Template:LR: Difference between revisions
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* Convalescent Phase- Its beginning is marked by the resolution of all clinical signs & symptoms. Normalization of ESR, which takes about 5-6 weeks from the onset of the disease, marks the end of the convalescent phase. | * Convalescent Phase- Its beginning is marked by the resolution of all clinical signs & symptoms. Normalization of ESR, which takes about 5-6 weeks from the onset of the disease, marks the end of the convalescent phase. | ||
== | ==Diagnosis== | ||
The diagnosis of Kawasaki Disease is clinical. The making a diagnosis requires the presence of high-grade fever for five days and four out of five other diagnostic criteria- | |||
*Desquamation of mucous membranes( strawberry tongue) | |||
*Polymorphous rash of hand & feet which can spread towards the trunk | |||
*Cervical lymphadenopathy (more than 15mm in size; often unilateral and unpainful) | |||
*Conjunctivitis | |||
*Edema of hand & feet | |||
==Microscopic Pathology== | ==Microscopic Pathology== | ||
==References== | ==References== | ||
Revision as of 20:09, 12 June 2020
"sandbox:LR"
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Kawasaki disease, also known as lymph node syndrome, mucocutaneous node disease, infantile polyarteritis, and Kawasaki syndrome, is a poorly understood self-limited vasculitis that affects many organs, including the skin, mucous membranes, lymph nodes, blood vessel walls, and the heart. There is no evidence that Kawasaki disease is contagious. It was first described in 1967 by Dr. Tomisaku Kawasaki in Japan[1]. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than 5 years of age. Additional risk factors in the United States include Asian race and male sex. Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms( reported in about 25% of cases). Common symptoms of Kawasaki disease include high-grade fever, red eyes, bright red and cracked lips, red mucous membranes in the mouth, strawberry tongue, white coating on the tongue or prominent red bumps (papillae) on the back of the tongue, red palms of the hands and soles of the feet, swollen hands and feet, and rash. Intravenous immunoglobulin(IVIG) and aspirin are indicated in Kawasaki disease.
Historical Perspective
Kawasaki disease was first discovered in 1961 by Tomisaku Kawasaki, a Japanese pediatrician. He published his first case report in 1967 in the Japanese language. He founded the Kawasaki Disease Research Center in 1990.
Epidemiology
Kawasaki disease is most commonly found in children under 5 years of age. It is a common cause of acquired childhood heart disease in the United States of America. It has been reported in over 60 countries. It has the highest incidence in North-East Asia esp. Japan and South Korea.japan has been conducting nationwide surveys to evaluate the incidence of Kawasaki disease every two years since 1970. The 24th nationwide survey[2] was conducted in 2017 to evaluate the incidence of Kawasaki disease for the years 2015 and 2016. The incidence was found to be 240 per 100000 for girls and 330 per 100000 for boys. The incidence in the USA is 9 per 100000.
Pathophysiology
The pathophysiology of Kawasaki disease is not well understood. Most of the current theories are of immunological origin. It is primarily a necrotizing inflammation of the medium-sized blood vessels affecting multiple organ systems. This can lead to various complications such as pericarditis, pneumonitis, myocarditis, aseptic meningitis, coronary artery aneurysms, etc. It leads to chronic inflammation of blood vessels which can evolve into stenosis as well as aneurysms. On microscopic analysis, ciliated bronchial epithelial cells frequently show intracytoplasmic inclusion-bodies.
Genetics
While genetic predisposition has been observed in Kawasaki disease, the pattern of inheritance is unclear. The children, as well as the siblings of Kawasaki disease patients, are at an increased risk of developing the disease. One of the genes implicated in the pathogenesis of Kawasaki disease is the ITPKC gene[3]. It codes for the enzyme Inositol 1,4,5-trisphosphate 3-kinase C (ITPKC), which is responsible for the negative regulation of T-cell activation. Polymorphisms of this gene lead to varying levels of potential for T-cell activation in different individuals.
Natural History and Prognosis
Kawasaki disease is a condition often found in children below five years of age. It has an excellent even when left untreated with a mortality of only 2%. It usually resolves on its own. The course of the disease consists of 3 phases[4]-
- Acute Febrile Phase- It is characterized by high fever, desquamation of mucous membranes, edema of hand & feet, and other diagnostic criteria. It usually lasts 1-2 weeks and can include development of other clinical features such as aseptic meningitis, joint pains, myocarditis, and hepatic dysfunction.
- Subacute Phase-Begins afters 1-2 weeks of the onset of disease and lasts about four weeks. Fever, cervical lymphadenopathy, and rash resolve. Irritability, conjunctival injection and anorexia persist. Coronary artery aneurysms might develop if the disease is untreated. The risk of sudden death is highest in this phase[5]. Certain children may also develop neurological symptoms such as cerebral infarcts, encephalopathy, facial nerve block, etc.
- Convalescent Phase- Its beginning is marked by the resolution of all clinical signs & symptoms. Normalization of ESR, which takes about 5-6 weeks from the onset of the disease, marks the end of the convalescent phase.
Diagnosis
The diagnosis of Kawasaki Disease is clinical. The making a diagnosis requires the presence of high-grade fever for five days and four out of five other diagnostic criteria-
- Desquamation of mucous membranes( strawberry tongue)
- Polymorphous rash of hand & feet which can spread towards the trunk
- Cervical lymphadenopathy (more than 15mm in size; often unilateral and unpainful)
- Conjunctivitis
- Edema of hand & feet
Microscopic Pathology
References
- ↑ Kawasaki T (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.
- ↑ Makino N, Nakamura Y, Yashiro M, Kosami K, Matsubara Y, Ae R; et al. (2019). "Nationwide epidemiologic survey of Kawasaki disease in Japan, 2015-2016". Pediatr Int. 61 (4): 397–403. doi:10.1111/ped.13809. PMID 30786118.
- ↑ Onouchi Y, Gunji T, Burns JC, Shimizu C, Newburger JW, Yashiro M; et al. (2008). "ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms". Nat Genet. 40 (1): 35–42. doi:10.1038/ng.2007.59. PMC 2876982. PMID 18084290.
- ↑ Castro PA, Urbano LM, Costa IM (2009). "[Kawasaki disease]". An Bras Dermatol. 84 (4): 317–29. doi:10.1590/s0365-05962009000400002. PMID 19851663.
- ↑ Rowley AH, Shulman ST (1998). "Kawasaki syndrome". Clin Microbiol Rev. 11 (3): 405–14. PMC 88887. PMID 9665974.