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 and mucous membranes, lymph nodes, blood vessel walls, and the heart. It does not seem to be contagious. It was first described in 1967 by Dr. Tomisaku Kawasaki in Japan. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // M00-M99 - Diseases of the musculoskeletal system and connective tissue (M00-M25) Arthropathies (M00-M03) Infectious arthropathies (M00) Pyogenic arthritis (M01) Direct infections of joint in infectious and parasitic diseases classified elsewhere (M02) Reactive arthropathies (M023) Reiters disease (M03) Postinfective and reactive arthropathies in diseases classified elsewhere (M05-M14... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... In medicine, vasculitis (plural: vasculitides) is a group of diseases featuring inflammation of the wall of blood vessels due to leukocyte migration and resultant damage. ... In zootomy and dermatology, skin is the largest organ of the integumentary system made up of multiple layers of epithelial tissues that guard underlying muscles and organs. ... The mucous membranes (or mucosae; singular: mucosa) are linings of mostly endodermal origin, covered in epithelium, and are involved in absorption and secretion. ... Lymph nodes are components of the lymphatic system. ... f you all The blood vessels are part of the circulatory system and function to transport blood throughout the body. ... The heart and lungs, from an older edition of Grays Anatomy. ... 1967 (MCMLXVII) was a common year starting on Sunday of the Gregorian calendar (the link is to a full 1967 calendar). ...

Incidence, causes, and risk factors

By far, the highest incidence of Kawasaki disease occurs in Japan (175 per 100,000), though its incidence in the United States is increasing. 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.

The causative agent of Kawasaki disease is still unknown. However, current etiological theories center primarily on immunological causes for the disease. Much research is being performed to discover a definitive toxin or antigenic substance, possibly a superantigen, that is the specific cause of the disease. An unknown virus may play a role as an inciting factor as well. Etiology (alternately aetiology, aitiology) is the study of causation. ... The word theory has a number of distinct meanings in different fields of knowledge, depending on their methodologies and the context of discussion. ... A scanning electron microscope image of a single neutrophil (yellow), engulfing anthrax bacteria (orange). ... This article or section does not cite any references or sources. ... An antigen is a molecule that stimulates an immune response. ... Superantigens (SAgs) are a group of virulent toxins that indiscriminately activate T-cells of the immune system causing system-wide inflammation and other serious, potentially fatal symptoms. ...

The cardiac complications are, by far, the most important aspect of the disease. Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms. These aneurysms can lead to myocardial infarction (heart attack) even in young children. Overall, about 10–18% of children with Kawasaki disease develop coronary artery aneurysms^[1], with much higher prevalence among patients who are not treated early in the course of illness. Kawasaki disease is the most common cause of acquired heart disease among children in the United States. This page is about the muscular organ, the Heart. ... The coronary circulation consists of the blood vessels that supply blood to, and remove blood from, the heart. ... Post surgical photo of brain aneurysm survivor. ... Acute myocardial infarction (AMI or MI), more commonly known as a heart attack, is a disease state that occurs when the blood supply to a part of the heart is interrupted. ... Acute myocardial infarction (AMI or MI), more commonly known as a heart attack, is a disease state that occurs when the blood supply to a part of the heart is interrupted. ...

Symptoms

Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal doses of acetaminophen or ibuprofen. The fever may persist steadily for up to two weeks and is normally accompanied by irritability. Affected children develop red eyes, red mucous membranes in the mouth, red cracked lips, a "strawberry tongue", iritis, keratic precipitates (detectable by an ophthalmologist but usually too small to be seen by the unaided eye), and swollen lymph nodes. Skin rashes occur early in the disease, and peeling of the skin in the genital area, hands, and feet (especially around the nails and on the palms and soles) may occur in later phases. Some of these symptoms may come and go during the course of the illness. If left untreated, the symptoms will eventually relent, but coronary artery aneurysms will not improve, resulting in a significant risk of death or disability due to myocardial infarction (heart attack). If treated in a timely fashion, this risk can be mostly avoided and the course of illness cut short. An analogue medical thermometer showing the temperature of 38. ... Acetaminophen (USAN) or paracetamol (INN), is a popular analgesic and antipyretic drug that is used for the relief of fever, headaches, and other minor aches and pains. ... Ibuprofen (INN) (IPA: ) is a non-steroidal anti-inflammatory drug (NSAIDs) originally marketed as Nurofen and since under various trademarks including Act-3, Advil, Brufen, Dorival, Herron Blue, Panafen, Motrin, Nuprin and Ipren or Ibumetin (Sweden), Ibuprom (Poland), IbuHEXAL, Moment (Italy), Ibux (Norway). ... The mucous membranes (or mucosae; singular: mucosa) are linings of mostly endodermal origin, covered in epithelium, and are involved in absorption and secretion. ... Strawberry tongue is the appearance of tongue with inflammed red papillae, giving an appearance of strawberry. ... Lymph nodes are components of the lymphatic system. ... For the manga by Tsukasa Hôjô, see Rash. ... A sex organ, or primary sexual characteristic, narrowly defined, is any of those parts of the body (which are not always bodily organs according to the strict definition) which are involved in sexual reproduction and constitute the reproductive system in an complex organism; namely: Male: penis (notably the glans penis...

• High-grade fever (greater than 39 °C or 102 °F; often as high as 40 °C or 104 °F) that normally lasts for more than a week if left untreated.
• Red eyes (conjunctivitis) without pus or drainage, also known as "conjunctival injection"
• Bright red, chapped, or 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 the soles of the feet
• Swollen hands and feet
• Rash which may take many forms, but not vesicular (blister-like), on the trunk
• Swollen lymph nodes (frequently only one lymph node is swollen), particularly in the neck area
• Joint pain (arthralgia) and swelling, frequently symmetrical
• Irritability
• Tachycardia (rapid heart beat)
• Peeling (desquamation) palms and soles (later in the illness); peeling may begin around the nails

The mucous membranes (or mucosae; singular: mucosa) are linings of mostly endodermal origin, covered in epithelium, and are involved in absorption and secretion. ... This page is a candidate to be moved to Wiktionary. ... Lymph nodes are components of the lymphatic system. ... Arthralgia is a term used to describe pain in the joints. ... This article or section does not cite any references or sources. ...

Signs and tests

A physical examination will demonstrate many of the features listed above.

Blood tests

• Complete blood count (CBC) may reveal normocytic anemia and eventually thrombocytosis
• Erythrocyte sedimentation rate (ESR) will be elevated
• C-reactive protein (CRP) will be elevated
• Liver function tests may show evidence of hepatic inflammation and low serum albumin

Other tests (may or may not be performed) Schematics of shorthand for complete blood count commonly used by physicians. ... Anemia (AmE) or anæmia (BrE), from the Greek () meaning without blood, is a deficiency of red blood cells (RBCs) and/or hemoglobin. ... Thrombocytosis is the presence of high platelet counts in the blood, and can be either reactive or primary (also termed essential and caused by a myeloproliferative disease). ... The erythrocyte sedimentation rate(ESR), also called a sedimentation rate, sed rate or Biernacki Reaction, is a non-specific measure of inflammation that is commonly used as a medical screening test. ... C-reactive protein (CRP) is a plasma protein, an acute phase protein produced by the liver. ... Liver function tests (LFTs or LFs), which include liver enzymes, are groups of clinical biochemistry laboratory blood assays designed to give information about the state of a patients liver. ... You may be looking for albumen, or egg white. ...

• Electrocardiogram may show evidence of ventricular dysfunction or, occasionally, arrhythmia due to myocarditis
• Echocardiogram may show subtle coronary artery changes or, later, true aneurysms.
• Ultrasound or computerized tomography may show hydrops (enlargement) of the gallbladder
• Urinalysis may show white blood cells and protein in the urine (pyuria and proteinuria) without evidence of bacterial growth
• Lumbar puncture may show evidence of aseptic meningitis
• Angiography was historically used to detect coronary artery aneurysms and remains the gold standard for their detection, but is rarely used today unless coronary artery aneurysms have already been detected by echocardiography.

“QRS” redirects here. ... In the heart, a ventricle is a chamber which collects blood from an atrium (another heart chamber) and pumps it out of the heart. ... A cardiac arrhythmia, also called cardiac dysrhythmia, is a disturbance in the regular rhythm of the heartbeat. ... In medicine (cardiology), myocarditis is inflammation of the myocardium, the muscular part of the heart. ... The echocardiogram is an ultrasound of the heart. ... Ultrasound is a form of cyclic sound pressure with a frequency greater than the upper limit of human hearing, this limit being approximately 20 kilohertz (20,000 hertz). ... CAT apparatus in a hospital Computed axial tomography (CAT), computer-assisted tomography, computed tomography, CT, or body section roentgenography is the process of using digital processing to generate a three-dimensional image of the internals of an object from a large series of two-dimensional X-ray images taken around... The gallbladder (or cholecyst, sometimes gall bladder) is a pear-shaped organ that stores about 50 ml of bile (or gall) until the body needs it for digestion. ... A urinalysis (or UA) is an array of tests performed on urine and one of the most common methods of medical diagnosis. ... In medicine, Pyuria refers to urine which contains white blood cells. ... Proteinuria (from protein and urine) means the presence of an excess of serum proteins in the urine. ... A patient undergoes a lumbar puncture at the hands of a neurologist. ... Aseptic meningitis is a condition in which the layers lining of the brain, or meninges, become inflamed and a pyogenic bacterial source is not to blame. ... Angiography or arteriography is a medical imaging technique in which an X-ray picture is taken to visualize the inner opening of blood filled structures, including arteries, veins and the heart chambers. ...

Diagnosis

Kawasaki disease is diagnosed clinically (by medical signs and symptoms), and there exists no specific laboratory test that can tell if someone has it. It is normally difficult to establish the diagnosis, especially early in the course of illness, and frequently children are not diagnosed until they have seen their doctor several times, or visited a number of different health care providers. Many other serious illnesses can cause similar symptoms, and must be considered in the differential diagnosis, including scarlet fever, toxic shock syndrome, and juvenile idiopathic arthritis. In medicine, a sign is a feature of disease as detected by the doctor during physical examination of a patient. ... The term symptom (from the Greek meaning chance, mishap or casualty, itself derived from συμπιπτω meaning to fall upon or to happen to) has two similar meanings in the context of physical and mental health: Strictly, a symptom is a sensation or change in health function experienced by a patient. ... This article or section does not cite any references or sources. ... Toxic shock syndrome (TSS) is a rare but potentially fatal disease caused by a bacterial toxin. ... This article does not deal with the more general topic of childhood arthritis. ...

Classically, five days of fever plus four of five diagnostic criteria must be met in order to establish the diagnosis. The criteria are: (1) erythema of the lips or oral cavity or cracking of the lips; (2) rash on the trunk; (3) swelling or erythema of the hands or feet; (4) red eyes (conjunctival injection) (5) swollen lymph node in the neck of at least 15 millimeters. Diagnosis (from the Greek words dia = by and gnosis = knowledge) is the process of identifying a disease by its signs, symptoms and results of various diagnostic procedures. ... ... Erythema is an abnormal redness of the skin caused by capillary congestion. ...

Many children, especially infants, eventually diagnosed with Kawasaki disease do not exhibit all of the above criteria. In fact, many experts now recommend treating for Kawasaki disease even if only three days of fever have passed and at least three diagnostic criteria are present, especially if other tests reveal abnormalities consistent with Kawasaki disease. In addition, the diagnosis can be made purely by the detection of coronary artery aneurysms in the proper clinical setting.

Treatment

Children with Kawasaki disease should be hospitalized and cared for by a physician who has experience with this disease. When in an academic medical center, care is often shared between pediatric cardiology and pediatric infectious disease specialists, although no infectious agent has been demonstrated. It is imperative that treatment be started as soon as the diagnosis is made to prevent damage to the coronary arteries. A diagram of a heart with an ECG indicator; diagrams like this are used in Cardiology. ... This false-colored electron micrograph shows a malaria sporozoite migrating through the midgut epithelia. ... In medicine, infectious disease or communicable disease is disease caused by a biological agent (e. ... The coronary circulation consists of the blood vessels that supply blood to, and remove blood from, the heart. ...

Intravenous gamma globulin (IVIGG) is the standard treatment for Kawasaki disease and is administered in high doses with marked improvement usually noted within 24 hours. An intravenous drip in a hospital Intravenous therapy or IV therapy is the administration of liquid substances directly into a vein. ... Gamma globulins are a class of proteins in the blood, identified by their position after serum protein electrophoresis. ...

Salicylate therapy, particularly aspirin, remains an important part of the treatment but salicylates alone are not as effective as IV gamma globulin. Aspirin therapy is started at high doses until the fever subsides, and then is continued at a low dose when the patient returns home. Except for Kawasaki disease and a couple of other indications, aspirin is otherwise normally not recommended for children due to its association with Reye's syndrome. Salicylic acid is a colorless, crystalline organic carboxylic acid. ... Aspirin, or acetylsalicylic acid (IPA: ), (acetosal) is a drug in the family of salicylates, often used as an analgesic (to relieve minor aches and pains), antipyretic (to reduce fever), and as an anti-inflammatory. ... Salicylic acid is a colorless, crystalline organic carboxylic acid. ... An intravenous drip in a hospital Intravenous therapy or IV therapy is the administration of liquid substances directly into a vein. ... Gamma globulins are a class of proteins in the blood, identified by their position after serum protein electrophoresis. ... Reyes syndrome is a potentially fatal disease that causes numerous detrimental effects to many organs, especially the brain and liver. ...

Corticosteroids have also been used, especially when other treatments fail or symptoms recur, but in a randomized controlled trial, the addition of corticosteroid to immune globulin and aspirin did not improve outcome. ^[2] In physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. ...

The are also treatments for iritis and other eye symptoms.

Prognosis

With early treatment, rapid recovery from the acute symptoms can be expected and the risk of coronary artery aneurysms greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited (i.e. the patient will recover eventually), but the risk of coronary artery involvement is much greater. Overall, about 2% of patients die from complications of coronary vasculitis. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every 1–2 years to screen for progression of cardiac involvement. The echocardiogram is an ultrasound of the heart. ...

It is also not uncommon that a relapse of symptoms may occur soon after initial treatment with IVIGG. This usually requires re-hospitalization and retreatment. Treatment with IVIGG can cause allergic and non-allergic acute reactions, aseptic meningitis, fluid overload and, rarely, other serious reactions. Aspirin may increase the risk of bleeding from other causes and may be associated with Reye's syndrome. Overall, life-threatening complications resulting from therapy for Kawasaki disease are exceedingly rare, especially compared with the risk of non-treatment. A relapse (etymologically, who falls again) occurs when a person is affected again by a condition that affected them in the past. ... Hypervolemia (or Fluid overload) is the medical condition where there is too much fluid in the body. ...

Media

Kawasaki disease was mentioned in the television programs Nip/Tuck and Without a Trace.^{[citation needed]} In the episode All In of the TV series House, it was, inexplicably, a possible diagnosis for a 6 year old boy that was admitted with bloody diarrhea and coordination problems, as well as an elderly woman with unexplained respiratory, cardiovascular and neural deficiencies. Maxie Jones, a fictional character on General Hospital suffers from it. Nip/Tuck is an Emmy and Golden Globe award-winning American television medical drama series created by Ryan Murphy for FX Networks. ... Without a Trace is an American television show set in New York City. ... All In is the seventeenth episode of the second season of House, which premiered on the FOX network on April 11, 2006. ... House, also known as House, M.D., is a critically-acclaimed American medical drama television series created by David Shore and executive produced by Shore and film director Bryan Singer. ... Maxie Jones, (born Maria Maximiliana Jones), is a fictional character on the popular ABC soap opera General Hospital. ...

Reference

• Kawasaki T (1967). "[Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]" (in Japanese). Arerugi 16 (3): 178-222. PMID 6062087. 

Footnotes

1. ^ Belay E, Maddox R, Holman R, Curns A, Ballah K, Schonberger L (2006). "Kawasaki syndrome and risk factors for coronary artery abnormalities: United States, 1994-2003.". Pediatr Infect Dis J 25 (3): 245-9. PMID 16511388. 
2. ^ Newburger JW et al, Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease, N Engl J Med. 2007 Feb 25;356(7):663-75

External links

• Kawasaki Disease Foundation
• Kawasaki Disease Canada
• Kawasaki Disease Forum
• Kawasaki Disease Research Program
• Kawasaki Disease information from Seattle Children's Hospital Heart Center
• 1751842816 at GPnotebook