Kawasaki Syndrome

Andrea Soung’s Spring Research Paper:

Period 1

4/23/11

            Nobody knows why she constantly cries. Something seems to bother her, but they cannot figure out what, besides the fact that she is an infant, barely a year old. They later find out after a few days of hospitalization. Their daughter, Andrea, has a case of an unknown disease. I, the daughter, am a victim of what has later become to be known as the Kawasaki Syndrome. Kawa-what? It is still a mystery to science today, but the Kawasaki Disease, or syndrome, is gradually becoming noticeable throughout the world. It’s history and definition has not been around for a long time. The syndrome’s causes, symptoms and effects, treatments, and outlooks for the future are still vague, but they are clear enough to perceive.

           To begin with, Kawasaki Syndrome’s history originates from the Red Cross Hospital in Tokyo, Japan. In 1961, a four year old boy was rushed to the hospital with an unusual rash and fever (Kim 2006, [53] ). Japanese pediatrician Tomisaku Kawasaki looked over this case and found it to be a new disease. Thus, he published his findings in 1967; it became known as Kawasaki Disease, naming it after himself (Kawasaki Disease Foundation 2011, [100]).Another pediatrician, named Takajiro Yamamato, found that cardiac involvement was associated with this disease. The first Japanese nationwide survey was conducted in 1970, describing ten cases of sudden cardiac death that resulted from complications of aneurysms developed in the coronary arteries following after the Kawasaki disease (Kim 2006, [54]). Cases of Kawasaki Disease outside of Japan were announced afterwords, approaching Hawaii first in 1976 (CDC 2010, [94]). Later, scientists changed the name of this “disease” to Kawasaki Syndrome. This disorder was not published in English until 1974 (Kim 2006, [54]). In 2000, a survey conducted that 77% of children younger than 18 diagnosed with the disease were 5 years old or younger. Children hospitalized with Kawasaki Syndrome showed an increase in 2006, but the percentage of children 5 or younger affected remained the same (CDC 2010, [96]). 

              Kawasaki Syndrome is defined as a condition that inflames the walls of small and medium sized arteries, including the coronary arteries, which supplies your heart with blood (Mayo Clinic 2011, [103]). This syndrome is originally called mucocutaneous lymph node syndrome because it also affects the skin, mouth, lymph nodes, and mucous membranes (Shiel Jr. 2011, [114]). It is a serious illness that often affects children under the age of five. Surprisingly, it is also the leading cause of acquired heart disease in children. Although the Kawasaki syndrome mainly affects younger children, it is possible for older people to obtain the disease (Kawasaki Disease Foundation 2011, [99]). Kawasaki Syndrome mostly affects Japanese and Korean children, but can have an effect on children of every descent (Klein 2008, [2]). About 19 out of every 100,000 kids in the United States acquires this syndrome, of which boys outweigh the girls. It usually occurs in the winter and spring, with occasional community-wide outbreaks (CDC 2010, [96]). Thankfully, it is not contagious (Kawasaki Disease Foundation 2011, [99]).

             The cause of Kawasaki Syndrome is unknown, although seasonal outbreaks indicate a possible infectious source. It is suggested that a bacterial superantigenic toxin triggers Kawasaki Disease. An outbreak of the disease after exposure to carpet cleaner supports arguments for an environmental cause (Wolff 2007, [85]). For example, John Travolta’s son, Jett Travolta, was hospitalized with Kawasaki Syndrome when he was 15 months old. He  later died at the age of 16. “It had nothing to do with the chemicals in the shampoo or rug fibers, but more likely with bacteria released into the air. There are whole ecosystems in a carpet... bacteria, spores and fungi that live and grow there.” (Finn 2009, [14]). Studies also found that the disease is reported more frequently among children from higher socioeconomic groups, of which Jett Travolta was certainly a member of. Brian Littrell’s (of the Backstreet Boys) son, Baylee was diagnosed with Kawasaki disease at six years old (Finn 2009, [12]). The cause of KD is unknown, although an agent, like a virus, is suspected. There is no currently accepted scientific evidence that KD is caused by exposure of carpet cleaning chemicals (Kawasaki Disease Foundation 2011, [100]). Efforts to identify an infectious agent in Kawasaki disease with conventional bacterial and viral cultures, serological methods, as well as with animal inoculation, have failed to identify an infectious cause. A genetic influence on disease susceptibility is suspected because Kawasaki disease is over-represented among Asian and Asian-American populations (Kim 2006, [70]). It may be an autoimmune disorder: a condition that occurs when the immune system mistakenly attacks and destroys healthy body tissue (Zieve 2009, [123]).

            Although the cause of Kawasaki Syndrome is uncertain, the effects from it are clear.  Kawasaki disease is characterized by various symptoms that occur in three stages: acute, subacute, and chronic (Wolff 2007, [87]). Symptoms begin at the acute stage with a cough, a runny nose, and a high fever that reaches more than 102 degrees fahrenheit, often attaining 104 degrees (Zieve 2009, [124]). This fever usually lasts for at least five days (Kawasaki Disease Foundation 2011, [101]). Extremely blood shot eyes (Zieve 2009, [124]), called conjunctivis (Shiel Jr., [113]), form along with red, chapped, or cracked lips that are inflamed (Zieve 2009, [124]). At worst-case, the lips can also bleed (Kim 2006, [57]). Kawasaki syndrome is also characterized as an inflammation of the blood vessels (Kawasaki Disease Foundation 2011, [101]). Most of the body becomes inflamed, including the inflammation of the tongue, often referred to as “strawberry tongue” (Shiel Jr. 2011, [115]). Lymph nodes in the neck usually start to swell, along with the reddening and swelling of the hands and feet. Rashes develop, especially on the trunk and genital areas of the body. Irritability is also an acute stage symptom(MayoClinic 2011, [104]). At the second phase of Kawasaki Syndrome, or the subacute stage, symptoms include skin peeling of the hands and feet, joint pain, abdominal pain, diarrhea, and vomiting (MayoClinic 2011, [105]). The peeling of the skin is due to dehydration. These breaks in the skin rupture into the immune system, making the patient more susceptible to infections (Patricia 2009, [130][131]). “Long term follow up of 259 cases of Kawasaki disease is led to the observation that 11% of children have episodes of recurrent peeling of the skin for several years after their recovery.” (Michie 2000, [98]). I was once part of this group of 11%. After recovering from this syndrome, my skin continued to peel at random times. Thankfully, my skin peeling had recently stopped roughly two years ago. A lack of certain vitamins, especially Vitamins A and B, could also be another factor responsible for this condition (Patricia 2009, [130]). Signs and symptoms slowly start to disappear during the third phase, or the chronic stage, unless complications develop. Each phase continues longer than the one previous to it. This last stage in the disease can persist for as long as 8 weeks (MayoClinic 2011, [106]). When cases of Kawasaki Syndrome proceed untreated, it can lead to other serious complications. The American Heart association reports that one in every five children diagnosed with Kawasaki Syndrome will suffer heart-related damage. Myocarditis, or inflammation of the heart muscle, can make it difficult for the heart to pump blood effectively throughout the body. This can result in discomfort, fatigue, and heart strain. Inflammation of the heart can also cause mitral valve regurgitation, or the back-flow of blood into the heart. Abnormal heart rhythm, or arrhythmia, can reduce the efficiency of heart activity and may lead to heart attacks. Another dangerous outcome of untreated Kawasaki disease is aneurysms. Aneurysms are balloons of blood that form in one spot. They can leak or burst, which causes blood to be released from the artery into the body. Approximately 20% of children with untreated Kawasaki disease develop coronary artery aneurysms (Choi 2003, [16]). Vasculitis is the inflammation of the blood vessels,which mostly affects the coronary arteries (Klein 2008, [5]). Other complications that can result from untreated Kawasaki Syndrome are gingivitis, a type of gum disease (Shiel Jr. 2011, [115]), and serious joint pains leading to arthritis (Otto 2010, [135]).

        Unlike other diseases, direct treatment for Kawasaki Syndrome cannot be given instantly. There is no specific test available to diagnose the young Kawasaki Syndrome, so it is a process of ruling out  other diseases that cause similar signs and symptoms (MayoClinic 2011, [111]). Treatment should begin as soon as possible within ten days of being diagnosed with Kawasaki syndrome (Klein 2008, [9]) in order to prevent injury to the heart and coronary arteries(Zieve 2009, [126]). Very few information concerning therapy later than the tenth day of diagnosis exists (Rowley and Shulman 2010). Children with Kawasaki disease are given initial treatment at the hospital because of the risk of serious complications. At the hospital, the doctor might run tests to omit other diseases (MayoClinic 2011, [112]) such as scarlet fever, measles, or an allergic drug reaction (Klein 2008, [8]). These examinations include urine tests, blood tests, electrocardiogram, echocardiogram (MayoClinic 2011, [111]), and chest x-rays (Zieve 2009, [125]). Treatment of Kawasaki Syndrome is intended to reduce inflammation, while long-term therapy is aimed at preventing damage (Kim 2006, [72]). One medical treatment, used to reduce inflammation in the acute stage, is high doses of aspirin (Kim 2006, [73]).Kawasaki Syndrome is a rare exception against the use of aspirin in children. Along with reducing inflammation, aspirin also helps in decreasing pain, joint inflammation, fever (MayoClinic 2011, [112]), and preventing blood clots (Shiel Jr. 2011, [119]) It is also a great support in reducing the risk of heart disease (Klein 2008, [9]). Once fever goes away, the dose of aspirin decreases from 80-100 mg/day to 3-5 mg/day. (Wolff 2007, [91]). These doses are continued for six to eight weeks until the patient shows no coronary changes (Kim 2006, [73]). Another standard treatment that is usually given is gamma globulin. Gamma globulin is an immune protein (MayoClinic 2011, [112]), or purified antibodies (Rowley and Shulman 2010), that can lower the risk of problems in the coronary arteries, such as aneurysms (Shiel Jr. 2011, [119]). Intravenous immunoglobulin (IVIG) is an ingredient of blood (Rowley and Shulman 2010) that is administered through the vein (Shiel Jr. 2011, [119]). Doses of IVIG are given every 10-12 hours (Kim 2006, [74]). Glucocorticoids, commonly known as steroids, is another type of treatment. It has been used to treat patients who have not responded to aspirin and IVIG (Kim 2006, [75]). Although steroid treatment is not recommended, it has reduced the rate of coronary aneurysms (Wolff 2007, [91]), fever, and hospitalization (Kim 2006, [75]). Rarely, Kawasaki Syndrome is treated with plasmapheresis, or plasma exchange, which is also used when patients do not respond to aspirin and IVIG. In plasmapheresis, plasma is removed from the blood and replaced with protein fluids. This removes antibodies and proteins that cause inflammation (Shiel Jr. 2011, [119]). Children diagnosed with Kawasaki Syndrome usually recover after 24 hours of treatment (Rowley and Shulman 2010). The disease lasts for about 12 days  for patients without treatment, but they are more likely to be part of the 25% who develop heart disease and coronary artery problems (MayoClinic 2011, [112]).

               Outlooks for the future are still unclear, but recent awareness of Kawasaki Syndrome has risen as incidents of the disease have inclined around the world (Dahdah 2010, [24]). Researchers are seeking ways to detect which diagnosed patients are at risk for developing coronary artery aneurysms (Shiel Jr., [120]). Over the next five years, scientists anticipate the identification of genes that influence Kawasaki Syndrome, as well as the development of coronary artery aneurysms. The best way to improve diagnosis and treatment would be to use bioinformatics of DNA and RNA sequences from tissues of Kawasaki syndrome patients (Rowley and Shulman 2010, [50]). Clinicians should consider Kawasaki Syndrome in patients with abnormal symptoms, and publish these details. (Wolff 2007, [92]). This would greatly transform tests, improve treatments, and help prevent this childhood illness (Rowley and Shulman 2010, [50]).

          Overall, Kawasaki Syndrome is a frightening disease that is  becoming recognized throughout the globe. It is gradually turning into an important topic for research, which can later help update its definition, causes, symptoms, and effects. Improvements in treatments can give us hope in later preventing Kawasaki Syndrome, and the outlook of research in the future looks successful. I am glad that I survived through the harshness of the Kawasaki syndrome. With further research, we can save many people that are diagnosed with this horrifying disease. They can become lucky like I am------ living happy and healthy------ and have a chance at life.