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Kawasaki disease

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Kawasaki disease is a unique disease that affects children of all races. The disease primarily targets children less than 5 years of age. Though extensive research has been done, scientists can only speculate on the actual cause of the illness. Kawasaki disease targets the coronary arteries and thickens blood vessels. If left untreated, a coronary aneurysm will develop and death occurs.

History

In 1961, a Japanese doctor, Tomisaku Kawasaki, encountered the first recorded case of Kawasaki disease (KD). After encountering KD, Kawasaki published a report in Japan, explaining the disease. Kawasaki didn't think that KD was linked to any complications, but in 1970, a nationwide search found 10 autopsy cases of KD patients who died of cardiac issues.[1] It was not until 1974, when Kawasaki published his first English-language report, that the connection was made between KD and coronary artery vasculitis.

Since that time, Japanese doctors have determined, through rigorous study, the unique factors to KD.

  • Initially, KD occurred as national epidemics before settling into sporadic cases limited to specific regions.
  • KD occurs in 120 to 150 children per 100,000 children less than 5 years of age.
  • 85% of cases are found in children patients.
  • KD is found to be 1.5 times more common in male patients.

United Sates doctors have also found these to be true, but the national report of KD cases in American children is immensely less than the Japanese: 4 to 15 cases per 100,000 children less than 5 years of age. However, despite all the studies, doctors still have yet to discover the definite cause of KD and whether or not it is a contagious disease. Studies are pointing more towards a non-contagious pathology.[1]

Symptoms

Symptoms of Kawasaki Disease.

The identifiable symptoms of KD begin with a rather high fever greater than 102 degrees Fahrenheit. The fever temperature can be as high as 104 degrees Fahrenheit. This fever, signaling KD, lasts for an average of 5 days and upwards to 2 weeks. It cannot be quelled by fever reducing drugs (acetaminophen or ibuprofen). There are many other identifying symptoms of KD. These include, but are not limited to: extremely bloodshot or red eyes, red mucous membranes in the mouth, strawberry tongue, white coating on the tongue, or prominent red bumps on the back of the tongue, red palms of the hands, swollen hands and feet, and swollen lymph nodes (frequently only one lymph node is swollen), particularly in the neck area. These symptoms are the classic signs of KD. However, these symptoms can be accompanied by irritability, diarrhea, vomiting, and abdominal pain, as well as cough and runny nose. If many of these signs are present in the patient, doctors conclude that the patient is infected with KD.[2]

Diagnosis

On the ninth day of Kawasaki disease, acute acalculous distention of the gallbladder (hydrops) was identified by an abdominal ultrasound in a one-year-old boy.

There is no specific type of test that can be done to diagnose KD. One of the most identifying signs of KD is a high fever lasting for a period equal or longer than 5 days. However, other illnesses can have a fever for 5 days or more. That is why doctors look of the other signs of KD before making the prognosis. They run many tests to confirm their suspicions if the fever has lasted for more than 5 days and has company of many of the classic symptoms. These tests include a chest x-ray, a complete blood count, an ECG and an echocardiography. These two (ECG and echocardiography) can show signs of myocarditis, pericarditis, arthritis, aseptic meningitis, and inflammation of the coronary arteries.[2]

Treatment

Once KD has been diagnosed upon the patient, treatment must begin immediately. The victim must be treated within seven to ten days of contracting the illness to prevent major coronary complications in the future. The major treatment of KD is an injection of intravenous immunoglobulin (IVIG). The IVIG reduces the fever and decreases the risk of a coronary aneurysm developing in the patient. In addition to the IVIG, a high dose of aspirin should be administered to control the fever. Once the temperature begins to decrease, the aspirin dose will be lowered. [3]

Each child reacts differently to the IVIG treatment. If the treatment of KD is not as successful as desired by the medical professional, the patient may develop coronary artery problems. Follow-up appointments are made to test for problems. If a minor coronary problem shows itself, the patient may be required to take an aspirin regiment. If a coronary aneurysm (blood-filled enlargement of an artery) develops, there are three common treatments for the problem: anticoagulant drugs, coronary angioplasty and stents, and coronary artery bypass surgery.

  • Anticoagulant drugs reduce the ability for blood to clot, thus not allowing an aneurysm to build up in the arteries.
  • A coronary angioplasty procedure involves running a catheter from the groin area and into the problem artery. Then, a small balloon is inflated to allow greater blood passage in the artery. A small metal stent may also be placed in the artery to keep the passage open for blood flow.
  • A coronary artery bypass surgery is a surgery that has a different blood vessel act as the artery since the original artery is beyond repair. This occurs when a bypass is made around the damaged artery. [4]

Modern Study

Schematic diagram of the pathogenesis of Kawasaki disease.

Children's Memorial Hospital is one of the leading facilities in the study of KD. Their medical trials to counteract the disease began in the 1980's. Through their research and studies, they have established the main treatment for KD by performing three separate treatment trials. The first treatment trial yielded that the gamma globulin, administered over a course of four days along with a high-dose of aspirin, was highly effective in preventing coronary artery complications and reduced many of the symptoms of KD. The second treatment trial compared the use of a single, large dose of IVIG to a small dose of IVIG over four days (each with a high dose of aspirin). The trial showed that the large, single dose of IVIG was more effective than the smaller dose over four days. The third trial was another comparison but with low and high doses of aspirin (while a large, single dose of IVIG remained the same). This trial showed that the high dose of aspirin was more effective of managing fever and reducing symptoms of KD.[5]

One doctor from Children's Memorial Hospital, Dr. Anne Rowley, has been working on finding a specific diagnostic test for KD and a vaccine to prevent the disease. Other researchers have suspected an unknown virus as a possible cause of KD. Through her research, Rowley has given significant evidence to support that idea. She and her team of researchers made a discovery, pointing towards a single respiratory virus or a closely related group of viruses. [6]

List of cardiovascular diseases

References

  1. 1.0 1.1 Burns, Jane; Kushner, Howard; Bastian, John; etc. Kawasaki Disease: A Brief History American Academy of Pediatrics. August 1, 2000. Accessed: October 20, 2011.
  2. 2.0 2.1 Zieve, David and Gonter, Neil J. Kawasaki Disease A.D.A.M. PubMed Health. Reviewed: June 20, 2011.
  3. Children’s Hospital Boston Kawasaki disease Children’s Hospital Boston, Harvard Medical School. Accessed: October 20, 2011
  4. Mayo Foundation for Medical Education and Research Kawasaki Disease Mayo Clinic. Accessed: October 20, 2011
  5. Children’s Memorial Hospital physicians Research on Kawasaki Disease Children’s Memorial Hospital. Last Reviewed: May 2009. Accessed October 20, 2011.
  6. Children's Memorial Hospital physiciansRowley zeroes in on cause of Kawasaki disease Children’s Memorial Hospital. Accessed November 16, 2011.