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Kawasaki disease

Kawasaki disease
Other namesKawasaki syndrome,[1] mucocutaneous lymph node syndrome[2]
A medical illustration depicting Kawasaki disease.
SpecialtyPediatrics, Rheumatology, Immunology
SymptomsFever > 5 days, large lymph nodes, rash, sore throat, diarrhea[1]
ComplicationsCoronary artery aneurysms[1]
Usual onset< 5 years old[1]
Duration~ 3 weeks[1]
CausesUnknown[1]
Risk factorsAge of < 5 years old
Diagnostic methodBased on symptoms, ultrasound of the heart[1]
Differential diagnosisScarlet fever, juvenile rheumatoid arthritis, paediatric multisystem inflammatory syndrome[3][1]
MedicationOral Aspirin and intravenous immunoglobulin[1]
PrognosisMortality 0.2% with treatment[4]
Frequency8–124 per 100,000 people under five[5]
Named afterTomisaku Kawasaki

Kawasaki disease (also known as mucocutaneous lymph node syndrome) is a syndrome of unknown cause that results in a fever and mainly affects children under 5 years of age.[6] It is a form of vasculitis, where medium-sized blood vessels become inflamed throughout the body.[1] The fever typically lasts for more than five days and is not affected by usual medications.[1] Other common symptoms include large lymph nodes in the neck, a rash in the genital area, lips, palms, or soles of the feet, and red eyes.[1] Within three weeks of the onset, the skin from the hands and feet may peel, after which recovery typically occurs.[1] The disease is the leading cause of acquired heart disease in children in developed countries, which include the formation of coronary artery aneurysms and myocarditis.[1][7]

While the specific cause is unknown, it is thought to result from an excessive immune response to particular infections in children who are genetically predisposed to those infections.[6] It is not an infectious disease, that is, it does not spread between people.[8] Diagnosis is usually based on a person's signs and symptoms.[1] Other tests such as an ultrasound of the heart and blood tests may support the diagnosis.[1] Diagnosis must take into account many other conditions that may present similar features, including scarlet fever and juvenile rheumatoid arthritis.[9] An emerging "Kawasaki-like" disease temporally associated with COVID-19[10] appears to be a distinct syndrome.[11]

Typically, initial treatment of Kawasaki disease consists of high doses of aspirin and immunoglobulin.[1] Usually, with treatment, fever resolves within 24 hours and full recovery occurs.[1] If the coronary arteries are involved, ongoing treatment or surgery may occasionally be required.[1] Without treatment, coronary artery aneurysms occur in up to 25% and about 1% die.[4][12] With treatment, the risk of death is reduced to 0.17%.[12] People who have had coronary artery aneurysms after Kawasaki disease require lifelong cardiological monitoring by specialized teams.[13]

Kawasaki disease is rare.[1] It affects between 8 and 67 per 100,000 people under the age of five except in Japan, where it affects 124 per 100,000.[5] Boys are more commonly affected than girls.[1] The disorder is named after Japanese pediatrician Tomisaku Kawasaki, who first described it in 1967.[5][14]

  1. ^ a b c d e f g h i j k l m n o p q r s t u "Kawasaki Disease". PubMed Health. NHLBI Health Topics. 11 June 2014. Archived from the original on 11 September 2017. Retrieved 26 August 2016.
  2. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. pp. 1232–34. ISBN 978-1-4160-2999-1.
  3. ^ Cite error: The named reference RCP2020 was invoked but never defined (see the help page).
  4. ^ a b Kim DS (December 2006). "Kawasaki disease". Yonsei Medical Journal. 47 (6): 759–72. doi:10.3349/ymj.2006.47.6.759. PMC 2687814. PMID 17191303.
  5. ^ a b c Lai WW, Mertens LL, Cohen MS, Geva T (2015). Echocardiography in Pediatric and Congenital Heart Disease: From Fetus to Adult (2 ed.). John Wiley & Sons. p. 739. ISBN 9781118742488. Archived from the original on 11 September 2017.
  6. ^ a b McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, Baker AL, Jackson MA, Takahashi M, Shah PB, Kobayashi T, Wu MH, Saji TT, Pahl E (2017). "Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association". Circulation. 135 (17): e927–e999. doi:10.1161/CIR.0000000000000484. PMID 28356445."Correction". Circulation. 140 (5): e181–e184. 2019. doi:10.1161/CIR.0000000000000703. PMID 31356128.
  7. ^ Owens, AM (2023). Kawasaki Disease. StatPearls Publishing.
  8. ^ Cite error: The named reference StatPearls was invoked but never defined (see the help page).
  9. ^ Cite error: The named reference SHARE2019 was invoked but never defined (see the help page).
  10. ^ Galeotti C, Bayry J (2020). "Autoimmune and inflammatory diseases following COVID-19". Nature Reviews. Rheumatology. 16 (8): 413–414. doi:10.1038/s41584-020-0448-7. PMC 7271827. PMID 32499548.
  11. ^ Abrams JY, Godfred-Cato SE, Oster ME, et al. (August 2020). "Multisystem inflammatory syndrome in children (MIS-C) associated with SARS-CoV-2: a systematic review". The Journal of Pediatrics. 226: 45–54.e1. doi:10.1016/j.jpeds.2020.08.003. PMC 7403869. PMID 32768466.
  12. ^ a b "Merck Manual, Online edition: Kawasaki Disease". 2014. Archived from the original on 2 January 2010. Retrieved 26 August 2016.
  13. ^ Brogan P, Burns JC, Cornish J, et al. (2020). "Lifetime cardiovascular management of patients with previous Kawasaki disease". Heart. 106 (6): 411–20. doi:10.1136/heartjnl-2019-315925. PMC 7057818. PMID 31843876.
  14. ^ Kawasaki T (March 1967). "[Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]". Arerugi. 16 (3): 178–222. PMID 6062087.
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