特发性血小板减少性紫癜262例

目的分析儿童特发性血小板减少性紫癜(ITP)发病规律、治疗及预后因素。方法1.总结北京儿童医院血液病中心2002~2003年住院ITP病例。分型并记录性别、年龄、血小板数、出血程度、诊断和治疗结果,比较急性与慢性、难治性ITP病例资料。2.对病程达0.5年以上患儿发信随访。3.总结死亡病例资料。结果ITP患儿262例,其中急性ITP 235例(89.7%),慢性24例(9.2%),难治性3例(1.15%)。1.急性组<6岁高发(81%),常有明显诱因,治疗恢复快。2.慢性、难治性组>6岁较多(33%),无明显诱因,治疗有效率低,恢复时间长。3.发病多为血小板重度、极重度减少(83.5%),临床多为轻度出血(70.2%)。4.随访66例:急性ITP18例6个月后确诊为慢性ITP;2例随访时确诊为再生障碍性贫血;3例(10%)慢性/难治性ITP病程6~12个月自行缓解。5.死亡1例,为慢性型,长期服用激素造成肾上腺皮质危象死亡。结论1.儿童ITP以急性为主,恢复快,预后好。2.部分慢性、难治性ITP患儿有自愈可能。3.切忌过度治疗。4.疗效不佳者需再评估。     

儿童免疫性血小板减少症360例临床特征分析

目的按目前国内外免疫性血小板减少症(ITP)诊疗建议分析ITP患儿各型临床特征。方法回顾性分析本院2008年1月1日-2013年12月30日住院治疗的360例资料完整并有随访记录的ITP患儿临床资料,统计各型临床特征等相关数据。结果 (1)360例ITP患儿中新诊断ITP、持续性ITP及慢性ITP分别占60.8%、26.9%、12.3%,男女比例为1.6∶1,各型男女比例无显著性差异(P>0.05);发病年龄以婴幼儿为主,慢性型以学龄儿为主(56.8%),各型年龄分布有显著性差异(P<0.05);发病季节春夏为主,各型无显著性差异(P>0.05)。(2)各型有诱因者分别为64.5%、53.6%、34.1%,慢性ITP诱因不明显(P<0.05);各型初诊病程分别为(11.49±7.50)d、(41.55±10.55)d、(402.83±28.26)d,慢性ITP病程较其他两型长(P<0.05);各型出血以轻度为主,重度出血有差异(P<0.05)。结论 (1)儿童ITP新诊断型多见,慢性型少见;发病年龄婴幼儿为主,慢性ITP以年长儿为主;多数患儿起病前有诱因,尤其新诊断ITP及持续性ITP。(2)各型ITP患儿临床以轻度出血为主,重度出血少见,但慢性ITP重度出血明显。     

小儿原发性血小板减少性紫癜64例临床分析

原发性血小板减少性紫癜(ITP),是儿童时期常见的出血性疾病,常规的治疗是首选皮质激素,但激素用药时间长,副作用大,80年代开始使用大剂量静脉注射免疫球蛋白(IVIG)治疗ITP取得较好疗效,近年来我科采用小剂量静脉注射免疫球蛋白配合激素治疗小儿ITP也取得一定疗效,现报告如下:资料与方法1一般资料我科2002~2005年收治ITP64例,均符合原发性血小板减少性紫癜诊断标准[1],其中男30例,女34例;急性型55例,慢性型9例。发病年龄2月~14岁,平均(64.16±40.09)月。将64例患儿随机分为两组:小剂量IVIG加激素(简称治疗组,以下同)33例;其中急性型2…     

持续性和慢性免疫性血小板减少症的临床研究

目的探讨持续性免疫性血小板减少症(persistent ITP,pITP)和慢性免疫性血小板减少症(chronic ITP,cITP)患儿临床特征和疗效。方法对2002年12月-2009年12月间于我院诊断和治疗的pITP和cITP患儿103例的临床资料进行回顾性分析。结果 (1)103例患儿中96.1%年龄分布在学龄前期及以后;男女性别比例为1.24∶1。(2)73.8%有诱因,其中上呼吸道感染占89.5%;患儿病原血清学IgM阳性率为45.0%,混合感染率为36.1%。(3)有黏膜出血者占61.2%,失血性贫血者占25.2%,以轻度贫血为主。就诊时血小板计数<25×109/L者占71.9%。黏膜出血组与非黏膜出血组的血小板计数比较差异无显著性;而血小板减少与贫血程度间比较差异亦无显著性(P>0.05)。(4)遵医嘱维持用药治疗者占59.2%,该组患儿黏膜出血的发生率(55.7%)低于维持间断治疗组(69.0%);而维持用药组患儿失血性贫血发生率(8.2%)显著低于间断治疗组(50.0%),差异有极显著性(χ2=23.034,P<0.001)。(5)单用激素组(79.7%)、激素+IVIG(静脉注射用人免疫球蛋白)组(78.6%)治疗有效率高于激素+VCR(长春新碱)组(40.0%);激素+IVIG与激素+VCR组间疗效差异有显著性(χ2=4.441,P=0.035);单用激素组与激素+VCR组间疗效差异有显著性(χ2=9.772,P=0.002)。结论 (1)pITP和cITP患儿主要见于学龄前期及学龄期儿童,性别差异不明显。(2)上呼吸道感染是儿童pITP和cITP发生的主要诱因,病毒感染在ITP病情反复中起着一定的作用。(3)pITP和cITP患儿出血程度较轻,血小板减少以重型、极重型居多;而出血表现、贫血程度与血小板减少间无相关性。(4)维持用药可减轻pITP和cITP患儿出血症状,降低失血性贫血发生率。(5)单用激素组、激素+IVIG组的治疗有效率显著高于激素+VCR组。     

婴儿特发性血小板减少性紫癜巨细胞病毒感染临床观察

目的探讨巨细胞病毒(CMV)感染对婴儿特发性血小板减少性紫癜(ITP)发病的影响。方法对2004年6月-2009年12月我院收治的178例婴儿ITP患儿,检测血清CMV IgM或DNA,根据其阳性与否分为观察组和对照组,对发病年龄、入院前病程、发病诱因、出血程度、实验室检查及转归进行回顾性分析。结果观察组81例,中位年龄3个月;对照组97例,中位年龄6个月;两组前驱感染率分别为37%和42%,差异无显著性(P>0.05),前驱感染主要为上呼吸道感染。两组分别有72%和75%在发病前有预防接种史,两组差异无显著性(P>0.05)。两组临床出血程度比较差异无显著性(P>0.05)。入院时两组均有半数以上血小板计数<10.00×109/L,其均值分别为12.28×109/L和11.67×109/L,两者比较差异无显著性(P>0.05)。两组骨髓涂片巨核细胞数均正常或增多,并有成熟障碍和血小板生成不良;两组分别有86%(31/36)和81%(26/32)存在免疫球蛋白异常,差异无显著性(P>0.05)。经泼尼松和(或)静脉注射用丙种球蛋白治疗后,观察组有75例(93%)、对照组有93例(96%)血小板于2周内恢复正常,两组比较差异无显著性(P>0.05)。结论婴儿ITP中CMV感染在小月龄患儿中常见,可能是血小板减少性紫癜的诱发因素之一。CMV感染的ITP患儿其临床经过与非CMV感染者无明显差异。婴儿ITP无论有无CMV感染,均对肾上腺皮质激素或大剂量丙种球蛋白治疗有良好反应,但如何减少过度治疗有待于进一步研究。     

儿童难治性特发性血小板减少性紫癜的治疗进展

特发性血小板减少性紫癜(ITP)是儿童最常见的出血性疾病,发病率高,对儿童健康造成很大威胁.传统治疗以糖皮质激素、静脉免疫球蛋白、免疫抑制剂等非特异性手段为主,但约有1/3患儿对治疗无效,成为难治性ITP.近年来随着对ITP自身免疫发病机制的深人探讨,许多定向免疫于预措施进入临床研究,该文就儿童难治性ITP分级治疗原则、传统疗法、定向免疫干预治疗等方面的进展作一综述.     

NK细胞在儿童免疫性血小板减少症发病和治疗中的意义

目的：探讨自然杀伤细胞（NK细胞）在儿童免疫性血小板减少症（ITP）的发病和治疗中的意义。方法采用流式细胞仪分别检测62例新诊断ITP患儿、43例持续性ITP患儿、21例慢性ITP患儿和51例对照组儿童外周血NK细胞百分比;并观察单独使用标准剂量静脉注射用人免疫球蛋白（IVIG）对NK细胞百分比正常及减少的新诊断的ITP患儿的疗效。结果新诊断ITP患儿、持续性ITP、慢性ITP患儿NK细胞百分比均较正常对照组儿童显著降低（P＜0.05）,但三组ITP患儿NK细胞百分比差异无显著性（P ！0．05）;NK细胞百分比正常的新诊断ITP患儿单独使用IVIG有效率为92．86％（26/28）,NK细胞百分比降低的新诊断ITP患儿单独使用IVIG有效率仅为14．70％（5/34）。结论 NK细胞表达变化与ITP发病存在一定关系,同时NK细胞百分比正常的新诊断ITP患儿可首选IVIG治疗。     

儿童慢性特发性血小板减少性紫癜的临床转归

目的总结儿童慢性特发性血小板减少性紫癜(cITP)的临床经过及主要临床特点,以期对儿童cITP的应对策略有所帮助。方法回顾性分析58例儿童慢性ITP的自发缓解情况与主要临床特点。结果(1)58例cITP患儿中,33例(56.9%)获得自发缓解,中位缓解时间1年;预计累计自发缓解率在诊断后6~12个月、2、3和4年内分别为40%,47%,47%,55%,无死于出血者。(2)儿童cITP的起病年龄、性别、前驱感染史、病初PLT、抗核抗体及病初对药物治疗的反应均与疾病转归无关(P≥0.05)。(3)急、慢性ITP患儿的最低血小板计数分布基本相同(P=0.156),轻、中度出血的分布没有差别(P=0.329),但cITP重度出血多于急性ITP,分别为13.8%和1.9%。结论儿童cITP中的部分病例也呈现自限性经过,起病时的临床表现及早期药物治疗对疾病转归无明显影响。     

婴幼儿特发性血小板减少性紫癜401例临床特点

目的 分析婴幼儿特发性血小板减少性紫癜(ITP)的临床特点,并比较婴儿与幼儿ITP的疗效.方法 收集401例婴幼儿ITP,均给予激素冲击治疗和静脉滴注免疫球蛋白治疗,疗效判定依据血小板计数的高低和出血症状的改善分为完全缓解、有效、无效.401例婴幼儿ITP按年龄分为婴儿组(≤1岁)和幼儿组(>1～3岁),按病程分为急性(病程≤6个月)和慢性(病程>6个月),对其临床资料进行回顾性分析,应用SPSS 12.0软件进行统计分析.结果 1.婴儿组与幼儿组均为男童比例高,但2组间性别比较差异无统计学意义(χ2=0.682,P>0.05).2.婴儿组入院时血小板中位计数低于幼儿组,差异有统计学意义(Z=2.668,P<0.05).3.婴儿组骨髓巨核细胞增高比例低于幼儿组,差异有统计学意义(χ2=16.322,P<0.001);婴儿组产板巨核细胞中位数低于幼儿组,差异有统计学意义(Z=2.065,P<0.05).4.婴儿组经治疗后血小板计数达到或超过100×109 L-1的时间短于幼儿组,差异有统计学意义(Z=3.542,P<0.001).5.急性患儿入院时血小板中位计数低于慢性患儿,差异有统计学意义(Z=2.100,P<0.05).6.输注血小板患儿的住院时间与未输注血小板患儿相比,差异无统计学意义(Z=1.385,P>0.05).结论 婴幼儿ITP中,男童比例高,大部分无明显诱因,以皮肤黏膜出血为主;婴儿入院时血小板中位计数较低,血小板上升至正常的时间较短,对治疗反应较幼儿好,幼儿急性ITP可以变为慢性;输注血小板并不能缩短ITP患儿的住院时间.     

儿童免疫性血小板减少症与幽门螺杆菌感染

目的探讨幽门螺杆菌(Hp)感染与儿童免疫性血小板减少症(ITP)发病的关系。方法应用酶联免疫法检测54例ITP患儿粪便Hp抗原,观察Hp抗原阳性与阴性患儿的临床表现、血小板减少程度及对治疗的反应。结果 54例患儿,Hp阳性率19%(10例),不同发病年龄患儿阳性率差异无显著性。47例急性ITP患儿中Hp阳性9例(19%),治疗后血小板恢复正常平均需7.3 d;38例Hp阴性患儿血小板恢复正常平均需5.1 d,两组间差异无显著性(P>0.05)。慢性ITP患儿Hp阳性率14%,与急性ITP差异无显著性。结论未发现ITP患儿Hp感染率高于一般人群;Hp阳性率与患儿年龄无明显相关;Hp感染不影响ITP患儿对治疗的反应。     

大剂量短疗程泼尼松治疗儿童急性免疫性血小板减少症的疗效观察

目的观察大剂量短疗程泼尼松(Pred)疗法对儿童急性免疫性血小板减少症(ITP)的疗效。方法 162例ITP患儿根据治疗方法不同随机分为大剂量静脉丙种球蛋白+甲基泼尼松龙组(IVIG+MP)、静脉丙种球蛋白组(IVIG)、甲基泼尼松龙组(MP)与Pred组。IVIG+MP组41例,采用IVIG(1g/kg,共1次)+MP[10 mg/(kg.d),每3天减半量,共9 d]冲击治疗,继之口服Pred[1.5～2.0 mg/(kg.d)],并逐渐减量维持治疗;IVIG组39例,采用丙种球蛋白(1 g/kg,共1次)冲击治疗,继之口服Pred[1.5～2.0 mg/(kg.d)]并逐渐减量维持治疗;MP组40例,采用MP[10 mg/(kg.d),每3天减半量,共9 d]冲击治疗,继之口服Pred[1.5～2.0 mg/(kg.d)]并逐渐减量维持治疗;Pred组42例,采用口服Pred[4 mg/(kg.d),共4 d]治疗后停药,无减量维持治疗。比较各组治疗前后血小板数、治疗有效率、不良反应发生率及药费支出。结果各治疗组治疗前后血小板数及治疗有效率差异无显著性,IVIG+MP组、IVIG组、MP组治疗不良反应发生率及药费支出均高于Pred组。结论大剂量短疗程Pred疗法治疗儿童急性ITP能有效提升血小板计数,有效率与IVIG及MP冲击治疗相比差异无显著性,不良反应少,花费低。     

Corticosteroid prophylaxis for neurologic complications of intravenous immunoglobulin G therapy in childhood immune thrombocytopenic purpura

To assess in a retrospective analysis if there is evidence suggesting corticosteroids can prevent the neurologic complications of intravenous immunoglobulin (IVIG) therapy in children with immune thrombocytopenic purpura (ITP). From March 1985 to September 1997, 112 children received IVIG (1 g/kg/day for one or two dosages) for the treatment of ITP. During the years 1990 to 1997, 23 children nonrandomly received a short course of prednisone (2 mg/kg/day during and for 3 days after the completion of IVIG therapy) as a prophylaxis against the neurologic complications of IVIG therapy. The authors analyzed the data of all 112 children and compared the incidence of neurologic complications in those who received prednisone prophylaxis with those who did not. The severity of the complications was assessed as follows: grade 1, headache only; grade 2, headache plus vomiting; grade 3, headache, vomiting, and fever; grade 4, headache, vomiting, fever, and meningeal signs (aseptic meningitis). Of the 23 children who received prednisone prophylaxis, 2 (8.7%) experienced headache and vomiting after the completion of prednisone prophylaxis. Of the 89 children without prednisone prophylaxis, 27 (30.3%) experienced neurologic symptoms of varying severity, including one patient with aseptic meningitis proven by examination of the spinal fluid. Twelve of these patients needed additional hospital care for the complications. Children receiving prednisone had a 78% lower risk of neurologic complications (OR = 0.22; CI = 0.05-0.90; P = 0.036). This retrospective study shows a short course of prednisone therapy, given during and until 3 days after the completion of IVIG infusion, is likely to decrease the incidence and severity of neurologic complications of IVIG in children with ITP.     

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目的了解单次小剂量(0.4g/kg)静脉输注免疫球蛋白(IVIG)提升初发免疫性血小板减少性紫癜(ITP)患儿血小板至安全范围(≥30×109/L)的作用。方法研究对象为北京大学第一医院儿科2008-04-01—2011-04-01收治初发ITP患儿62例,其中2008-04-01—2009-10-01收治的30例为激素组,初始接受常规剂量醋酸泼尼松治疗;2009-10-02—2011-04-01就诊的32例为IVIG组,初始接受0.4g/(kg·d)IVIG治疗1～5d,每天复查血常规,血小板升至安全范围则规范停用。比较两组治疗第1、3、5天时血小板升至安全范围比例及长期随访结果。结果治疗前,激素组和IVIG组血小板中位值分别是10×109/L和6×109/L。治疗1d后两组血小板升至安全范围的比例分别是3.33%和43.75%,差异有统计学意义(P<0.01)。随访7～42个月后激素组和IVIG组分别有3.45%和3.23%血小板未升至正常(≥100×109/L)。所有患儿均无颅内出血发生及死亡。结论单次小剂量IVIG可使近半数初治ITP患儿血小板升至≥30×109/L相对安全范围,明显高于常规剂量醋酸泼尼松疗效。     

The clinical course of immune thrombocytopenic purpura in children who did not receive intravenous immunoglobulins or sustained prednisone treatment

OBJECTIVE: To demonstrate the result of watchful waiting without specific therapy in unselected children with acute immune thrombocytopenic purpura (ITP). STUDY DESIGN: Between May 1992 and October 1999, 55 consecutive children (aged 2 months to 16 years; 28 boys and 27 girls) with acute ITP did not receive intravenously administered immune globulin G (IVIG) or sustained prednisone treatment. Patients with extensive mucosal bleeding were given prednisone, 2 mg/kg/d, for 3 days. RESULTS: In 37 of 55 patients the initial platelet count was <10,000/microL. Ten of these patients had active mucosal bleeding. Five additional patients with bleeding had platelet counts between 10,000 and 20,000/microL. Four patients were given a 3-day course of prednisone. Chronic ITP occurred in 7 (13%) of the patients; 29 patients achieved remission within 6 weeks, and 19 patients, between 6 weeks and 6 months. No life-threatening bleeding occurred, and no patient died. CONCLUSION: Most children with severe thrombocytopenia do not have active mucosal bleeding. This management approach, which did not administer specific therapy, avoided side effects, reduced cost, and was effective.     

Clinical responses of patients with Kawasaki disease to different brands of intravenous immunoglobulin

OBJECTIVE: To determine whether different brands of intravenous immunoglobulin (IVIG) administered to children with Kawasaki disease (KD) result in different outcomes. STUDY DESIGN: We analyzed children with KD and divided them into 4 groups according to the brand of IVIG. A coronary artery abnormality (CAA) was defined as having a lumen diameter (inner border to inner border) of > or =3 mm in KD cases <5 years old and > or =4 mm in cases > or =5 years old, and giant aneurysm was defined as a lumen diameter > or =8 mm. Patients were considered nonresponsive to IVIG therapy if fever persisted longer than 2 days after completion of treatment and needed retreatment with IVIG. RESULTS: We collected 437 cases, 29 (6.6%) were nonresponsive, 17 (3.9%) had CAA at convalescence, and 3 (0.7%) had giant aneurysm, 2 of whom had development of myocardial infarcts. Patients receiving Brand C IVIG, prepared with beta-propiolactone, had higher rates (10%, 9/93, P = .01) of CAA at convalescence and nonresponsiveness (13%, 12/93, P = .001); giant aneurysm occurred in 3/93 (3%) receiving Brand C IVIG and in 0/344 who received the other 3 brands (P = .008). CONCLUSIONS: IVIG, prepared with beta-propiolactone, was most significantly associated with nonresponsiveness, CAA at convalescence, and giant aneurysm. Physicians should be cautious when using IVIG prepared with beta-propiolactone or enzyme digestion to treat KD.     

A cost-utility analysis of treatment for acute childhood idiopathic thrombocytopenic purpura (ITP)

BACKGROUND: The primary objective in the treatment of acute pediatric idiopathic thrombocytopenic purpura (ITP) is to rapidly increase the platelet count. METHODS: We built a decision analytic model to evaluate the cost-utility of four commonly used treatment strategies: intravenous immunoglobulin G (IVIG) 0.8 g/kg, anti-D 75 mcg/kg, methylprednisolone (30 mg/kg for 3 days), and prednisone (4 mg/kg/day for 4 days). In our baseline model, all children were hospitalized upon presentation, and discharged once the platelet count reached > or =20,000. We performed a literature search to estimate time to platelet count > or =20,000 for each strategy, as well as the probability of side effects. We obtained cost data and quality of life measures from institutional and published data sources. RESULTS: Total cost of one-time treatment for a 20 kg child was US dollars 786 with prednisone, US dollars 1,346 with methylprednisolone, US dollars 2,035 with anti-D, and US dollars 2,492 with IVIG. The strategies of IVIG and methylprednisolone were less effective and more expensive than anti-D and prednisone, respectively. Although anti-D caused the most rapid rise in platelet counts, the incremental cost-utility ratio (costs incurred by using anti-D instead of prednisone divided by health benefit of using anti-D instead of prednisone) was US dollars 7,616 per day of severe thrombocytopenia avoided, primarily due to the much higher medication cost of anti-D. Utilizing an outpatient model, the cost difference between anti-D and prednisone was even more striking. CONCLUSIONS: The clinical benefit of anti-D is offset by a substantial cost increase. Although often overlooked in favor of newer agents, a brief course of high-dose prednisone is an inexpensive and effective treatment for acute ITP.     

Neutropenia following IVIG therapy in pediatric patients with immune-mediated thrombocytopenia

It has been observed that some children with immune-mediated thrombocytopenia (ITP) who are treated with intravenous immunoglobulin (IVIG) experience a decline in their absolute neutrophil count (ANC). The aim of this study was to investigate the incidence of neutropenia following IVIG therapy in a large cohort of children with ITP. This retrospective comparative cohort study determined the incidence of neutropenia in 104 patients (110 treatment courses) admitted for ITP to the Children's Hospital of Philadelphia from January 2000 to October 2003. Post-treatment ANCs were compared between patients who received IVIG and patients who received anti-D immunoglobulin. The incidence of neutropenia in each group was analyzed using the Fisher exact test. Pretreatment ANCs were not significantly different between the two treatment groups (P = 0.72). Neutropenia (ANC < 1,500/microL), developed during 18 of 64 (28%) treatment courses with IVIG, compared with 0 of 46 (0%) treatment courses with anti-D immunoglobulin (P < 0.001). This study suggests that IVIG may cause neutropenia commonly in children with ITP. While this is likely to be a transient condition, its recognition may affect clinical decisions such as the need for a bone marrow examination.     

静脉注射不同剂量丙种球蛋白治疗川崎病的临床研究

目的 评价静脉注射丙种球蛋白（intravenous immune globulin,IVIG）1g／kg单次静脉注射治疗川崎病（Kawasaki disease,KD）的临床效果。方法 242例KD患儿随机分为IVIG1s／kg组与IVIG 2g／kg组,对两种治疗方法的疗效进行前瞻性对比研究。分别采用IVIG 1g／kg和2s／kg单次静脉注射,观察患儿总热程、退热时间、黏膜充血、手足肿胀和颈淋巴结肿大消退时间,监测外周血白细胞计数（white blood cells count,WBC）、血小板计数（platelet count,PLT）、血清丙种球蛋白（immunoglobulin,Ig）、C反应蛋白（Creacting protein,CRP）、血沉（erythrocyte sedimentation rate,ESR）、心电图（electrocardiogram,ECG）和冠状动脉病变（coronary artery lesion,CAL）恢复情况,并对治疗前后组内结果、治疗后组间结果进行比较。结果 IVIG 1g／kg组平均热程为10．6d,WBC、PLT、CRP、ESR及ECG异常率与治疗前比较显著降低（P〈0．001）,IVIG1g／kg组与IVIG2g／kg组比较差异无统计学意义（P〉0．05）。WIG1g／kg组CAL发生率为29．5％（36／122）,随访1年有87．5％的CAL恢复正常,12．5％未能恢复正常,其中9．4％为IVIG耐药病例;IVIG2g／kg组CAL发生率为24．2％（29／120例）,随访1年有89．3％的CAL恢复正常,10．7％未能恢复正常,均为IVIG耐药病例,两组比较,差异亦无统计学意义（P〉0．05）。结论 IVIG1g／kg单次静脉注射治疗KD,可有效缓解临床症状,减低CAL发生率,减轻心血管系统损害,与IVIG2g／kg比较具有同样的近期和远期治疗效果。     

川崎病合并冠状动脉瘤63例临床分析

目的分析川崎病(KD)合并冠状动脉(以下简称冠脉)瘤患儿的临床特点。方法对首都医科大学附属北京儿童医院2000—2007年收治的63例超声心动图诊断为冠脉瘤的KD患儿临床资料、实验室检查、超声及心电图检查结果、治疗情况及随诊资料进行回顾性分析。结果(1)冠脉瘤患儿男性明显多于女性,男∶女为5.3∶1;冠脉巨大瘤男女比例为8.3∶1;<1岁患儿多发,占28.6%。(2)本组患儿中不完全KD、静脉注射丙种球蛋白(IVIG)抵抗以及KD复发的发生率均较高,分别为36.5%、30.2%和7.9%;急性期57例(90.5%)患儿使用IVIG冲击治疗,3例未用,3例使用情况不详;36例(57.1%)患儿发病至丙种球蛋白应用的时间间隔大于10 d。(3)超声检查发现小冠脉瘤患儿7例,中等冠脉瘤19例,巨大瘤37例,左冠脉受累者占76.2%,其中58.3%发生在前降支;右冠脉受累者达87.3%,其中47.3%发生在右冠Ⅱ段;双侧冠脉同时受累者占63.5%。(4)随诊发现71.4%冠脉瘤呈现回缩趋势,45.2%的受累分支冠脉瘤消退,平均消退时间为(2.1±1.5)年。结论对于男性、发病年龄<1岁、不完全KD、发生IVIG抵抗、复发患儿及应用IVIG治疗较晚患儿要警惕冠脉瘤的发生;左冠前降支及右冠脉瘤样病变最多见,多数冠脉瘤在恢复期发生回缩。