Kawasaki disease (KD) is a childhood illness that causes fever and an acute inflammation of the body's blood vessels. Approximately 80 percent of cases occur in children under the age of 5 says the Kawasaki Disease Foundation (KDF). The exact cause remains unclear, but it is thought to be the body's response to some type of infection, explains the KDF. The autoimmune system takes over to fight the infection, causing acute, dangerous inflammation of blood vessels. The main area of involvement in KD is the cardiovascular system, with children under 12 months of age at the highest cardiovascular risk. Early detection and treatment is critical; for without treatment, the KDF estimates that approximately 25 percent of children with KD will develop long-term cardiac problems.
IV Gamma Globulin
IV gamma globulin, also known as IVIG (intravenous immunoglobulin), is the most effective treatment for KD. IVIG is believed to work by slowing down the immune system, which appears to be in overdrive. If given within 10 days of the start of the illness, it is shown to reduce the incidence of coronary artery abnormalities, explains Alexandra F. Freeman, M.D., and Sanford T. Shulman, M.D., in the October 2006 issue of the "American Family Physician" journal. High-dose therapy (2 g/kg) given over 10 to 12 hours is recommended, with additional infusions continued as necessary.
Aspirin
High-dose aspirin is given initially as an anti-inflammatory measure. The recommendation is 80 to 100 mg/kg/day, administered every six hours, until the fever subsides, states Freeman and Shulman. Once the fever subsides, a lower dose of aspirin is given to help reduce platelets (3 to 5 mg/kg/day). Because the platelet count in patients with KD is elevated, low-dose aspirin therapy is continued, until the platelet count returns to normal (usually six to eight weeks).
Steroids
The use of steroids for the treatment of KD is not part of the traditional standard of care; although, a limited study in the February 2007 edition of "The New England Journal of Medicine" by Jane W. Newburger, M.D., et al, suggested its use to improve outcomes. In 2007, the results of a more comprehensive study were published in the "Journal Watch of Infectious Diseases" by Robert S. Baltimore, M.D. This study found that steroid use was not beneficial overall in the treatment of KD but may benefit a small group of patients who were at a higher risk for coronary artery abnormalities.
Summary
Kawaski disease is a potentially serious illness that can cause severe, long-term damage to the heart if not treated. Most children who are treated with IVIG and aspirin therapy will recover fully. However, death rates increase when cardiovascular complications arise. Children with a fever for five or more days, accompanied by bilateral conjunctivitis (without discharge), dryness or reddening of the lips or tongue, peeling of the hands and feet, and rash on the torso, legs and groin, should be immediately evaluated by a physician to rule out a diagnosis of Kawasaki disease.
References
- AAFP: "Kawasaki Disease: Summary of the American Heart Association Guidelines"; Alexandra F. Freeman, M.D., and Stanford T. Shulman, M.D.; October 2006
- "Wong's Essentials of Pediatric Nursing"; Marilyn J. Hockenberry, Ph.D., RN-CS, PNP, FAAN; 2005
- JournalWATCH Specialties: "Steroids for Children with Kawasaki Disease?"; Robert S. Baltimore, M.D.; February 2007
- The New England Journal of Medicine: "Randomized Trial of Pulsed Corticosteroid Therapy for Primary Treatment of Kawasaki Disease"; J.W. Newburger et al; February 2007
- Kawaski Disease Foundation
