川崎病108例临床分析
摘要
川崎病（Kawasaki disease, KD）又称皮肤粘膜淋巴结综合征（mucocutaneous lymph node syndrome, MCLS），是一种病因及发病机制尚未完全明了的儿童血管炎综合征。资料表明近年该病发病率逐渐增高，尤其在日本、中国、美国，其心血管并发症已成为儿童获得性心脏病的主要原因，它可导致严重的心血管损伤，如冠状动脉瘤、冠状动脉狭窄和闭塞及心肌缺血等，严重影响儿童身体健康。早期诊断、及时适当治疗可减少并发症，改善预后。
本文通过对108例住院的川崎病患儿的临床特点（包括发病年龄构成比，主要的临床表现及特异性的改变）、辅助检查（实验室检查及其他相关检查）、及各种治疗方法（阿司匹林、静注丙种球蛋白、肾上腺皮质激素等）进行分析、总结，并将川崎病患儿分为三组分别给予不同剂量的静注丙种球蛋白(IVIG)，通过统计学检验得出静注丙种球蛋白1g/(kg.d) ×1天组与2g/(kg.d) ×1天组在平均热退时间（小时）及冠状动脉病变发生率方面差异无统计学意义，而400 mg/(kg.d) ×5天与前两组比较平均热退时间（小时）较前两组时间长，冠状动脉病变发生率较前两组高。显示静注丙种球蛋白剂量在1g/(kg.d)以上能有效预防冠状动脉病损的发生。
通过临床观察及分析，加强对不典型病例的认识，一旦疑为川崎病应尽早做心脏二维彩色超声心动图（UCG）如有冠状动脉扩张即使达不到美国心脏病协会（America Heart Association, AHA）制定的诊断标准，也建议及早应用IVIG以预防和减轻冠状动脉病损。对不典型病例的诊断还需结合辅助检查如血常规中白细胞（WBC）、血小板（PLT）增高，血红蛋白（HB）降低，血沉（EST）加快，C反应蛋白（CRP）增高，蛋白电泳α2增高，以助早期诊断。从而达到早期诊断，早期适当治疗，减少冠状动脉的损害，改善预后。同时要加强对本病的随访，观察冠状动脉病损的远期结果，采取相应措施。
Kawasaki disease is also named as mucocutaneous lymph node syndrome. It is a vasculitis syndrome. Its pathogeny and mechanism still remain elusive. It is reported that the incidence rate of this disease is gradually increasing, especially in U.S.A., Japan and China, and the incidence of heart complications in Kawasaki disease is now becoming the commonest cause of acquired heart diseases in children. It can result in severe coronary artery aneurysm, stenosis, occlusion, myocardial infarction and so on. It damages the health of children severely. Early diagnosis and appropriate treatment can decrease the complications and improve the prognosis. So the approach to diagnosis and treatment is essential.
The present study aimed at analyzing the clinical presentation, laboratory inspection and various treatments of 108 cases with Kawasaki disease. The clinical presentation mainly includes the constitutional rate of age, main symptoms and the characteristic change of this disease. To discussion the various treatments of aspirin, intravenous immunoglobulin(IVIG) and corticosteroid. The 108 cases with Kawasaki disease were divided into three groups, and each group was given difference dose intravenous immunoglobulin (IVIG). The results of statistic analysis showed that there is no statistic significant difference between the group of intravenous immunoglobulin (IVIG) 1g/(kg.d) ×1day and 2g/(kg.d) ×1day,but there is statistic significant difference between the group of intravenous immunoglobulin(IVIG) 400mg (kg.d) for 5 days and the front two groups. So in order to not only decrease the incidence rate of coronary artery lesion but also decrease the economic burden of patients. We can choice the optimal dose.
By clinical observation and analysis, we can improve our recognition to KD, especially to non-typical KD. So once we meet suspected cases, we should give them early check of UCG. If there is coronary artery lesion, we can
diagnose him as KD even though he dose not meet American Heart Association (AHA) criteria and give him IVIG early in order to decrease coronary artery lesion. As to non-typical KD, we should also combine clinical symptoms with laboratory inspections, such as the increase of WBC, PLT, EST, CRP and protein electrophoresis α2 and the decrease of HB, so that we can come to early diagnose and treatment to bring down the coronary artery lesion and meliorate the prognosis. And we should reinforce the follow-up at this disease and observe the long-term consequence of regressed coronary artery lesion and take corresponding measures.
本文通过对108例住院的川崎病患儿的临床特点（包括发病年龄构成比，主要的临床表现及特异性的改变）、辅助检查（实验室检查及其他相关检查）、及各种治疗方法（阿司匹林、静注丙种球蛋白、肾上腺皮质激素等）进行分析、总结，并将川崎病患儿分为三组分别给予不同剂量的静注丙种球蛋白(IVIG)，通过统计学检验得出静注丙种球蛋白1g/(kg.d) ×1天组与2g/(kg.d) ×1天组在平均热退时间（小时）及冠状动脉病变发生率方面差异无统计学意义，而400 mg/(kg.d) ×5天与前两组比较平均热退时间（小时）较前两组时间长，冠状动脉病变发生率较前两组高。显示静注丙种球蛋白剂量在1g/(kg.d)以上能有效预防冠状动脉病损的发生。
通过临床观察及分析，加强对不典型病例的认识，一旦疑为川崎病应尽早做心脏二维彩色超声心动图（UCG）如有冠状动脉扩张即使达不到美国心脏病协会（America Heart Association, AHA）制定的诊断标准，也建议及早应用IVIG以预防和减轻冠状动脉病损。对不典型病例的诊断还需结合辅助检查如血常规中白细胞（WBC）、血小板（PLT）增高，血红蛋白（HB）降低，血沉（EST）加快，C反应蛋白（CRP）增高，蛋白电泳α2增高，以助早期诊断。从而达到早期诊断，早期适当治疗，减少冠状动脉的损害，改善预后。同时要加强对本病的随访，观察冠状动脉病损的远期结果，采取相应措施。
Kawasaki disease is also named as mucocutaneous lymph node syndrome. It is a vasculitis syndrome. Its pathogeny and mechanism still remain elusive. It is reported that the incidence rate of this disease is gradually increasing, especially in U.S.A., Japan and China, and the incidence of heart complications in Kawasaki disease is now becoming the commonest cause of acquired heart diseases in children. It can result in severe coronary artery aneurysm, stenosis, occlusion, myocardial infarction and so on. It damages the health of children severely. Early diagnosis and appropriate treatment can decrease the complications and improve the prognosis. So the approach to diagnosis and treatment is essential.
The present study aimed at analyzing the clinical presentation, laboratory inspection and various treatments of 108 cases with Kawasaki disease. The clinical presentation mainly includes the constitutional rate of age, main symptoms and the characteristic change of this disease. To discussion the various treatments of aspirin, intravenous immunoglobulin(IVIG) and corticosteroid. The 108 cases with Kawasaki disease were divided into three groups, and each group was given difference dose intravenous immunoglobulin (IVIG). The results of statistic analysis showed that there is no statistic significant difference between the group of intravenous immunoglobulin (IVIG) 1g/(kg.d) ×1day and 2g/(kg.d) ×1day,but there is statistic significant difference between the group of intravenous immunoglobulin(IVIG) 400mg (kg.d) for 5 days and the front two groups. So in order to not only decrease the incidence rate of coronary artery lesion but also decrease the economic burden of patients. We can choice the optimal dose.
By clinical observation and analysis, we can improve our recognition to KD, especially to non-typical KD. So once we meet suspected cases, we should give them early check of UCG. If there is coronary artery lesion, we can
diagnose him as KD even though he dose not meet American Heart Association (AHA) criteria and give him IVIG early in order to decrease coronary artery lesion. As to non-typical KD, we should also combine clinical symptoms with laboratory inspections, such as the increase of WBC, PLT, EST, CRP and protein electrophoresis α2 and the decrease of HB, so that we can come to early diagnose and treatment to bring down the coronary artery lesion and meliorate the prognosis. And we should reinforce the follow-up at this disease and observe the long-term consequence of regressed coronary artery lesion and take corresponding measures.
引文
龚方戚，白石裕比湖，桃井真里子。川崎病患儿冠状动脉病变的随访及超声与造影的对照研究。中华儿科杂志。2000，4038：634-635。
华益民，朱琦，刘瀚旻，等。川崎病冠状动脉病变的远期预后观察。中国实用儿科临床杂志，2000，39（5）：306。
胡亚美，江载芳主编。诸福棠实用儿科学。第7版，北京，人民卫生出版社，2002： 698-705。
梁翊常，王乃坤，柴晓敏。我国川崎病概况。中国实用儿科杂志，1995，120：302-303。
Shulma ST, Melish M, Inoue O, et al. Immunoglobulin allotypic markers in Kawasaki disease [J]. J Pediatr, 1993; 122: 84.
Cesan D, Pavan E, Sacchetto A, et al. Endothelin-1: A scientist's Curiosity, or a player in ischemic heart disease; Am Hearts, 1996; 132(6): 1236.
Brogan, PA, Bose A, Burgner D, Tulloh R, Michie C, Klein N, Booy R, Levin M, Dillon MJ. Kawasaki disease.: an evidence based approach to diagnosis, treatment, and proposals for future research.. Arch Dis Child, 2002 Apr, 86(4): 286-290.
李万镇。加强对川崎病诊治研究。中华儿科杂志，2002，40（2）：65-67。
Witt MT, Minich LL, Bohnsack JF, et al. Kawasaki disease: more patients are being diagnosed who do not meet American Heart Association criteria. Pediatrics, 1999,104(1): 10.
Burns JC, Kushner HI, Bastian JF, et al. Kawasaki disease: A brief histry, pedia tric, 2000,106(2): 27.
Akagi T, Kato H, Inoue O, Sato N. A study on the optimal dose of aspirin therapy in Kawasaki disease-clinical evaluation and arachidonic acid metabolism.Kurume Med J.1990,37(3): 203-208.
Fuiushok, Kamiye T, Nakano H, et al. High-dose intravenous gamma globulin for Kawasaki disease (in Japanese)[J]. Clin Rev, 1983,17:659.
Rowley AH, Shulman ST. Kawasaki disease.In: Behrman RE, Kliegman RM, Arvin AM, eds. Nelson textbook of pediatrics.Philadelphia: Sadnders, 2000,725-727.
Oates-Whitehead RM, Baumer JH, Haines L, Love S, Maconochie IK, Gupta A, Roman K, Dua JS, Flynn I. Intravenous immunoglobulin for the treatment of Kawasaki disease in children. Cochrane Database Syst Rev. 2003;(4): CD004000.
李永柏，易岂建，李秋，等。优选静脉免疫球蛋白剂量治疗川崎病临床与免疫学效应研究。中华儿科杂志，1999，37（2）：97。
顾承萍，傅雅珠。不同剂量丙种球蛋白治疗川崎病疗效分析。小儿急救医学，2003，10（3）：162-163。
周太光，刘春艳，胡晓。不同剂量丙种球蛋白对川崎病心血管系统的影响。小儿急救医学，2003，10（2）：111。
Kato H, Ichinose E, Kawasaki T. Myocardial infarction in Kawasaki disease: clinical analyses of 195 cases. J Pediatr, 1986,108:923-927.
Zenji N, Kihei M, Takeshi O, et al. Randomized controlled study of intravenous prednisolone and gammaglobulintreatment in 100 cases with Kawasaki disease. In: Hirohisa K, ed. Kawasaki disease. Netherland: Elsevier, 1995.328-331.
Wriht D, Newburger JW, Baker A, et al. Treatment of immune globulin-resistant Kawasaki disease with pulsed doses of corticosteroids [J]. Pediatr, 1996; 128(1): 146-149.
Fukunishi M, Kikkawa M, Hamana K, et al. Prediction of non-responsiveness to intravenous high-dose γ-globulin therapy in patients with Kawasaki disease at onset [J]. J Pediatr, 2000; 137(2): 172-176.
杜军保，张清友。川崎病治疗的若干新观点。实用儿科临床杂志，1999，37（2）：97。
王宏伟，程佩萱。小儿风湿病诊治进展。中国实用儿科杂志，2002，17（5）：267。
华益民，朱琦，刘瀚旻，等。川崎病冠状动脉病变的远期预后观察。中国实用儿科临床杂志，2000，39（5）：306。
胡亚美，江载芳主编。诸福棠实用儿科学。第7版，北京，人民卫生出版社，2002： 698-705。
梁翊常，王乃坤，柴晓敏。我国川崎病概况。中国实用儿科杂志，1995，120：302-303。
Shulma ST, Melish M, Inoue O, et al. Immunoglobulin allotypic markers in Kawasaki disease [J]. J Pediatr, 1993; 122: 84.
Cesan D, Pavan E, Sacchetto A, et al. Endothelin-1: A scientist's Curiosity, or a player in ischemic heart disease; Am Hearts, 1996; 132(6): 1236.
Brogan, PA, Bose A, Burgner D, Tulloh R, Michie C, Klein N, Booy R, Levin M, Dillon MJ. Kawasaki disease.: an evidence based approach to diagnosis, treatment, and proposals for future research.. Arch Dis Child, 2002 Apr, 86(4): 286-290.
李万镇。加强对川崎病诊治研究。中华儿科杂志，2002，40（2）：65-67。
Witt MT, Minich LL, Bohnsack JF, et al. Kawasaki disease: more patients are being diagnosed who do not meet American Heart Association criteria. Pediatrics, 1999,104(1): 10.
Burns JC, Kushner HI, Bastian JF, et al. Kawasaki disease: A brief histry, pedia tric, 2000,106(2): 27.
Akagi T, Kato H, Inoue O, Sato N. A study on the optimal dose of aspirin therapy in Kawasaki disease-clinical evaluation and arachidonic acid metabolism.Kurume Med J.1990,37(3): 203-208.
Fuiushok, Kamiye T, Nakano H, et al. High-dose intravenous gamma globulin for Kawasaki disease (in Japanese)[J]. Clin Rev, 1983,17:659.
Rowley AH, Shulman ST. Kawasaki disease.In: Behrman RE, Kliegman RM, Arvin AM, eds. Nelson textbook of pediatrics.Philadelphia: Sadnders, 2000,725-727.
Oates-Whitehead RM, Baumer JH, Haines L, Love S, Maconochie IK, Gupta A, Roman K, Dua JS, Flynn I. Intravenous immunoglobulin for the treatment of Kawasaki disease in children. Cochrane Database Syst Rev. 2003;(4): CD004000.
李永柏，易岂建，李秋，等。优选静脉免疫球蛋白剂量治疗川崎病临床与免疫学效应研究。中华儿科杂志，1999，37（2）：97。
顾承萍，傅雅珠。不同剂量丙种球蛋白治疗川崎病疗效分析。小儿急救医学，2003，10（3）：162-163。
周太光，刘春艳，胡晓。不同剂量丙种球蛋白对川崎病心血管系统的影响。小儿急救医学，2003，10（2）：111。
Kato H, Ichinose E, Kawasaki T. Myocardial infarction in Kawasaki disease: clinical analyses of 195 cases. J Pediatr, 1986,108:923-927.
Zenji N, Kihei M, Takeshi O, et al. Randomized controlled study of intravenous prednisolone and gammaglobulintreatment in 100 cases with Kawasaki disease. In: Hirohisa K, ed. Kawasaki disease. Netherland: Elsevier, 1995.328-331.
Wriht D, Newburger JW, Baker A, et al. Treatment of immune globulin-resistant Kawasaki disease with pulsed doses of corticosteroids [J]. Pediatr, 1996; 128(1): 146-149.
Fukunishi M, Kikkawa M, Hamana K, et al. Prediction of non-responsiveness to intravenous high-dose γ-globulin therapy in patients with Kawasaki disease at onset [J]. J Pediatr, 2000; 137(2): 172-176.
杜军保，张清友。川崎病治疗的若干新观点。实用儿科临床杂志，1999，37（2）：97。
王宏伟，程佩萱。小儿风湿病诊治进展。中国实用儿科杂志，2002，17（5）：267。