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The Informed Parent

Kawasaki Syndrome

by Louis P. Theriot, M.D., F.A.A.P.
Published on Mar. 09, 2009

The world was saddened by the recent death of John Travolta’s son. There was an initial flurry of news reports stating that he died from complications of Kawasaki syndrome--a disease he had as a young child. Jett Travolta was 16 years old and apparently had suffered from seizures at the time of his death. This sparked much discussion in the media which provoked confusion and speculation. The parents of a patient of mine who had Kawasaki syndrome called in a panic, wondering if they had to worry about their son who was now an adolescent. Clearly there was much confusion and many inaccuracies with the early reports.

Kawasaki syndrome was first reported in 1967 by Dr. Tomisaku Kawasaki in Japan. It was described as acute febrile mucocutaneous lymph node syndrome. While it is more prevalent in the Asian population, Kawasaki syndrome (KS) has been seen in all races and has been reported worldwide.

KS occurs more often in boys than in girls. The majority of cases are seen in children under the age of five years. Most cases occur in the two-to-five year age range. The exact cause of KS is unknown. However, there are many different theories including a viral cause. To this date nothing has proved conclusive.

There are no specific tests available to make a diagnosis of KS, as it is purely a clinical diagnosis. The diagnostic features of KS include:

  1. fever of at least five days duration
  2. body rash that can take on many forms and can look like many other illnesses
  3. swollen hands and feet
  4. red and bloodshot eyes
  5. swollen red and cracked lips
  6. strawberry tongue
  7. swollen lymph nodes in the neck region.

Most patients with KS appear ill and irritable. In the resolution stages often there is peeling of the skin around the fingertips. Other organ systems can be involved with KS. The main concern is the heart. It has been reported that as many as 20% of untreated patients with KS can develop coronary artery aneurysms. This is a dilatation of the artery that serves the heart. It can lead to a heart attack and death.

While there is no treatment for KS itself, there is a standard treatment for patients with KS that can greatly decrease the risk of coronary aneurysm. This is intravenous gamma globulin and aspirin. The goal is to start this treatment regimen within ten days of the onset of the fever. It’s effectiveness is quite remarkable.

Seizures are in no way a part of KS. It was later clarified that Jett Travolta did have KS as a young child, but this played no role in his untimely death. There were also early reports that he might have contracted KS “from carpet cleaning chemicals” that were used in their home in Colorado. There was a paper published in the journal PEDIATRICS in May 1991 that was titled, “Outbreak of Kawasaki Syndrome in Denver, Colorado: Association With Rug and Carpet Cleaning.” While this was a hot topic of discussion in the early 1990’s, this association has been disproved and is no longer felt to be of any significance or relevance.

Unfortunately patients may present with many of the symptoms of KS but don’t quite fit the diagnostic criteria. These are the atypical cases of KS that are not so clear-cut. In these cases it is imperative to rule out any coronary artery dilation by doing a cardiac echocardiogram. Even if the results were negative, most experts would recommend treating this patient as a case of KS, regardless. The consequences of missing a case of KS are devastating.

Most patients who are treated for KS with the standard regimen do very well and fully recover. They usually need a repeat echocardiogram in a couple of months to be sure that everything is still normal. There have been reports of recurrences of KS, but this is very rare, on the order of 2%.

While I had my patient on the phone, I ardently assured him that his 9-year-old son, former KS patient, was well and at no risk for seizures or ANY complications from the KS that he had five years ago.

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