儿童 2 年内川崎病多次复发 1 例报告#br#

目的：探讨儿童川崎病（KD）多次复发情况。方法回顾分析1例多次复发KD患儿2年的临床资料。结果患儿,男,4岁。于2岁时第1次患典型KD,无冠状动脉损伤,以后2年内又患不完全KD共4次,均表现为发热伴颈部淋巴结肿痛、双侧球结膜充血、唇红、皲裂、杨梅舌,无咽痛、咽炎、口腔溃疡,血常规白细胞计数、C反应蛋白、红细胞沉降率均升高,颈部淋巴结超声均未见化脓性改变,大剂量丙种球蛋白静脉输注后均退热,每次发热持续时间为6~8 d,相邻2次发热之间的无热间隔期约为2.5、1.5、4.5、13.5个月,临床表现和基因检测结果均不符合周期性发热、口疮性口炎、咽炎及颈淋巴结炎综合征（PFAPA）。结论儿童2年内患5次KD罕见,需要长期随访。     

儿童川崎病与关节炎关系的研究进展

儿童川崎病(Kawasaki disease,KD)或不典型川崎病(incomplete Kawasaki disease,IKD)是一种以全身血管炎病变为主要病理特征的疾病,临床主要表现为无明显诱因的发热,伴有皮肤黏膜及淋巴结的损害.由于本病常伴有严重的心血管并发症如冠状动脉瘤,故引起人们的重视.但由于约三分之一患儿可伴有关节炎或关节痛,早期不易与幼年特发性关节炎区别,尤其是与全身型幼年特发性关节炎(sys-temic-onset juvenile idiopathic arthritis,SoJIA)的早期区分困难.KD和SoJIA是对于儿童长期发热、皮疹、淋巴结肿大的不同诊断,诊疗程序也截然不同,但在早期临床诊断上完全区分两者是很困难的,尤其是IKD和早期SoJIA.故该文对KD与关节炎的关系作一综述,旨在提醒临床医生重视KD与IKD的诊断,以免延误治疗.     

Castleman's disease (angiofollicular hyperplasia) in children

This unusual clinicopathologic entity was first recognized by Castleman. Onset is usually in adolescence or early adulthood, occasionally childhood. A single, large mass with no tendency to compress develops, usually in the mediastinum, occasionally at other lymph node sites (superficial, retroperitoneal, abdominal), and exceptionally in extranodal locations. A variety of systemic manifestations may be found, including fever, anemia, hypergammaglobulinemia, and a broad spectrum of inflammatory and autoimmune disorders, presumably associated with specific humoral factors produced by the tumor. Histologic examination shows the characteristic features of angiofollicular lymph node hyperplasia. Two variants have been described, i.e., the plasma cell type (active stage) associated with systemic manifestations, and the hyaline vascular type (quiescent stage). Surgical removal of the tumor ensures permanent (local and systemic) recovery in the overwhelming majority of cases. A role of microorganisms in the etiology of this condition has been suggested. Identical histologic changes can be found in adults with immune dysfunction syndromes. The etiology, pathogenesis and nosology of this very benign disease are still poorly known.     

Kikuchi-Fujimoto disease of 16 children in a single center of Korea

Kikuchi-Fujimoto disease (KFD) is subacute necrotizing lymphadenitis characterized by fever, leukopenia and cervical lymphadenopathy. There are few reports on the clinical characteristics and laboratory findings of KFD in the pediatric literature. In this study, we evaluate the characteristics and outcome of KFD in children. A total of 412 patients were studied with fever and peripheral lymphadenopathy at Pusan National University Hospital from January 1998 to December 2003. Among the total 412 there were 16 patients diagnosed with KFD by lymph node biopsy. We analyze the clinical, laboratory and outcome after review of the medical records retrospectively. The mean age of the patients was 10.6 +/- 3.4 yr (range: 4-17 yr). The male to female ratio was 1:1. Almost all patients, except two, had cervical lymph node swelling. The size of the involved lymph node was less than 4 cm in the greatest dimension in 75% of the patients. All the children had fever as the chief complaint and the mean duration of the fever was 17.7 +/- 11.2 days (range: 2-122 days). Approximately 87% of the patients had leukopenia (WBC < 4000/mm(3)) and 43.8% of the patients had a mild increase in the transaminases on liver function testing. A total of 8 out of the 16 patients were initially misdiagnosed as an infectious disease and treated with antibiotics which caused prolonged hospitalization for most patients. Six patients were treated with prednisone and the prolonged fever subsided immediately after steroid therapy. KFD should be considered in the differential diagnosis of prolonged fever in children with cervical lymphadenopathy. Early cervical lymph node biopsy is necessary to minimize inappropriate examinations and treatments in such cases.     

5岁以上年长儿川崎病临床特征分析

目的 总结5岁以上儿童川崎病（KD）的临床及实验室特征。方法 将确诊为KD或不完全KD的250例年长儿分为 > 5~9岁（191例）及 > 9岁亚组（59例），与266例1~5岁KD患儿进行回顾性分析比较，比较3组间的临床及实验室特点。结果 3组间手足肿胀、指端或肛周脱屑的发生率均以 > 9岁组最低，而颈淋巴结肿大的发生率以 > 5~9岁组最高，总热程以 > 9岁组最长（P < 0.05）。3组间皮疹、球结膜充血、口唇改变的发生率及不完全KD所占比例、静脉丙种球蛋白（IVIG）无反应发生率的差异无统计学意义（P > 0.05）。PLT、白蛋白水平以 > 9岁组最低，中性粒细胞百分比以 > 5~9岁组最高（P < 0.05）。3组间WBC总数及CRP、ESR、ALT的差异无统计学意义（P > 0.05）。轻度冠脉扩张以1~5岁组最多，中型冠状动脉瘤的发生以 > 9岁组最多（P < 0.05）；3组间巨大冠脉瘤发生率的差异无统计学意义（P > 0.05）。结论 5岁以上年长儿KD临床表现不典型，但颈部淋巴结肿大发生率和中性粒细胞百分比相对较高，PLT及白蛋白水平偏低。年龄越大发生中型冠状动脉瘤的风险越大。     

Long-term consequences of Kawasaki disease among first-year junior high school students.

OBJECTIVE: To determine whether height, weight, and electrocardiographic (ECG) abnormalities differ at the age of 13 years between adolescents with and without Kawasaki disease (KD) (also known as mucocutaneous lymph node syndrome). DESIGN: A population-based cohort study. SETTING: School community and public health service. METHODS: Data from the nationwide surveys of KD in Japan were linked with those of junior high school cardiac screening in Tochigi Prefecture between April 1, 1986, and June 30, 1998 (except data for 1988 and 1994). Using this database, students with KD and students without KD in the first year of junior high school were compared. MAIN OUTCOME MEASURES: Height, weight, and ECG abnormalities. RESULTS: Eight hundred sixty students (486 adolescent boys and 374 adolescent girls) with KD and 308 729 students (158 193 adolescent boys and 150 536 adolescent girls) without KD were located. There was no difference in average height and weight between the students with and without KD (using the P values for a t test of the means). The proportion of abnormal ECG findings was 10% and 3% among those with and without KD, respectively. CONCLUSION: Even at age 13 years, the proportion of abnormal ECG findings was about 3 times higher among adolescents with KD (using a chi(2) test of the difference in 2 binomial proportions).     

巨噬细胞活化综合征的临床和实验室特征分析

目的分析巨噬细胞活化综合征(MAS)患儿诊断初期的临床和实验室特征,探索早期识别MAS的方法。方法回顾性分析21例MAS患儿的临床、实验室特征,以及治疗和转归。结果 MAS患儿的原发病包括全身型幼年特发性关节炎(SJIA)14例、川崎病(KD)5例和结缔组织病(CTD)2例。发生MAS的中位时间为19 d,以KD-MAS发生最快,CTD-MAS发生最晚(P=0.009)。前10位的临床症状依次为发热(95%),皮疹(86%),淋巴结肿大(67%),骨髓吞噬现象(63%),肺部病变(62%),浆膜腔积液(62%),肝肿大(52%),脑脊液异常(50%),中枢神经系统损害(43%)和脾肿大(38%)。实验室特征方面,血红蛋白降低;超敏C反应蛋白、血沉升高、血清铁蛋白明显升高;谷丙转氨酶、谷草转氨酶、乳酸脱氢酶和甘油三酯升高;纤维蛋白原降低,D-二聚体明显升高;IL-6、IL-10和IFN-γ明显升高。21例患儿中20例好转出院。结论风湿性疾病患儿如出现持续发热,肝功能损害,凝血功能异常,甚至多脏器损害,以及IL-10、IFN-γ明显升高和血清铁蛋白持续升高,要高度警惕MAS发生。     

Studies of Peripheral Blood Toxic Neutrophils as a Predictor of Coronary Risk in Kawasaki Disease—the Pathogenetic Role of Hematopoietic Colony-Stimulating Factors (GM-CSF, G-CSF)

Many toxic neutrophils, characterized by cytoplasmic swelling, vacuolization and toxic granulation, can be seen in the acute phase of Kawasaki Disease (KD; mucocutaneous lymph node syndrome). Polymorphonuclear neutrophils (PMNs) of 36 patients with acute and convalescent phase KD were studied with reference to their morphological characteristics. The percentage of toxic neutrophils in the acute phase tended to be higher in the group with coronary artery lesions (CAL) than in the group without CAL. The results suggest that observation of toxic neutrophils in the acute phase of KD may be useful in predicting a high risk of CAL. In vitro cultivation studies of PMNs from healthy persons, in the presence of diverse hematopoietic colony-stimulating factors (CSF), revealed that GM-CSF (granulocyte-macrophage-CSF) and G-CSF (granulocyte-CSF) participated in the induction of cytoplasmic vacuolization of PMNs, and that the healthy PMNs morphologically mimicked peripheral PMNs seen in the acute phase of KD.     

Hemophagocytic lymphohistiocytosis preceded by Kikuchi disease in children

Background  Kikuchi disease (KD) is a type of benign, self-limiting lymphadenitis, but it has also been associated with hemophagocytic lymphohistiocytosis (HLH). To date, only a few reports have suggested an association between HLH and KD. Objective  To report the imaging findings and clinical characteristics of KD accompanied by HLH in children. Materials and methods  Five children with a prolonged fever and cervical lymphadenopathy were diagnosed as having HLH accompanied by KD. The authors retrospectively analyzed the clinical characteristics and the imaging findings in these children. The histology of excision biopsy samples of cervical lymph nodes in all children confirmed the diagnosis of KD. HLH was confirmed by bone marrow biopsy and laboratory criteria provided by the Histiocyte Society. Results  The greatest dimension of the enlarged nodes ranged from 0.5 cm to 2.5 cm and the nodes were most frequently located at level V. CT scans visualized perinodal infiltrates in most of the affected cervical nodes (four of five children) and extracervical nodes (three of three children). On enhanced CT scans, nonenhancing necrosis within the affected cervical nodes was noted in three children. Conclusion  KD might be related to HLH in children. Systemic evaluations and follow-up of children with KD might help to identify HLH related to KD.     

Varying clinical features of Turkish Kawasaki disease patients

Kawasaki disease (KD) is an acute, systemic and self-limited vasculitis that is complicated with the development of coronary artery (CA) aneurysms. We present the clinical features of Turkish KD patients from a tertiary referral center. When 33 KD patients were assessed, a number of features stood out as differing from the expected, for example, periungual peeling 7.5 +/- 7.5 days after fever onset--42.4% of patients had periungual peeling within 14 days after fever onset. CA involvement was detected at an average of 12.3 +/- 7.9 days after fever onset. Fifty percent of the patients had been diagnosed to have CA involvement within eight days after the onset of fever. The performance of criteria suggested by American Heart Association was satisfactory, with 19 of 29 patients (65.5%) having three or more of the required laboratory features (sensitivity 65.5%). We believe Turkish patients may present differences in the course of KD.     

川崎病治疗前发热时间与丙种球蛋白耐药的相关性临床研究北大核心CSCD

目的 探讨川崎病(Kawasaki disease,KD)患儿丙种球蛋白(intravenous immunoglobulin,IVIG)治疗前发热时间与IVIG耐药的关系。方法 回顾性收集2018年1月至2020年12月收治KD患儿317例的病例资料,根据IVIG治疗前发热时间分为短热程组(发热时间≤4 d,n=92)和长热程组(发热时间>4 d,n=225),根据是否发生IVIG耐药将每组再分为耐药组和非耐药组。分析比较不同热程耐药组及非耐药组的基线资料及实验室结果,并采用多因素logistic回归分析IVIG耐药的影响因素。结果 短热程组中IVIG耐药19例(20.7%),并发冠状动脉瘤5例(5.4%);长热程组中IVIG耐药22例(9.8%),并发冠状动脉瘤19例(8.4%);短热程组IVIG耐药率明显高于长热程组(P<0.05),而冠状动脉瘤发生率在两组间差异无统计学意义(P>0.05)。短热程组中,耐药患儿治疗前血钠水平低于非耐药患儿,而降钙素原、C反应蛋白及N末端B型利钠肽原水平则明显高于非耐药患儿(P<0.05)。长热程组中,耐药患儿治疗前血钠及肌酸激酶水平低于非耐药患儿(P<0.05)。多因素logistic回归分析显示,血钠水平降低与长热程组KD患儿IVIG耐药有关(P<0.05)。结论 KD患儿IVIG耐药因IVIG治疗前发热时间不同而异。在治疗前发热时间>4 d的KD患儿中,血钠降低与IVIG耐药具有相关性。     

Clinical features of recurrent Kawasaki disease and its risk factors

The clinical features and risk factors for recurrence of Kawasaki disease (KD) remain unclear. In order to summarize clinical features of recurrent KD and identify risk factors associated with recurrence, we conducted a retrospective review of the medical records of consecutive cases of KD from January 2002 to December 2010. Demographic, clinical, laboratory, and echocardiographic data were analyzed. The maximum coronary artery Z score normalized against body surface area was assessed using coronary artery diameters. At the first onset of recurrent KD, children had longer durations of fever before intravenous immunoglobulin (IVIG) treatment and higher levels of alanine aminotransferase, serum aspartate aminotransferase (AST), and lower hemoglobin levels than those with a single episode of KD. Multivariate logistic regression analysis showed that long durations of fever before IVIG treatment, high AST levels, and reduced hemoglobin levels were significantly associated with recurrent KD. Ten of the 22 recurrent KD children had coronary artery complications during the first onset episode, and six (60 %) of these also had coronary artery complications during the recurrence. Children with longer durations of fever, lower hemoglobin levels, and higher AST levels may be at increased risk for KD and coronary artery complications are more likely to occur in children with recurrent KD if they were present during the first episode.     

Kikuchi–Fujimoto disease following vaccination against human papilloma virus infection and Japanese encephalitis

Kikuchi-Fujimoto disease (KFD) is a benign and self-limited syndrome, characterized by regional lymphadenopathy and fever. Although the pathogenesis of KFD remains unclear, infectious agents, autoimmune causes, and physicochemical factors have been suggested as triggers. However, KFD following vaccination has never been reported. We present a 14-year-old girl who suffered from fever and cervical lymph node swelling following simultaneous administration of human papilloma virus vaccine and Japanese encephalitis virus vaccine. The patient was diagnosed with KFD based on the histopathologic findings of a lymph node biopsy, and her fever and swelling resolved with oral corticosteroid therapy. Although the exact pathogenesis of the development of KFD following immunization remains unknown, this should be added to the list of potential triggers or factors associated with the development of KFD.     

283例川崎病的临床分析

目的 总结川崎病住院患儿的临床表现及心血管系统的并发症,为尽早、正确判断川崎病的病情与预后提供临床依据。方法 回顾性研究1992—2002年住院川崎病患儿283例,分析:(1)川崎病各种临床表现的发生率;(2)合并冠状动脉瘤发生的危险因素。结果(1)283例中男186例,女97例;男女比例为1.9:1,年龄分布以婴幼儿为主,3岁以内(包括3岁)占71％;发病季节以春、夏季为主;不典型川崎病55例(19.4％);就诊前平均发热天数6.1 d,除发热外,其他症状出现频率的次序是:口腔黏膜改变(97.5％),淋巴结肿大(95.4％),球结膜充血(91.2％),指、趾端脱皮(89.8％),皮疹(81.5％)。(2)心血管改变情况:治疗前合并冠状动脉(简称冠脉)扩张共103例(36.9％),住院2周后新发生冠脉扩张28例(13.3％);有13例形成动脉瘤(4.7％),在冠状动脉病变人群中占9.9％。分析动脉瘤的形成是与性别、发热持续时间(>8.9 d)有关(P<0.05)。治疗前合并心房、室腔扩大40例(14.3％);心包积液11例(3.9％);合并心电图改变有57例(20.8％)。结论 川崎病心血管并发症出现较早,经治疗后有13.3％新发冠状动脉病变。动脉瘤的形成与性别、发热持续时间有关。     

儿童免疫相关性疾病的常规免疫学指标研究

目的比较特发性血小板减少性紫癜(ITP)、过敏性紫癜(HSP)及皮肤黏膜淋巴结综合征(KD)的常规免疫学指标变化及静脉注射免疫球蛋白(IVIG)的应用。方法用流式细胞仪测T细胞亚群,免疫比浊法测免疫球蛋白(Ig)及补体。常规加IVIG治疗。结果ITP患儿CD3 CD4 T细胞降低并IgG升高;HSP患儿IgA升高伴CD3 T细胞增高。KD患儿CD3 CD8 T细胞降低。IVIG可明显改善3种疾病临床症状,尤其有助KD患儿冠状动脉损害发生率减少。结论T细胞亚群、lg 应作为ITP、HSP及KD的常规检查项目。早期应用IVIG对改善预后及缩短病程有重要意义。     

川崎病延迟诊断的危险因素分析

背景：目前对川崎病（KD）尤其是不典型KD的及时诊断仍面临困难。 目的：分析KD患儿延迟诊断的影响因素。 设计：病例对照研究。 方法：采用日本川崎病研究委员会2005年发布的第5版KD诊断标准,6条主要诊断标准中满足≥5条即可诊断KD。纳入2009年1月至2014年12月在浙江大学医学院附属儿童医院住院的KD患儿,发病10 d后诊断KD者为延迟诊断组,反之为无延迟诊断组。截取患儿的一般信息、临床表现、总热程、发病后首次实验室检查结果、IVIG治疗后的第1次血常规等,在两组间比较的基础上行多因素logistic回归分析,危险因素的拟合优度检验采用Hosmer and Lemeshow检验。 主要结局指标：延迟诊断KD的影响因素。 结果：2 194例KD患儿中,男1 354例,女840例,发病月龄中位数21（11~43）个月,延迟诊断组168例,无延迟诊断组2 026例。延迟诊断组中男性、＞5岁比例较高,总热程较长,入院时满足主要诊断标准数较少、不典型KD患儿比例较高; ALB、血钠较高,ALT、TBil、GGT较低;冠状动脉损伤和扩张的发生率较高;IVIG治疗前后CRP、N绝对值和%、N/L比例均较低;差异均有统计学意义。总热程≥10 d（OR=36.1,95%CI：9.7~133.8）是延迟诊断KD的危险因素,入院满足诊断标准数≥5个（OR=0.40,95%CI：0.29~0.54）是延迟诊断KD的保护因素。 结论：总热程≥10 d和入院满足主要诊断标准数＜5个可能是KD延迟诊断的危险因素。     

Early treatment with intravenous immunoglobulin in patients with Kawasaki disease

OBJECTIVES: To determine if a shorter interval between Kawasaki disease (KD) treatment with intravenous immunoglobulin (IVIG) and fever onset results in increased treatment failures, need for adjunctive therapy, or development of coronary artery lesions. STUDY DESIGN: Patients with KD (n = 178; 89 matched pairs) diagnosed between 1987 and 1999 were included in this case-control study. All patients had fever plus at least 4 of the 5 clinical criteria for KD. Eighty-nine patients who received IVIG at day 5 or earlier were matched to patients diagnosed within 4 weeks and given IVIG at days 6 to 9 of fever. Compiled data from a detailed chart review included demographics, clinical features, fever duration, investigations, disease course, and response to therapy. Differences between matched case and control pairs were analyzed by means oft tests and McNemar tests. RESULTS: No demographic differences were noted between the two groups. Patients treated on day 5 or less of fever had a shorter total fever duration (5.2 +/- 1.9 days vs 8.0 +/- 1.8 days, P <.0001), longer fever after IVIG treatment (1.5 +/- 1.9 days vs 0.8 +/- 1.3 days, P =.008), and less coronary artery ectasia at 1 year after KD onset (4% vs 16%, P =.02). There was no significant difference between cases and control patients in the number of patients with KD recrudescence, need for repeat courses of IVIG, need for corticosteroids, length of hospitalization, or development of coronary artery aneurysms within the first 3 months. Patients who were treated on day 5 or less of fever had higher levels of serum albumin (36 +/- 5 g/L vs 33 +/- 5 g/L, P <.01) and serum ALT (115 +/- 155 U/L vs 46 +/- 49 U/L, P <.001) as well as a lower platelet count (354 +/- 131 vs 403 +/- 166, P =.02) than did control patients during the acute phase. CONCLUSIONS: Early treatment of KD resulted in less coronary ectasia at 1 year after KD onset but was not associated with a quicker resolution of fever, an increased number of treatment failures, an increased need for adjunctive therapy, length of hospitalization, nor development of coronary artery lesions. In children with fever and classic clinical and laboratory findings of KD, treatment with IVIG on or before 5 days of fever resulted in better coronary outcomes and decreased the total length of time of clinical symptoms.     

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??Pediatric mesenteric lymph node enlargement is one of the most common causes of pediatric abdominal pain. The diagnostic criteria for most current report is??the longitudinal diameter > 0.5 cm??vertical and horizontal diameter ratio ??L/T?? more than 2??more than two of lymph nodes changed diffusely and uniformly. Ultrasound can not only diagnose lymph node enlargement timely and accuratly but also evaluate the morphology and blood flow of lymph node??helping us to determine whether there’s a mesenteric lymph node enlargement pathologically and provide the basis for clinical diagnosis and treatment.     

Harada评分预测川崎病冠状动脉病变的临床意义

目的 探讨Harada评分对中国人群样本中川崎病（KD）合并冠状动脉病变高危因素判断的临床价值。方法 参照KD诊断标准及Harada评分标准，回顾性分析187例KD息儿发病10d内白细胞（WBC）计数、红细胞压积（HCT）、C反应蛋白（CRP）、发热天数、心电图及超声心动图等各项指标；采用χ^2检验和多元回归分析，分别检验上述因素与KD合并冠脉损害的相关性；比较冠脉损害组与非冠脉损害组Harada评分的差异。结果发病10d内单因素分析发病年龄、WBC计数、HCT、CRP、心电图改变及发热天数与KD合并冠脉损害有密切相关性。多因素分析结果显示，发病10d内CRP、发热天数与KD合并冠脉损害的发生独立相关。发生冠脉损害患儿Harada评分的平均分高于无冠脉损害者。结论 Harada评分可作为川崎病冠状动脉病变的一项预见性指标，也可作为中国人群川崎病患者早期是否应用静脉丙球的筛选指标。     

Clinical and laboratory manifestations of Kikuchi's disease in children and differences between patients with and without prolonged fever

BACKGROUND: Kikuchi's disease (KD) is characterized by cervical lymphadenopathy with or without fever. It has been recognized worldwide but seldom reported in pediatric patients. METHODS: From January 1985 through December 2001, 64 patients younger than 18 years of age with pathologic proof of KD were enrolled in this study. The clinical manifestations, laboratory data and outcomes were reviewed. RESULTS: There were 35 male patients and 29 female patients with age ranging from 2 to 18 years and a median age of 16. All patients had cervical lymphadenopathy except 1 who had generalized lymphadenopathy. Lymph nodes of 32 patients (50%) were painful or tender or both. Lymphadenopathy was unilateral in 52 patients (82.5%). Lymphadenopathy associated with fever was observed in 21 patients (32.8%). Other signs such as skin rash, hepatomegaly or body weight loss were less common. Twenty-six patients (40.6%) had leukopenia and 2 patients had leukocytosis. Nearly one-fourth of the patients had mild liver dysfunction. Virologic or immunologic studies were normal in most patients. Patients with prolonged fever were more likely to have leukopenia (P < 0.05). All patients recovered, but 1 developed systemic lupus erythematosus 5 years later, and the other had vasculitis syndrome 2 years later. CONCLUSIONS: The clinical presentation of KD in pediatric patients is similar to that of adults. KD is a benign, self-limiting disease; prolonged fever occurred only in 32.8% of pediatric patients in our cohort. Leukopenia was the only feature significantly associated with prolonged fever.