Objective: The study was designed to investigate the clinical characteristics and the effects of therapeutic proposal on Kawasaki disease (KD).
Methods: Clinical features, diagnosis and treatment for totally 942 patients with KD hospitalized during Jan, 2000 to Dec, 2004 were reviewed. Clinical features of typical and incomplete KD were compared. Also, influential factors for KD resistant to intravenous immune globulin (IVIG) therapy were analyzed. Five hundred and ten cases were followed up for analyzing the prognosis of coronary artery lesion (CAL).
Results: (1) 774 cases were diagnosed as typical KD, and 168 cases as incomplete KD. The incidence of infants with incomplete KD was higher than that of infants with typical KD (18.5% vs. 10.1%, P < 0.01). As compared with typical KD, the cases of incomplete KD had a long duration of fever before final diagnosis [(7.7 +/- 2.9) d vs. (7.0 +/- 2.4) d, P < 0.01], high hemoglobin level [Hb, (106.6 +/- 13.4) g/L vs. (103.5 +/- 12.3) g/L, P < 0.01], high hematocrit [Hct, (32.0 +/- 4.3)% vs. (31.0 +/- 4.0)%, P < 0.01], and high prevalence of CAL (23.8% vs. 16.8%, P < 0.05), respectively. The occurrence rate and emerging time of clinical manifestations in incomplete KD and in typical KD were presented, respectively: non-exudative conjunctivitis [occurrence rate, 64.9% vs. 93.5%; emerging time, (4.4 +/- 1.4) d vs. (4.0 +/- 1.6) d, respectively (P < 0.05 or P < 0.01)], erythema and cracking of lips [occurrence rate, 50.6% vs. 94.8%; emerging time, (4.9 +/- 1.4) d vs. (4.5 +/- 1.6) d, respectively (P < 0.05 or P < 0.01)], rash [occurrence rate, 35.1% vs. 87.7%; emerging time, (3.9 +/- 1.9) d vs. (3.4 +/- 1.7) d, respectively (P < 0.05 or P < 0.01)], erythema and edema of extremity [occurrence rate, 26.8% vs. 71.4%; emerging time, (6.7 +/- 1.5) d vs. (5.3 +/- 1.7) d, respectively (P < 0.01)], cervical lymphadenopathy [occurrence rate, 34.5% vs. 68.0%; emerging time, (4.3 +/- 2.5) d vs. (3.6 +/- 2.2) d, respectively (P < 0.05 or P < 0.01)], strawberry tongue [occurrence rate, 31.0% vs. 59.8%; emerging time, (5.6 +/- 2.2) d vs. (4.9 +/- 1.8) d, respectively (P < 0.05 or P < 0.01)], membranous desquamation of fingertips [occurrence rate, 34.5% vs. 56.3%; emerging time, (11.7 +/- 3.3) d vs. (10.3 +/- 2.7) d, respectively (P < 0.01)], and desquamation peri-anus [occurrence rate, 42.9% vs. 50.0%; emerging time, (6.7 +/- 2.7) d vs. (6.9 +/- 2.5) d, respectively (P > 0.05)]. Except for peri-anus desquamation, other clinical manifestations in incomplete KD were sporadical as compared to typical KD. (2) Six per cent (51/857) of cases were resistant to the IVIG therapy. As compared to the group responding to IVIG therapy, high prevalence of CAL (31.4% vs. 17.1%, P < 0.05), long fever duration [(10.6 +/- 3.9) d vs. (7.5 +/- 2.3) d, P < 0.01], low Hb level [(99.9 +/- 14.1) g/L vs. (104.3 +/- 12.4) g/L, P < 0.01], low Hct [(30.1 +/- 4.5)% vs. (31.2 +/- 4.0)%, P < 0.05], low platelet [PLT, (256.9 +/- 142.4) x 10(9)/L vs. (309.7 +/- 131.5) x 10(9)/L, P < 0.05], and low albumin level [ALB, (27.8 +/- 8.4) g/L vs. (33.5 +/- 6.7) g/L, P < 0.01] were found in the group resistant to IVIG therapy, respectively. (3) In patients who received IVIG 1 g/kg and 2 g/kg, the recovery rates from CAL were 83.1% and 89.7% (P > 0.05), respectively. The prevalence of CAL in those without CAL in acute and subacute stages was 0.9% and 3.5% (P > 0.05), respectively, during 2 year-follow-up period.
Conclusion: (1) Infants appeared to have more chances to suffer from incomplete KD. Incomplete KD had high prevalence of CAL. The peri-anus desquamation might be an important clue for early diagnosis of incomplete KD. (2) In acute stage, the influential factors for KD resistance to IVIG therapy included prolonged fever, non-elevated PLT, and persistent decrease in Hb, Hct and ALB levels. (3) Children receiving IVIG 1 g/kg and 2 g/kg had the similar effects on recovery and prevention from CAL within the first two years after KD onset.