Kawasaki disease is a potentially life-threatening disease and is now ahead of rheumatic fever as the most common cause of acquired heart disease in children in the U.S. Hawai'i has the highest rate of Kawasaki's in the nation. An outbreak of 33 cases, apparently the first in the U.S., occurred in Hawai'i in early 1978. Outbreaks often occur every three to five years.

Though most frequent in Asians and Pacific Islanders, kids of African and European descent also can be affected. In fact, Japanese-Americans in Hawai'i have an even higher rate of Kawasaki's than Japanese kids in Japan, where it was discovered. Thought to be caused by an infection by a yet unknown agent, it causes inflammation of blood vessels throughout the body. Weakening of heart vessels with ballooning occurs in 20 to 25 percent of those who are not treated, sometimes leading to heart attacks.

Though standard textbooks report the age of peak occurrence as 18 to 24 months, in Hawai'i Kawasaki's is most common in infants less than a year old. Eighty percent are younger than 5. Kids older than 8 rarely develop Kawasaki's, while the diagnosis is often delayed in infants younger than 6 months because of atypical symptoms.

The symptoms include a fever that may come and go, lasts at least five days, and generally tops 102 and often 104 degrees. At least four of the following five features clinch the diagnosis, though they may not all be there at once: rash, conjunctivitis, redness in the mouth and lips, swelling and redness of the palms and soles of the hands and feet, and swollen lymph glands, typically a large one on one side of the neck. The mouth and lip redness can include lip dryness or cracking or a strawberrylike tongue.

Kids and infants with fever more than four days but only two or three other features should have sedimentation rate and c-reactive protein screenings, as well as other tests. A heart ultrasound may be ordered based on lab results. There may also be joint pain, diarrhea, vomiting, abdominal pain and irritability.

With four features, the diagnosis can be made on Day 4 of the fever, but experienced doctors who have treated many Kawasaki patients may establish the diagnosis earlier. One such doctor would be Marian Melish, a John A. Burns School of Medicine professor of pediatrics, who in 1971 was the first person outside of Japan to recognize Kawasaki's. She helped develop intravenous gamma globulin as a major treatment to reduce damage to heart vessels, and is truly a pioneer in the field.

Dr. Landis Lum is a family-practice physician for Kaiser Permanente and an associate clinical professor at the University of Hawai'i-Manoa John A. Burns School of Medicine. Send your questions to: Prescriptions, Island Life, The Advertiser, P.O. Box 3110, Honolulu, HI 96802; islandlife@honoluluadvertiser.com; or fax 535-8170. This column is not intended to provide medical advice.