942例川崎病的临床分析

目的总结川崎病（Kawasaki disease,KD）的临床特征,探讨KD预后与治疗的关系。方法回顾性分析2000年1月—2004年12月期间942例住院KD患儿的临床资料：（1）比较典型KD与不完全性KD（incomplete KD）的临床特征;（2）总结KD对静脉注射免疫球蛋白（intravenous immune globulin,IVIG）治疗无反应的影响因素;（3）随访观察其中的510例KD患儿,比较IVIG 1g／kg和2g／kg治疗的远期疗效。结果（1）942例中,典型KD774例,不完全性KD168例。不完全性KD冠状动脉病变（coronary artery lesion,CAL）发生率较高（P〈0．05）,除肛周脱屑外,其他临床症状发生较少,出现较晚（P〈0．05或0．01）;（2）与IVIG治疗反应敏感组比较,IVIG治疗无反应组的发热时间较长,血红蛋白（Hb）、白蛋白（ALB）、血细胞比容（Hct）及血小板（PLT）较低（P〈0．05或0．01）;（3）WIG 1g／kg和2g／kg治疗组在KD发病后2年内,CAL的恢复率及新发生率,两组差异均无统计学意义（P〉0．05）。结论（1）不完全性KD的CAL发生率较高,肛周脱屑可以作为不完全性KD的早期诊断依据之一;（2）急性期发热时间较长,PLT无升高及Hb、Hct、ALB持续降低是IVIG治疗无反应的影响因素;（3）IVIG 1g／kg和2g／kg治疗KD的疗效在KD发病后2年内相似。

Clinical analysis of 942 cases of Kawasaki disease

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.