Kawasaki Disease (KWD)

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Disease Ontology 11 DOID:13378 OMIM® 57 611775 ICD9CM 34 446.1 MeSH 43 D009080 NCIt 49 C34825 SNOMED-CT 68 75053002 ICD10 31 M30.3 ICD10 via Orphanet 32 M30.3

UMLS via Orphanet 72 C0026691 Orphanet 58 ORPHA2331 MedGen 40 C0026691 C2936917 SNOMED-CT via HPO 69 102594003 111583006 112625008 11934000 128241005 16932000 18165001 20741006 21522001 233703007 248274002 253645007 267038008 267060006 271807003 29738008 31996006 3238004 368009 3723001 37796009 386661006 42343007 423666004 44808001 45534005 50177009 50920009 57676002 62315008 698247007 7180009 73013002 76581006 7847004 79654002 84114007 84229001 9826008 more EFO 16 EFO_0004246 ICD11 33 540285662 UMLS 71 C0026691 C2936917

MedlinePlus: ⁴¹ What is Kawasaki disease? Kawasaki disease is a rare illness that usually affects small children. Other names for it are Kawasaki syndrome and mucocutaneous lymph node syndrome. It is a type of vasculitis, which is inflammation of the blood vessels. Kawasaki disease is serious, but most children can fully recover if they are treated right away. What causes Kawasaki disease? Kawasaki disease happens when the immune system injures the blood vessels by mistake. Researchers do not fully know why this happens. But when it does, the blood vessels become inflamed and can narrow or close off. Genetics may play a role in Kawasaki disease. There may also be environmental factors, such as infections. It does not seem to be contagious. This means that it cannot be passed from one child to another. Who is at risk for Kawasaki disease? Kawasaki disease usually affects children under the age of 5. But older children and adults can sometimes get it. It is more common in boys than girls. It can affect children of any race, but those with Asian or Pacific Islander descent are more likely to get it. What are the symptoms of Kawasaki disease? The symptoms of Kawasaki disease may include: High fever lasting at least five days A rash, often on the back, chest, and groin Swollen hands and feet Redness of the lips, lining of the mouth, tongue, palms of the hand, and soles of the feet Pink eye Swollen lymph nodes What other problems can Kawasaki disease cause? Sometimes Kawasaki disease can affect the walls of the coronary arteries. These arteries bring supply blood and oxygen to your heart. This can lead to: An aneurysm (bulging and thinning of the walls of the arteries). This can raise the risk of blood clotss in the arteries. If the blood clots are not treated, they could lead to a heart attack or internal bleeding. Inflammation in the heart Heart valve problems Kawasaki disease can also affect other parts of the body, including the brain and nervous system, the immune system, and the digestive system. How is Kawasaki disease diagnosed? There is no specific test for Kawasaki disease. To make a diagnosis, your child's health care provider may use many tools: A physical exam, which includes looking at the signs and symptoms Blood and urine tests to rule out other diseases and check for signs of inflammation Tests to check for damage to the heart, such as an echocardiogram and electrocardiogram (EKG) What are the treatments for Kawasaki disease? Kawasaki disease is usually treated in the hospital with an intravenous (IV) dose of immunoglobulin (IVIG). Aspirin may also be part of the treatment. But do not give your child aspirin unless the health care provider tells you to. Aspirin can cause Reye syndrome in children. This is a rare, serious illness that can affect the brain and liver. Usually treatment works. But if it is not working well enough, the provider may also give your child other medicines to fight the inflammation. If the disease affects your child's heart, he or she might need additional medicines, surgery, or other medical procedures.

MalaCards based summary: Kawasaki Disease, also known as mucocutaneous lymph node syndrome, is related to exanthem and conjunctivitis, and has symptoms including fever, congestion of ocular conjunctivae and reddening of lips. An important gene associated with Kawasaki Disease is THRIL (TNF And HNRNPL Related Immunoregulatory Long Non-Coding RNA), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Immunologic Factors and Immunoglobulins, Intravenous have been mentioned in the context of this disorder. Affiliated tissues include body, lymph node and heart, and related phenotypes are proteinuria and cheilitis

OMIM®: ⁵⁷ Kawasaki disease is an acute, self-limited vasculitis of infants and children characterized by prolonged fever unresponsive to antibiotics, polymorphous skin rash, erythema of the oral mucosa, lips, and tongue, erythema of the palms and soles, bilateral conjunctival injection, and cervical lymphadenopathy (Kawasaki, 1967). Coronary artery aneurysms develop in 15 to 25% of those left untreated (11,12:Kato et al., 1975, 1996), making Kawasaki disease the leading cause of acquired heart disease among children in developed countries. Treatment with intravenous immunoglobulin (IVIg) abrogates the inflammation in approximately 80% of affected individuals and reduces the aneurysm rate to less than 5%. Cardiac sequelae of the aneurysms include ischemic heart disease, myocardial infarction, and sudden death. Epidemiologic features such as seasonality and clustering of cases suggested an infectious trigger, although no pathogen had been isolated. Several lines of evidence suggested the importance of genetic factors in disease susceptibility and outcome. First, the incidence of Kawasaki disease is 10 to 20 times higher in Japan than in Western countries (Cook et al., 1989). Second, the risk of Kawasaki disease in sibs of affected children is 10 times higher than in the general population, and the incidence of Kawasaki disease in children born to parents with a history of Kawasaki disease is twice as high as that in the general population (Fujita et al., 1989; Uehara et al., 2003). Hata and Onouchi (2009) reviewed current knowledge on Kawasaki disease, including epidemiology, genomewide linkage analysis, and molecular genetics. (611775) (Updated 08-Dec-2022)

MedlinePlus Genetics: ⁴² Kawasaki disease is a sudden and time-limited (acute) illness that affects infants and young children. Affected children develop a prolonged fever lasting several days, a skin rash, and swollen lymph nodes in the neck (cervical lymphadenopathy). They also develop redness in the whites of the eyes (conjunctivitis) and redness (erythema) of the lips, lining of the mouth (oral mucosa), tongue, palms of the hands, and soles of the feet.Without treatment, 15 to 25 percent of individuals with Kawasaki disease develop bulging and thinning of the walls of the arteries that supply blood to the heart muscle (coronary artery aneurysms) or other damage to the coronary arteries, which can be life-threatening.

GARD: ¹⁹ 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.

PubMed Health : ⁶³ Kawasaki disease: Kawasaki (KAH-wah-SAH-ke) disease is a rare childhood disease. It's a form of a condition called vasculitis (vas-kyu-LI-tis). This condition involves inflammation of the blood vessels. In Kawasaki disease, the walls of the blood vessels throughout the body become inflamed. The disease can affect any type of blood vessel in the body, including the arteries, veins, and capillaries. Sometimes Kawasaki disease affects the coronary arteries, which carry oxygen-rich blood to the heart. As a result, some children who have Kawasaki disease may develop serious heart problems.

Disease Ontology: ¹¹ A lymphadenitis characterized by swelling of cervical lymph nodes in infants and young children and inflammation of medium-sized blood vessels located in body, has symptom fever, has symptom congestion of ocular conjunctivae, has symptom reddening of lips, has symptom reddening of oral cavity, has symptom protuberance of tongue papillae and has symptom edema of extremities.

CDC: ² Kawasaki disease (KD), also known as Kawasaki syndrome, is an acute febrile illness of unknown etiology that primarily affects children younger than 5 years of age. The disease was first described in Japan by Tomisaku Kawasaki in 1967, and the first cases outside of Japan were reported in Hawaii in 1976.

Orphanet: ⁵⁸ A rare inflammatory disease characterized by an acute febrile, systemic, self-limiting, medium-vessel vasculitis primarily affecting children. It often causes acute coronary arteritis which is associated with coronary arterial aneurysms (CAA) that may be life threatening when untreated.

UniProtKB/Swiss-Prot: ⁷³ An acute, self-limited vasculitis of infants and children characterized by prolonged fever unresponsive to antibiotics, polymorphous skin rash, erythema of the oral mucosa, lips, and tongue, erythema of the palms and soles, bilateral conjunctival injection, and cervical lymphadenopathy.

Wikipedia: ⁷⁵ Kawasaki disease is a syndrome of unknown cause that results in a fever and mainly affects children... more...

Clinical features from OMIM®:

611775 (Updated 08-Dec-2022)

#	Description	GenomeRNAi Source Accession	Score	Top Affiliating Genes
1	Reduced mammosphere formation	GR00396-S	9.17	CCL2 FCGR2A HSPD1 MYD88 S100A12 SELE

Medicines are the main treatment for Kawasaki disease . Rarely, children whose coronary (heart ) arteries are affected may need medical procedures or surgery . The goals of treatment include: Reducing fever and inflammation to improve symptoms Preventing the disease from affecting the coronary arteries

Search NIH Clinical Center for Kawasaki Disease

Search GEO for disease gene expression data for Kawasaki Disease.