No new epidemiological data had been published on Kawasaki disease in
France since 1982. The authors sent questionnaires to 285 French pedia
tric centers, to gather data on children with Kawasaki disease, hospit
alized between January 1, 1990 and December 31, 1994. Patients were di
vided in 3 groups according to clinical criteria (complete, incomplete
, and doubtful syndrome). Evolution was estimated according to 4 possi
bilities (usual, prolonged, relapse within 30 days following onset of
apyrexia, or relapse after these 30 days). 168 centers answered (58% r
esponse rate) giving 550 cases sorted in 3;1 2 complete, 101 incomplet
e, and 7 doubtful cases of Kawasaki syndrome (these last 7 cases were
not taken into account because they were doubtful). The number of case
s varied from 82 to 126 per year. Mean patient age was 3.0 +/- 2.8 yea
rs. Patient sex ratio (boy/girl) was 1.76. Coronary artery abnormaliti
es were found in 17% of the whole sample (complete + incomplete) and i
n 21% of the complete syndrome group. There were 6 myocardial infarcts
(1.1% of the whole sample and 1.3% of the complete syndrome group). C
oronary artery abnormalities were more important in younger children:
64.5% of coronary artery abnormalities were found in children under 2
years of age, even though these children represented only 47% of the w
hole sample. Among biological data studied on admission and during evo
lution of the disease (white blood cell count, thrombocytes cell count
, erythrocyte sedimentation rate on the first hour, C reactive protein
, and fibrinogen) only thrombocyte cell count, on admission and at max
imum rate, was significantly higher in children with coronary artery a
bnormalities in the complete group. 136 children were treated by immun
oglobulins 400 mg/kg/day during five days or 500 mg/kg/day during four
days and 216 were treated by 1 g/kg/day during 2 days or 2 g/kg/day o
ne day. 88% of the complete group had an expected evolution. 6.8% had
a prolonged evolution, 4,5% relapsed within 30 days following onset of
apyrexia, and 0.7% relapsed after these 30 days. Death rate of the wh
ole sample and complete syndrome group reached 0.18% and 0.22% respect
ively. The survey response rate allows for a reliable estimation of ar
ound 200 cases of Kawasaki disease in France. Coronary artery abnormal
ities are still frequent in spite of gammaglobulin treatment. The deat
h rate sharply decreased from 8.5% in 1982 to 0.2% in 1994.