川崎病诊治专家共识

111例川崎病临床分析【摘要】目的：分析111例川崎病患者的临床表现与特征。

方法：2012年12月到2021年10月，选取111例川崎病患者进行研究，其中完全性川崎病（CKD）患者55例，不完全性川崎病（IKD）患者56例，汇总患者的各项资料，并进行统计分析。

结果：CKD患儿的性别、年龄与IKD患儿比较无差异（P＞0.05）。

CKD患儿的淋巴结肿大、皮疹、眼结膜充血、草莓舌、四肢变化、唇充血皲裂发生率较IKD患儿更大（P＜0.05）。

CKD患儿的C反应蛋白（CRP）、谷丙转氨酶（ALT）水平较IKD患儿更高（P＜0.05），CKD患儿的白蛋白（ALB）水平较IKD患儿更低（P＜0.05），CKD患儿的住院治疗时间IKD患儿更短（P＜0.05）。

CKD患儿的CRP＞30mg/L百分比较IKD患儿更大（P＜0.05）。

CKD患儿的冠状动脉分级百分比与IKD患儿比较无差异（P＞0.05）。

结论：CKD、IKD患儿的症状表现、CRP、ALT、ALB水平存在差异，临床需充分认识这一点，积极明确两种疾病的差异涉及范围，积极辅助患者完成可明确差异的检查，辅助临床早期鉴别疾病，才能尽快进行针对性治疗。

关键词：完全性川崎病；不完全性川崎病；症状表现；C反应蛋白；白蛋白；谷丙转氨酶川崎病（Kawasaki Disease，KD）又称皮肤黏膜淋巴结综合征，5岁以内婴幼儿是高发对象[1]。

该病是一种全身血管炎性疾病，突出的病理特征是全身非特异性血管炎以及冠状动脉损害，可引起全身各系统的表现。

本文主要分析该病临床特点，报道如下。

1·资料与方法1.1临床资料选取2020年9月到2021年12月在我院住院的111例川崎病患者进行研究，其中完全性川崎病患者55例，不完全性川崎病患者56例。

纳入标准：（1）患者均满足《川崎病诊断和急性期治疗专家共识》中川崎病的诊断标准[3]，分为完全性川崎病（CKD）和不完全性川崎病（IKD）两种类型。

川崎病的诊断标准引言川崎病，又称混合性血管炎，是一种儿童常见的全身性疾病，起病急骤，可累及多个器官。

该病的早期诊断对预防并发症的发生至关重要。

本文将介绍川崎病的诊断标准和相关评估参数。

诊断标准川崎病的诊断标准通常基于国际儿科学会议上提出的川崎病诊断标准（The 5th Revised Indian and American Criteria）。

根据该标准，川崎病的诊断必须符合以下 5 个主要标准中的至少 4 个：1.持续发热：持续 5 天以上的高热（≥ 39℃），在使用抗生素治疗后热退或热型不符合抗生素反应。

2.蜜糖舌炎：舌黏膜出现鲜红的草莓状疹，伴随舌苔脱落。

3.双手掌红肿：双手掌红肿，可伴指甲剥脱；4.双脚足底红肿：双足足底红肿，足底可出现鲜红的草莓舌状疹；5.蜂窝组织炎：淋巴结炎性肿大，主要位于颈部区域。

在诊断川崎病时，如果孩子符合上述 4 个主要标准中的 3 个，但同时具备以下4 个附加标准中的至少 2 个，则也可被诊断为川崎病：1.口腔黏膜改变：咽炎、嘴唇炎、舌下疹等。

2.枕部淋巴结肿大：一侧或双侧颈部淋巴结肿大，直径大于 1.5 厘米。

3.皮疹：包括四肢上非特异性皮疹、躯干红斑或斑块，或是紫疹。

4.结膜炎：双侧结膜充血、结膜水肿、结膜红斑。

评估参数除了诊断标准，还有一些评估参数可以辅助川崎病的诊断和治疗：1.血液检查：包括白细胞计数、血清C反应蛋白（CRP）、血小板计数等。

这些指标在川崎病发病早期往往异常升高。

2.心肌炎标志物：心肌肌钙蛋白Ⅰ和肌钙蛋白T是诊断心肌炎的重要指标，其临床表现可能与川崎病相关。

3.心电图检查：心电图检查有助于评估心脏病变，例如冠状动脉或冠状动脉瘤形成。

4.足背动脉超声：足背动脉超声检查可用于早期评估年幼患者是否存在冠状动脉损害。

5.心脏超声：心脏超声检查是评估川崎病合并心血管并发症的首选方法，可以观察冠状动脉瘤、心功能降低等。

结论川崎病是一种全身性疾病，早期诊断对预防并发症的发生至关重要。

川崎病专题讨论会纪要《中华儿科杂志》编辑委员会中华医学会儿科学分会心血管学组中华医学会儿科学分会免疫学组川崎病(Kawasaki diseases，KD)是一种主要发生在5岁以下婴幼儿的急性发热出疹性疾病，属急性自限性血管炎综合征，其发病率有逐年增高趋势，在发达国家或地区，KD所致的冠状动脉病变(coronary artery lesions，CAL)已取代风湿热成为小儿最常见的后天性心脏病，并且可能成为成年后缺血性心脏病的危险因素之一。

KD病因及免疫发病机制仍未完全清楚，大量流行病学及临床观察提示KD可能是感染因素所致的急性免疫调节紊乱，遗传因素亦与KD发病相关。

已报道多种细菌、病毒、支原体及其代谢产物(如链球菌和葡萄球菌超抗原)等与KD发病有关，但目前仍未检测到致KD的单一病原微生物。

感染导致免疫活性细胞(如T细胞、单核/巨噬细胞)异常活化，所产生的细胞因子可能参与血管内皮损伤及干扰自身免疫耐受。

急性自限性血管炎症反应易导致冠状动脉病变，KD治疗的重点是尽快阻止促炎症细胞因子的产生和清除，降低炎症反应，预防和减轻冠状动脉病变。

《中华儿科杂志》编委会分别于2006年12月5日和2007年3月28日组织了川崎病专题讨论会，川崎病超声心动图诊断专题讨论会，儿科心血管和免疫的专家就KD的临床热点问题如不完全型(不典型)KD的诊断、IVIG无反应型(不敏感)KD处理等问题进行了十分热烈的讨论，现归纳如下。

一、KD诊断标准因缺乏特异诊断方法，KD的诊断主要靠临床表现，某些实验室检查可以协助临床诊断。

KD可分为典型和不典型两类。

1．典型KD：2002年日本川崎病研究组提出典型KD诊断标准，经第七届国际川崎病大会讨论通过后发表于Pediatrics International(2005)。

美国心脏病协会(AHA)亦提出典型KD诊断标准并发表于Pediatrics(2004)和Circulation(2004)上。

GuidelinesRevision of diagnostic guidelines for Kawasaki disease(6th revised edition)Purpose and background of the revisionThe diagnostic guidelines for Kawasaki disease(KD)were last revised in20021(5th revision).Major points of revision included:(i)the definition of fever was defined as fever per-sisting5days or more(inclusive of cases in which the fever had subsided before the fifth day in response to therapy)and(ii)to explicitly state that incomplete KD cases can have coro-nary artery lesions.After the5th revised edition of the diagnostic guidelines for KD was published,the proportion of patients receiving early treatment increased and the incidence of coronary artery lesions decreased nationwide.On the other hand,the number of incomplete KD cases increased yearly from10%to the cur-rent level,which is greater than20%of all KD patients.In recent years,a standard method for expressing the coro-nary artery internal diameter of Japanese children was estab-lished2and allowed us to define coronary artery internal dimensions in terms of standard deviations from the mean,or Z scores).Incorporation of Z scores to facilitate the diagnosis of incomplete KD was a motivating factor for this revision.In the5th edition,the section titled“Other significant symptoms or findings”was not changed;therefore,the description from the4th edition lasted more than 30 years and was due for an update. In 2017, we consulted with the Japan Kawasaki Disease Society Steering Committee Members regarding the necessity for a revi-sion of the 5th revised edition, and 75% of the committee mem-bers agreed torevise. The Japan Kawasaki Disease Research Center and study group for vasculitis funded by the Ministry of Health, Labor and Welfare also agreed to the revision.In this revision, the writing group members conducted dis-cussions from 2018 to 2019. The original draft was presented to the 38th Annual Meeting of the Japanese Society of Kawa-saki Disease in Wakayama. The draft was revised again, based on the steering committee members’ suggestions, and the final draft of the 6th revision was completed. In the future it will be interesting to evaluate the impact of these revised guideli-nes on the diagnosis of KD in Japan.The previous 5th edition1 was published as an article in Japan Today in 2005, and was titled “Diagnostic Guidelines.” The recent format of the “guidelines” has changed and requires full supporting evidence; “diagnostic guidance” or “criteria with clinical findings” may be more appropriate as the title for this revision because there is not enough evidence for the diag-nosis of this disease. However, as the previous title has been familiar with most pediatricians and primary care physicians, we would prefer to use the same title with only the change of the edition number from the fifth to the sixth revised edition. Additionally, because such clinicians use these guidelines as the diagnostic criteria, it is desirable to be as concise as possi-ble and to be presented as a few brief sheets of 1 or 2 pages.As we also believe more detailed explanations are neces-sary to describe each item, including many examination findings, an additional “guidebook” will be written by the committee members for publication.The major changes of the revision are described below.Principal clinical featuresSeveral changes were made to the six principal clinical fea-tures,which have been well understood and disseminated for almost all clinicians in Japan(Tables1–3and Figure1).1.The requirement for a specific duration of fever was deleted.In Japan,more than90%of KD patients received high dose intravenous immunoglobulin(IVIG)in a single dose.Although most pediatricians or primary care physi-cians know that the classic definition of KD required a duration of fever for more than5days,the24th Nation-wide Surveillance reports that approximately9%,25%,and35%of KD patients received the first IVIG treatment on the3rd,4th,and5th days of illness,respectively,and the prevalence of coronary artery lesions(CAL s)has been lower than before. As we expect a continuous decrease in CALs, we modified the fever definition to reflect current practice.Table1Principal clinical features1.Fever.2.Bilateral bulbar conjunctival injection.3.Changes of lips and oral cavity:reddening of lips,strawberry tongue,diffuse injection of oral and pharyngeal mucosa.4.Rash(including redness at the site of Bacille Calmette-Gu e rin(BCG)inoculation).5.Changes of peripheral extremities:(Initial stage)reddening of palms andsoles,edema.(Convalescent stage)periungual desquamation.6.Non-supparative cervical lymphadenopathy.Table 2Definition of complete or incomplete KDNumber of principalclinical featuresCoronary artery abnormalities (+)Coronary artery abnormalities (À)6Complete (a)Complete (a)5Complete (a)Complete (a)4Complete (b)Incomplete (d)3Incomplete (c)Incomplete (d)Table 3 Other significant demographic, clinical, echocardio-graphic, and laboratory features1. Kawasaki disease may be suspected in the presence of fewer than four principal clinical features when the following findings are observed:•Elevation of hepatic transaminases early in the course of the disease.•Increased leukocytes in the urine sediment of an infant.•Thrombocytosis in the convalescent phase •Elevation of BNP or NT-pro BNP •Mitral valve regurgitation or pericardial effusion by echocardiography •Enlargement of the gallbladder (hydrops of gallbladder)•Hypoalbuminemia or hyponatremia2. If a KD patient manifests the following findings, the patient should be considered for admission of a critical care unit.•Hemodynamically significant myocarditis •Hypotention (shock)•Paralytic ileus •Decreased level of consciousness3. Risk scores to predict intravenous immunoglobulin resistance may be applied to guide patient management. The following features are elements of the risk scores for predicting intravenous immunoglobulin resistance.•Leukocytosis with left shift •thrombocytopenia •hypoalbuminemia •hyponatremia •hyperbilirubinemia (jaundice)•elevation of CRP •Age <1year4. Other non-specific findings which may be observed in Kawasaki Disease and should not exclude the diagnosis.•Irritability •Cardiovascular: abnormal extra heart sounds, electrocardio-gram changes, aneurysm of peripheral arteries other than coronary (axillary etc.),•Gastrointestinal: abdominal pain, vomiting, diarrhea •Hematologic: increased erythrocyte sedimentation rate, ane-mia •Dermatologic: micropustular rash, transverse grooves across the finger nails.•Respiratory: cough, rhinorrhea, retropharyngeal edema, infiltrate on chest radiograph.•Rheumatologic: pain, swelling.•Neurologic: cerebrospinal fluid pleocytosis, seizures, facial nerve palsy, paralysis of the extremities.BNP, brain natriuretic protein; KD, Kawasaki disease; NT-pro BNP, N terminal pro-brain natriuretic protein.1.Mortality in the acute phase: <0.1%.2.Recurrence rate: 3–4%; proportion of siblings’ cases, 1–2%.3.Nonsuppurative cervical lymphadenopathy (multiple hypoe-choic, enlarged nodes observed on ultrasound) is less fre-quently encountered (approximately 65%) compared with other principal clinical features during the acute phase. Non-suppurative cervical lymphadenopathy is observed in approxi-mately 90% of older children and often can be the firstclinical feature of KD with fever.To diagnose complete or incomplete KD, the exclusion of other febrile illnesses is essential.a: A patient who fulfills the criteria with five or six signs is diagnosed as complete KD.b: A patient who fulfills the criteria with four signs and coro-nary artery abnormality by echocardiography (Figure 1-h) is diag-nosed with complete KD.c: A patient who has three principal clinical features with coro-nary artery abnormality by echocardiography (Figure 1-h) and in whom other febrile illnesses have been excluded fulfills the crite-ria in “c.”d: When the patients who fulfill three or four signs in the the principle clinical features without coronary artery dilation but with some features from the list of ‘Other significant clinical fea-tures’ can be diagnosed as incomplete KD, if other diseases are ruled out.e: Incomplete KD may also be considered in the presence of only one or two principal clinical features after excluding other diagnoses.2. The clinical feature of “Polymorphous exanthema” was changed to “Rash” and now includes “redness at the site of Bacille Calmette-Gu e rin (BCG) inoculation”. Japanese pediatricians have recognized that redness at the site of BCG inoculation is a specific clinical sign that appears at the onset of KD. In the present revision, we included redness at the site of BCG inoculation as a clinical feature that counts as “rash,” even in the absence of more diffuse dermatologic changes.In particular, this sign is observed in more than 70% of the patients whose ages are from 6 to 20 months old3. When the patient do not show polymorphous exanthema but shows the redness of BCG scar and other 4 principle sign, that patient can be diagnosed as not incomplete KD, but (com-plete) KD. The impact of this change to the principal diagnostic clini-cal features will require further study and should be moni-tored.a bc de fg hFig.1(a)Bulbar conjunctival injection;(b)reddening of lips,strawberry tongue;(c)rash;(d)redness at the site of Bacille Calmette-Gu e rin (BCG)inoculation;(e)reddening of palms with edema;(f)periungual desquamation;(g)non-supparative cervical lymphadenopa-thy;(h)Echocardiographic finding of coronary artery aneurysm.3.Non-suppurative cervical lymphadenopathy(multiple hypoechoic,enlarged nodes observed by ultrasound)is lessfrequently encountered(approximately65%)than other principal clinical features during the acute phase.Nonsuppurative cervical lymphadenopathy is observed in approximately90%of older children and often can be the first clinical feature of KD with fever.This phenomenon is described as“Remark”1at the last part.4.The precise clinical definitions of complete and incom-plete KD are now clearly delineated as outlined in the appendixand are based on the number of principal clini-cal features and the presence of coronary artery abnormalities.A patient who fulfills the criteria in“a”or“b”is diagnosed as complete KD.A patient who has three prin-cipal clinical features with coronary artery abnormality by echocardiography and in whom other febrile illnesses have been excluded fulfills the criteria in“c”and is diag-nosed as incomplete KD.Patients who fulfill the criteria in“d”are also diagnosed as incomplete Kawasaki disease defined as the presence of three or four principal clinical features without coronary artery dilation but with features from the list of“Other significant clinical features”.Incomplete Kawasaki disease may also be considered in the presence of only one or two principal clinical features after very careful,sufficient observation and excluding other diagnoses.For these patients,particularly careful consideration of the differential diagnosis is essential.For reference,in the24th nationwide survey,0.7%and 5.4%of all KD patients were reported as incomplete KD with only one or two clinical features,respectively.However,the possibilities of other disease are higher than for incomplete KD,if the coronary artery is not involved and the principal signs are limited to less than two.Evaluation of the coronary arteries using Z scoresThe revised guidelines recommend the use of Z scores for defining coronary artery dilation.When Z-score of internal coronary artery diameter≥2.5SD units,it is defined as coro-nary artery dilation.However,in case that the examiner has a difficulty to use Z score, conventional criteria using traditional measurements of inner diameter≥3mm(<5years old)or≥4mm(≥5years old)can be used for the diagnosis of coronary artery dilation.While Z scores are a more quantitative assess-ment,we realize that they have not been adopted by all centers in Japan.This change of definition may affect the incidence of coronary artery dilation,especially transient dilation and small aneurysm.Assessment of the impact of this change will there-fore be important in future epidemiologic surveys. Other significant demographic,clinical,echocardiographic,and laboratory featuresIn this section,we substantially revised the description of specific clinical features that can be associated with KD.1.Seven clinical features are described that may be helpful in the recognition of incomplete KD cases.We hope that futureclinical research will provide more accurate diag-nostic tools including the optimum cut-off values for these variables.Although it is expected that the quantitative cut-off values of all findings in these items will be deter-mined,there have been very few Japanese studies of suffi-cient quality.In the future,an algorithm using such cut-off values is expected to be constructed.2.Clinical findings that warrant referral to a tertiary medical center with experience in treating critically ill pediatric patientsare outlined.3.Risk scores to predict intravenous immunoglobulin resis-tance may be applied to guide patient management.We definedseven features that are elements of risk scores pre-dicting intravenous immunoglobulin resistance.These fea-tures may be useful in risk stratification of patients.4.Other non-specific findings that may be observed in KD and should not exclude the diagnosis have been added.Theseeight features may support the diagnosis of KD.This summary describes the major changes to the6th revised edition of the KD diagnostic guidelines.We hope that this revision will lead to standardization of the definition of incomplete KD and diagnostic approaches,and will further reduce number of coronary artery lesions suffered by KD patients.。

2022年ACR/VF川崎病管理指南（全文）川崎病（KD）又称皮肤黏膜淋巴结综合征，1967年由日本川崎富作医生首次报道。

2022年4月，美国风湿病学会（ACR ）和血管炎基金会（VF）发布了川崎病（KD ）的管理指南，包括诊断、治疗和管理，并更侧重于为临床诊疗过程中遇到的问题提供治疗建议。

本次2022年ACR年会上，美国华盛顿特区国家儿童医院风湿科主任Sangeeta Sule 教授以02021 ACR/VF Kawasaki Disease GUidelineS〃为题,分享ACR与VF共同制定的2021年版川崎盛KD ）指南1, Karen James 博士以n CIinicaI Scenarios: Practical application of the 2021 American College of Rheumatology/ Vasculitis Foundation Kawasaki Disease Guidelines”为题，结合临床病例探讨了该指南的实际应用场景2OKD的诊断KD的诊断主要依靠临床表现并结合实验室检查，并排除其他疾病。

KD 包括完全性KD和不完全性KD两种类型，急性KD定义为KD 发热初期。

完全性KD不明原因的发热≥5d ,且具有以下5项中至少4项主要临床特征：1 ）非渗出性双眼球结膜充血2 ）口唇及口腔变化：口唇干红、皴裂，草莓舌，口咽部黏膜弥漫性充血3）四肢末梢改变：急性期手足发红、肿胀，恢复期甲周脱皮4）皮疹，常见弥漫性斑丘疹、猩红热样或多形红斑样皮疹，还包括单独出现的卡疤红肿5 ）非化脓性颈部淋巴结肿大（至少1个淋巴结直径＞1.5Cm ）,通常为单侧不完全性KD不明原因的发热N 5d ,但上述主要临床特征不足4项的患儿按图1 流程评估。

诊断性影像学1 ）对于疑似不完全性KD 和发热的儿童，强烈建议进行冠状动脉超声 心动图检查。

与典型KD 患者相比，不完全性KD 患者的冠状动脉病变风险增加。

川崎病治疗方案川崎病，也被称为Mucocutaneous Lymph Node Syndrome（黏膜淋巴结综合征），是一种儿童常见的急性全身性血管炎疾病。

该疾病最常发生在儿童年龄段，尤其是在5岁以下的儿童中。

川崎病病因尚不明确，但早期诊断和及时治疗非常关键，以避免出现严重并发症，特别是对心脏的影响。

目前，川崎病的治疗主要侧重于控制病情、减轻炎症反应，并防止并发症的发生。

以下是常用的川崎病治疗方案：1. 高剂量免疫球蛋白（IVIG）治疗：IVIG是目前最主要的川崎病治疗药物之一。

通常以2g/kg剂量通过静脉注射给药，疗程为单次给药。

IVIG的主要作用是降低体内炎症反应，减轻血管壁炎症，并预防冠状动脉病变的发生。

该药物常与其他治疗手段结合使用，以取得更好的治疗效果。

2. 高剂量阿司匹林治疗：阿司匹林通常在IVIG治疗后开始使用，剂量为80-100mg/kg/日分次服用。

该药物主要通过抗炎和抗血小板聚集作用，减轻川崎病患儿的症状，降低冠状动脉病变的风险。

在恢复期，剂量逐渐减少，以防止副作用，但需根据患儿体重和临床情况进行调整。

3. 丙种球蛋白治疗：对于IVIG无效或临床症状持续存在的患儿，丙种球蛋白治疗是一种备选方案。

通常以1-2g/kg剂量通过静脉注射给药，疗程可根据疾病进展进行调整。

丙种球蛋白可中和体内炎症介质，改善病情，预防并发症。

4. 其他治疗措施：除上述主要治疗方案外，川崎病患儿还可以接受其他治疗措施，如局部皮肤护理、镇痛药物、降温措施等，以缓解疾病症状和不适。

川崎病的治疗过程中，以下几点需要注意：1. 早期诊断和治疗：川崎病的症状与其他感染性疾病相似，因此早期诊断非常重要。

一旦怀疑川崎病，应立即就诊，并尽早启动治疗以避免并发症的发生。

2. 专业医生的指导：治疗川崎病应由经验丰富的小儿科医生或专科医生进行指导。

他们可以根据患儿年龄、病情严重程度和其他相关因素，制定个体化的治疗方案。

3. 长期随访和监测：川崎病的治疗通常需要长期随访和监测，以确保患儿的病情得到有效控制，并及时发现并预防任何可能的后遗症，尤其是对心脏的影响。

川崎病疾病诊断标准、丙球冲击治疗、丙球无反应型川崎病无效风险、激素治疗指征和糖皮质激素给药方案川崎病也称粘膜皮肤淋巴结综合征，是一种急性全身性中小动脉血管炎，常见于6个月至5岁儿童。

川崎病引发冠状动脉病变是儿童获得性心血管疾病的最主要原因。

静脉注射丙种球蛋白冲击联合阿司匹林口服是川崎病的一线治疗，10%至20%川崎病患儿对IVIG耐药，为IVIG无反应型川崎病。

川崎病诊断川崎病一般通过临床表现和辅助检查相结合确立诊断。

对于诊断指南，六项诊断标准中应有五项即可诊断KD，将发热作为强制性标准，其余五项标准中满足四项可明确诊断。

表1. 川崎病诊断标准约15% 的川崎病患儿仅满足上述诊断标准的3～4 项，但在病程中经超声心动图或冠脉造影证实存在冠状动脉瘤，或满足上述诊断标准中的 4 项，但超声心动图可见冠状动脉壁辉度增强，称为不完全/非典型川崎病。

除超声心动图外，实验室检查指标对诊断川崎病有一定意义（。

表2. KD 常见的实验室检查异常丙球冲击治疗：川崎病一线治疗IVIG可显著降低川崎病并发冠状动脉瘤风险、减轻患者发热等临床表现，已被用于川崎病的标准一线治疗长达40年。

IVIG 的作用机制可能与调节炎性细胞因子、中和细菌或病毒介导的超抗原抑制和抑制T 细胞的活化从而减少抗体的生成有关。

在KD 症状出现后10天内一次性给予2 g/kg，10～12 h 内输注完毕。

症状出现超过10天给药可能缓解川崎病活动期患者的症状，但对预防冠状动脉瘤作用有限。

IVIG 给药方案有三种：1）400 mg/(kg·d)，用5 d；2）1 g/(kg·d)，用2 d；3）2 g/(kg·d)，用1 d。

IVIG无反应型KD诊断接受IVIG 治疗36 h 后发热不退（T > 38 ℃）或退热后2～7 d 后再次出现发热并伴有至少一项川崎病主要临床特征，则判断为IVIG 无反应型川崎病。

IVIG 无反应的风险因素包括：男性、年龄<12 个月、发病超过10天后给予 IVIG、血小板减少、低白蛋白血症、贫血、低钠血症、IVIG 输注后 CRP 持续升高、IVIG 输注后白蛋白持续降低。