. This website is maintained by the National Library of Medicine.
  • The Vasculitis Foundation offers information on Kawasaki disease to assist patients, family members, and medical professionals in learning more about the condition.
  • 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.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Heart, Lung, and Blood Institute (NHLBI) has information on this topic. NHLBI is part of the National Institutes of Health and supports research, training, and education for the prevention and treatment of heart, lung, and blood diseases.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Kawasaki disease. Click on the link to view a sample search on this topic.

Videos/Presentations

  • 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.

News & Events News & Events


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News

  • National DNA Day Reddit "Ask Me Anything" (AMA) Series
    April 11, 2018

GARD Answers GARD Answers


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Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • My son had Kawasaki disease last year. I am expecting another baby. Is this baby at-risk to develop Kawasaki disease? See answer

  • Eighteen months ago my daughter was diagnosed with Kawasaki syndrome. Although she has recovered medically, she appears to have some behavioral and developmental problems. Can Kawasaki syndrome cause behavioral problems or slow a child's development? See answer

  • I know someone who had Kawasaki disease when he was a child. Is there any information about the long-term consequences of this condition in surviving adults? See answer

  • My child was diagnosed with Kawasaki disease last month, and I found out that another child in the same daycare center was diagnosed with Kawasaki disease one year ago. I know this condition is rare and I thought it was unusual for two kids from the same day care center to have Kawasaki disease. Is there a method for tracking Kawasaki disease? See answer


Have a question? Contact a GARD Information Specialist.

References References


  1. Starkebaum GA. Kawasaki disease. MedlinePlus. April 20, 2013; http://www.nlm.nih.gov/medlineplus/ency/article/000989.htm.
  2. Kawasaki disease. National Heart Lung and Blood Institute Web site. September 20, 2011; http://www.nhlbi.nih.gov/health/health-topics/topics/kd/.
  3. Sundel R. Kawasaki disease: Initial treatment and prognosis. UpToDate. 2016; http://www.uptodate.com/contents/kawasaki-disease-initial-treatment-and-prognosis.
  4. Kawasaki disease. Genetics Home Reference (GHR). September 2015; http://ghr.nlm.nih.gov/condition/kawasaki-disease.
  5. Sundel R. Kawasaki disease: Epidemiology and etiology. UpToDate. 2016; https://www.uptodate.com/contents/kawasaki-disease-epidemiology-and-etiology?source=see_link.
  6. What is Kawasaki disease?. American Heart Association. 2015; http://www.heart.org/idc/groups/heart-public/@wcm/@hcm/documents/downloadable/ucm_300320.pdf.
Do you know of a review article? We want to hear from you.
You can help advance
rare disease research!
You can help advance rare disease research!
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