[How does the time of diagnosis affect the course of disease in children with kawasaki syndrome? A retrospective analysis at one center].

BACKGROUND

Kawasaki syndrome was described for the first time by Tomisaku Kawasaki in 1967. This disease is characterized by panvasculitis of the small blood vessels of the skin, the mucous membranes, the internal organs and the coronary vessels and has an unclear etiology. Inflammatory changes in the coronary vessels or late diagnosis are prognostically unfavorable for the early and late mortality.

AIM OF THE STUDY

Since two of our patients with Kawasaki syndrome with a short, severe course died despite receiving state-of-the-art treatment, we retrospectively evaluated the medical records of all the children we have treated since October 1978 with regard to the symptoms at the time of diagnosis, intervals between the onset of the disease, diagnosis, beginning of treatment and the result of treatment.

PATIENTS

Kawasaki syndrome was diagnosed in 80 patients in the period from October 1978 to October 2007. The patients were grouped according to the phase of the disease and the number of organs affected at the time of diagnosis (Asai-Score) as well as the treatment carried out. The time of the first presentation for diagnosis by the pediatrician was also considered.

METHOD

This is a single-institution retrospective analysis of the medical records, echocardiography and angiography findings of all patients. In view of the change of therapy in that year, patients who had been diagnosed before 1987 were compared with those diagnosed after 1987.

RESULTS

Before 1987, the patients were treated solely with high doses of acetylsalicylic acid (50-100 mg/kg/day p.o. over two to four weeks). Out of a total of 36 patients, 13 showed involvement of the coronary arteries that persisted in seven patients despite treatment. After 1987, all patients received intravenous immunoglobulins (4 x 0.5 g/kg/day resp. 1 x 2 g/kg i.v. over 12 hours). In 18 out of 44 patients, the coronary arteries were affected at the time of diagnosis, but this did not persist in any of the patients. One child died in each group. Comparing the two treatment groups also revealed that a physician was consulted for the first time after a very much shorter duration of the disease in the second treatment period (3+/-1.8 vs. 6+/-2.4 days after onset of the illness) and that a pediatrician was consulted much more frequently as the first port of call. This was reflected in a significantly earlier beginning of treatment and a simultaneous significantly lower Asai score.

CONCLUSION

The retrospective evaluation of all medical records did not reveal any plausible explanation for the fatal course of the disease in one child in each of the two treatment periods. Besides the combination therapy with intravenous immunoglobulin and oral administration of acetylsalicylic acid, the greater age and the earlier commencement of treatment appeared to be salient factors resulting in complete cure of the disease in the surviving patients in the second period of treatment.