Corticosteroids in the treatment of the acute phase of Kawasaki disease.

OBJECTIVES: Corticosteroids are considered to be contraindicated during the acute phase of Kawasaki disease (KD) based on unfavorable results in early studies. In our hospital, however, corticosteroids have been used in some cases of KD with satisfactory results. We analyzed outcomes of patients with KD treated with or without corticosteroids. STUDY DESIGN: Medical records of 299 children with KD treated with one of the 4 regimens were reviewed retrospectively. Regimen 1 consisted of aspirin, dipyridamole, and propranolol; regimen 2 was regimen 1 plus prednisolone, 2 mg/kg/d, for 1 week, followed by tapering over 2 weeks; regimen 3 was regimen 1 plus intravenous gamma-globulin (IVGG), 200 or 400 mg/kg/d, for 5 consecutive days; and regimen 4 was regimen 1 plus both prednisolone and IVGG. RESULTS: Although patients treated with regimens 2 and 4 were more ill at presentation than those treated with regimens 1 and 3, respectively, the duration of fever was shorter in the former patient groups (P =.0013). Coronary aneurysms developed least frequently in patients treated with regimen 4 and less frequently with regimen 2 than with regimen 1 (P =.0730). Multiple regression analysis showed significant reductions of fever and coronary aneurysm incidence with prednisolone (P <.0001 and P =.0307, respectively). CONCLUSION: Our data suggest a possible role of corticosteroids in the treatment of the acute phase of KD.     

糖皮质激素联合免疫球蛋白用于川崎病初始治疗有效性的Meta分析

目的 系统评价糖皮质激素(GCs)联合静脉注射免疫球蛋白(IVIG)用于川崎病(KD)初始治疗的有效性和安全性。方法 计算机检索MEDLINE数据库、PubMed数据库、CNKI、万方数据库、维普电子期刊全文数据库等,收集GCs联合IVIG初始治疗儿童KD的前瞻性对照研究或回顾性对照研究,检索时间为各数据库建库至2016年3月。由 2位研究者独立筛选文献、提取资料并对纳入文献进行质量评价后,采用RevMan5.2软件进行Meta分析。结果 共纳入11篇文献,均为英文文献,其中前瞻性研究7项,回顾性研究4项。Meta分析结果显示:与单用IVIG相比,GCs联合IVIG组的冠状动脉损害(CAL)发生率更低 (OR = 0.44,95%CI: 0.23~0.86,P = 0.02),发热持续时间更短 (MD = -1.66,95%CI: -2.32 ~ -1.01,P < 0.00001),且首次治疗未反应率低于单用IVIG组 (OR = 0.37,95%CI: 0.27~ 0.51,P < 0.00001)。两组复发率和不良反应发生率比较差异均无统计学意义。结论 GCs联合IVIG初始治疗KD能降低患儿CAL发生率及首次治疗未反应率,缩短发热时间,且不增加复发率和不良反应的发生。     

联合糖皮质激素初始治疗川崎病的临床分析

目的 探讨糖皮质激素在联合静脉丙种球蛋白和阿司匹林传统治疗方面的优势,探索激素运用在整个病程中的合适时机和合适剂量.方法 参照川崎病诊断标准,将363例川崎病患儿随机分为常规治疗组和联合激素组,将联合激素组根据临床表现分为完全川崎病组和不完全川崎病组,分别分析治疗前和治疗1周内两组患儿发病年龄、性别、治疗应用后发热病程、丙种球蛋白治疗时间、住院天数、总住院费用;以及患儿病程急性期,恢复期中有关化验指标,1月后冠状动脉损害情况.结果 联合激素组比常规治疗组在治疗后热程(P<0.05)、住院天数(P<0.05)和住院费用(P<0.05)中,有明显减少,两者差异有统计学意义,在各项临床化验指标及冠状动脉损害病变中无明显统计学意义(P值大于0.05).在不同类型川崎病中,完全组激素治疗较不完全组同样在治疗后热程(P<0.02),住院天数(P<0.03)和住院费用(P<0.04)方面显著减少,两者对比存在统计学意义,而在各项临床化验指标及冠状动脉损害病变中无明显统计学意义(P>0.05).结论 联合激素治疗对缩短川崎病治疗后热程及患儿经济负担方面有积极作用.     

Orlistat treatment of unconjugated hyperbilirubinemia in Crigler-Najjar disease: a randomized controlled trial

Unconjugated hyperbilirubinemia in Crigler-Najjar (CN) disease is conventionally treated with phototherapy and phenobarbital. Orlistat treatment increases fecal fat excretion and decreases plasma unconjugated bilirubin (UCB) concentrations in Gunn rats, the animal model for CN disease. We determined in CN patients the effects of orlistat treatment on plasma UCB concentrations, and on fecal excretion of fat and UCB. A randomized, placebo-controlled, double-blind, cross-over trial was conducted in 16 patients, simultaneous with their regular treatment (phototherapy, n = 11, and/or phenobarbital, n = 6). Patients received orlistat or placebo, each for 4-6 wk. Compared with placebo, orlistat increased fecal fat excretion (+333%) and fecal UCB excretion (+43%). Orlistat treatment significantly decreased plasma UCB concentration (-9%). In 7 of 16 patients, the decrease in plasma UCB levels was clinically relevant (>10%, mean 21%). In patients with a clinically relevant response, plasma UCB concentrations during orlistat were strongly, negatively correlated with fecal fat excretion (r = -0.93). Clinically relevant response to orlistat treatment was not correlated with age, sex, CN type, BMI, or co-treatment with phototherapy or phenobarbital, but appeared correlated with a relatively lower dietary fat intake. In conclusion, orlistat treatment decreases plasma UCB concentrations, particularly in a subgroup of CN patients. Dietary fat intake may determine the responsiveness to orlistat treatment.     

Corticosteroids in the treatment of gastrointestinal disease

Corticosteroids remain the mainstay of anti-inflammatory and immunosuppressive therapy for many gastrointestinal conditions. We are now starting to understand their mechanism of action and the phenomenon of corticosteroid resistance. Because of the ubiquity of corticosteroid receptors in virtually all cells of the body, side effects of therapy are common and may affect multiple body sites. Newer corticosteroid analogues are being developed to minimize these complications, and concomitant use of other immunomodulatory drugs often facilitates corticosteroid dosage reduction or even withdrawal in chronic inflammatory states.     

Cholestasis as the initial feature of Kawasaki disease

Hepatobiliary involvement is uncommon in Kawasaki disease, and it is usually described as obstructive jaundice. From January 01, 2000 to August 31, 2010, 31 Kawasaki disease cases were diagnosed in our center. Three of them (9.7%) developed jaundice, but there were no gallbladder or bile duct abnormalities by ultrasonography, a feature rarely reported. Resolution of cholestasis paralleled improvement of the illness.     

肾上腺糖皮质激素联合乌司他丁治疗儿童川崎病的非随机对照临床研究

目的 探讨肾上腺糖皮质激素联合乌司他丁治疗儿童川崎病的临床疗效。方法 根据患儿病情和家长意愿,将2011年1月至2013年12月入院确诊为典型川崎病(KD)的104例患儿分为乌司他丁组(甲基强的松龙+乌司他丁,n=46)和静脉注射丙种球蛋白(IVIG)组(n=58)。观察两组患儿治疗前、治疗后1周、3个月及6个月时的冠状动脉内径的变化,热退时间,再次治疗情况,治疗前、治疗后1周及3周时白细胞(WBC)、血小板(PLT)、血红蛋白(HB)、C反应蛋白(CRP)、血沉的变化以及住院总费用。结果 两组患儿在治疗前、治疗后1周、3个月及6个月时冠状动脉内径比较差异均无统计学意义(P> 0.05)。治疗48 h乌司他丁组患儿体温均正常(100%),正常率高于IVIG组(83%)(PPP结论 甲基强的松龙联合乌司他丁治疗儿童KD没有增加冠状动脉瘤的发生风险;并能大大降低住院费用;与IVIG治疗相比,在KD急性期能更好的控制患儿的实验室指标,缩短发热时间。     

Serum albumin level predicts initial intravenous immunoglobulin treatment failure in Kawasaki disease

Objectives: Kawasaki disease (KD) is a systemic vasculitis primarily affecting children who are <5 years old. Intravenous immunoglobulin (IVIG) is the standard therapy for KD. However, many patients with KD still show poor response to initial IVIG treatment. This study was conducted to investigate the risk factors for initial IVIG treatment failure in KD. Methods: Children who met KD diagnosis criteria and were admitted for IVIG treatment were retrospectively enrolled for analysis. Patients were divided into IVIG‐responsive and IVIG‐resistant groups. Initial laboratory data before IVIG treatment were collected for analysis. Results: A total of 131 patients were enrolled during the study period. At 48 h after completion of initial IVIG treatment, 20 patients (15.3%) had an elevated body temperature. Univariate analysis showed that patients who had initial findings of high neutrophil count, abnormal liver function, low serum albumin level (≤2.9 g/dL) and pericardial effusion were at risk for IVIG treatment failure. Multivariate analysis with a logistic regression procedure showed that serum albumin level was considered the independent predicting factor of IVIG resistance in patients with KD (p = 0.006, OR = 40, 95% CI: 52.8–562). There was no significant correlation between age, gender, fever duration before IVIG treatment, haemoglobin level, total leucocyte and platelet counts, C‐reactive protein level, or sterile pyuria and initial IVIG treatment failure. The specificity and sensitivity for prediction of IVIG treatment failure in this study were 96% and 34%, respectively. Conclusion: Pre‐IVIG treatment serum albumin levels are a useful predictor of IVIG resistance in patients with KD.     

Pulse methylprednisolone with gammaglobulin as an initial treatment for acute Kawasaki disease

Approximately 15–20% of patients with Kawasaki disease (KD) are not responsive to high-dose intravenous gammaglobulin (IVIG). We have previously reported a predictive method for identifying IVIG-non-responsive patients (high-risk KD patients). We determined the safety and effectiveness of pulse methylprednisolone with high-dose IVIG (mPSL+IVIG) as a primary treatment for high-risk KD patients. Sixty-two high-risk KD patients were treated with pulse methylprednisolone 30 mg/kg over 2 h, followed by IVIG 2 g/kg over 24 h (mPSL+IVIG group) and were compared with a historical control group of 32 high-risk patients treated with IVIG 2 g/kg alone at the participating hospitals before this study was opened (IVIG group). High-risk patients were identified with at least two of three predictors (C-reactive protein ≥7 mg/dL, total bilirubin ≥0.9 mg/dL or aspartate aminotransferase ≥200 IU/L). Sixty-six percent (95% confidence interval [CI] 54–78%) of patients had a prompt defervescence in the mPSL+IVIG group compared with 44% (95% CI 26–62%) for the IVIG group (p = 0.048). Coronary artery lesions were observed in 24.2% (95% CI 13.2–35.2%) and 46.9% (95% CI 28.6–65.2%) of patients in the mPSL+IVIG and IVIG groups, respectively (p = 0.025). This is the first report showing that mPSL+IVIG is effective and safe as a primary treatment for high-risk KD patients.     

Clinical trial of single-dose intravenous gamma globulin in acute Kawasaki disease. Preliminary report

Gamma globulin administered in a single dose of 1 g/kg of body weight intravenously caused prompt clinical improvement in 27 of 32 consecutive children with Kawasaki disease treated by the 12th day of illness. Response was equally good for the 20 children treated in the first week and the 12 treated in the second week. Fever and clinical signs abated within the first day after treatment, the mean white blood cell count normalized by 48 hours, and the sedimentation rate continued to be elevated for about 2 weeks, while the platelet count rose during the first 2 weeks after treatment and returned to normal approximately 1 month after treatment. Five children with incomplete relief needed more than the single dose before resolution of signs and symptoms occurred. Coronary aneurysms in 2 patients before treatment regressed by 2 weeks. No patient developed coronary aneurysms. No child had sequelae of Kawasaki disease at a follow-up of 2 to 31 months. We believe that although this was a one-arm, uncontrolled pilot study, the results suggest that this protocol provides a safe, flexible, and effective treatment for acute Kawasaki disease.     

静脉注射免疫球蛋白无应答不完全性川崎病1例临床经验交流北大核心CSCD

该文报道1例静脉注射免疫球蛋白(intravenous immunogloblin,IVIG)无应答不完全性川崎病患儿。1岁女性患儿,表现为发热、皮疹、指端脱皮、冠状动脉扩张,给予第一剂IVIG治疗结束36 h后仍发热,再次给予第二剂IVIG治疗后体温恢复正常,出院1个月后随访冠状动脉内径恢复正常。该文总结了IVIG无应答不完全性川崎病的诊治经验,以期减少冠状动脉损害的发生。     

甲泼尼龙治疗川崎病的临床研究

目的探讨川崎病(KD)患儿初期治疗中应用甲泼尼龙的疗效。方法研究对象为2000年3月～2005年6月该院收治的74例KD患儿。根据甲泼尼龙的使用与否,分成两组,对照组(38例)单用静脉丙种球蛋白治疗[1g/(kg·d),连用2d];治疗组(36例)用甲泼尼龙[20～30mg/(kg·d),连用3d] 静脉丙种球蛋白[1g/(kg·d),连用2d]。然后对两组急性炎症控制及冠状动脉病变(CAD)情况进行对比。结果治疗组和对照组在体温、C-反应蛋白(CRP)、血沉(ESR)、血小板(PLT)恢复正常时间方面差异有显著性(P<0·05)·病程14～21d时发生CAD例数:治疗组3例(8%),对照组11例(29%),两组比较差异有显著性(P<0·05)。随访3个月,CAD例数:治疗组1例(3%),对照组8例(21%),两组比较差异亦有显著性(P<0·05)。结论川崎病患儿初期治疗中应用甲泼尼龙能缩短病程,安全有效降低冠状动脉病变发生。     

Septated pericarditis associated with Kawasaki disease: a brief case report

Kawasaki disease (KD) is primarily the systemic vasculitis of childhood that affects mainly the medium-sized arteries, such as the coronary arteries. KD is the leading cause of acquired heart disease, whereas the incidence of rheumatic fever has declined. The most serious complication is coronary artery involvement. Among the children with KD who developed cardiac complications, pericarditis is a rare complication, with an incidence of 0.07%. We report our experience in a 5.5-year-old child with KD complicated with aneurysm of the left anterior descendant coronary artery and septated pericardial effusion, which has not been reported in the literature. The pericardial effusion disappeared very dramatically with intravenous immunoglobulin (IVIG) therapy. We would like to point out that septated pericardial effusion in cases of KD do not need any further therapy other than IVIG and high-dose acetylsalicylic acid.     

川崎病远期并发缺血性脑卒中1例

No abstract available

     

姑表兄弟同患川崎病2例报道

病例1:男,1岁11个月,因间断发热13 d入院。患儿入院前13 d无明显诱因出现发热,体温最高39℃,热型不规则,无咳嗽,无呕吐,于当地医院治疗(具体不详),体温仍不稳定。入院前4 d查血支原体抗体阴性;血常规:WBC 12.77×109/L,L33.4%,N 54.9%,PLT 501×109/L,HGB 102 g/L;