首页 | 本学科首页   官方微博 | 高级检索  
相似文献
 共查询到20条相似文献,搜索用时 15 毫秒
1.
We studied the effect of γ-globulin (IVGG) and aspirin (ASA) on the development of the coronary artery lesions (CAL) of Kawasaki disease (KD) in three different protocols. Within 29 days of the onset of KD the echocardiographic evidence of CAL had developed in 39–42% of the patients in the ASA group, but only in 13.7–20.8% of the patients treated with IVGG (200 or 400 mgγkgX5). In long-term follow-up observation of CAL of these patients the evidence of CAL in both the ASA and the IVGG group regressed gradually; however, the residual rate of CAL was significantly low in the IVGG group at all times up to 24 months after onset. These facts suggest that when using IVGG for KD, we should select a dose of intact γ-globulin, 1,000 mgγkg or more in total, to prevent the occurrence of CAL. We have demonstrated not only a significant reduction in the occurrence of CAL in patients treated with IVGG but a reduction in the residual rate of CAL for two years as compared with those treated by ASA.  相似文献   

2.
Clinical effects of high-dose γ-globulin therapy in Kawasaki disease have been evaluated from the viewpoints of its inhibitory effects on platelet adhesion and thrombus formation on the vessel wall. Platelet adhesion to the subendothelium is the first step of thrombosis as well as platelet interaction with the vessel wall, which can be observed experimentally by Baumgartner's method. Twelve patients with Kawasaki disease treated with intact intravenous γ-globulin (IVGG) showed decreased platelet adhesion in contrast to ten patients treated with only aspirin (ASA) or flurbiprofen (FP). Addition of intact IVGG to normal blood in Baumgartner's method also resulted in decreasing platelet adhesion and thrombus formation; however, other pepsin-treated IVGG caused enhanced platelet adhesion and thrombus formation. Moreover, pretreatments of the vessel wall with both types of IVGG showed effects similar to those of addition. In conclusion, high-dose therapy with intact IVGG has inhibitory effects on platelet adhesion and thrombus formation. Although the mechanism of the effects is not yet clear, some competitive inhibition between intact IgG and adhesive protein such as von Willebrand factor is suggested, and Fc receptors of the platelet membrane and Fab and Fc receptors of the subendothelium of the vessel wall may have some role in the interaction.  相似文献   

3.
BACKGROUND: High-dose intravenous gamma-globulin (IVGG) plus aspirin (ASA) treatment is effective in preventing coronary artery complications in acute Kawasaki disease (KD). However, gamma-globulin is very expensive, especially in Japan. Furthermore the indication for IVGG treatment and the optimal dose of gamma-globulin remain controversial. OBJECTIVES: To examine these two issues, we used Harada's scoring system to investigate whether a single 2 g/kg dose therapy has any advantage over the 5 day 400 mg/kg per day therapy. METHODS: We studied 203 patients with KD who had no coronary artery complications on admission. Of these, 145 patients scored 4 or more on Harada score within the first 9 days of illness and were treated with IVGG treatment. Using a random number table, 72 patients were selected to receive a single 2 g/kg dose (2 g group), while the remaining 73 patients were treated with 400 mg/kg per day for 5 consecutive days (400 mg group). Those who had a Harada score of three or less received no IVGG (non-IVGG group) treatment (58 patients). RESULTS: The incidence rate of coronary artery complications in the 2 g group was significantly lower than in the 400 mg group. The duration of high fever, positive duration of C-reactive protein and the number of hospital days in the 2 g group were each significantly shorter than in the 400 mg group. The total medical expense in the 2 g group was significantly lower than in the 400 mg group. There were no coronary artery complications in the non-IVGG group. CONCLUSIONS: It was found to be clinically more effective and more cost effective to select a patient by Harada's scoring system and, where a score of four or more was obtained, to administer a single 2 g/kg intravenous dose of gamma-globulin for acute KD.  相似文献   

4.
Kawasaki disease in Adelaide: A review   总被引:1,自引:0,他引:1  
Abstract The role of Kawasaki disease (KD) as a contributor to early childhood cardiac morbidity in Adelaide was investigated by a review of hospital admission and case-note data from January 1979 to June 1990. There were 57 episodes in 55 patients. The epidemiological data in this South Australian series are similar to that seen in other Australian and New Zealand centres and correlate better with the clinical data from North America than from Japan. The average age of admission was 3.2 years (median 2.7 years) with 38 and 85% of cases being less than 2 and 5 years respectively. The male to female ratio was 1.5. The incidence of KD in the 0–5 year age group was 3.9 cases per 100000 children. This series represents a minimum number of cases for this period and illustrates an association of aneurysm-risk with prolonged fever, improved defervescence with the combination of intravenous γ-globulin (IVGG) and aspirin compared with aspirin alone; and a more severe disease process in the very young. The series supports the efficacy of single dose IVGG therapy. Antibiotics were given prior to diagnosis of KD in 79% of patients, often causing diagnostic confusion with possible drug reactions. The pathogenic mechanisms of KD are reviewed and a new hypothesis is proposed that incorporate mechanisms of vessel pathology resulting from release of endothelin and recognized mediators of endothelial damage including tumour necrosis factor-α and interleukin-1β.  相似文献   

5.
AIMS: To assess the hypothesis that an additional intravenous gammaglobulin (IVGG) infusion, if administered early, may prevent coronary artery lesions (CAL) in patients with Kawasaki disease (KD) who do not respond to initial IVGG therapy. METHODS: Forty four KD patients (17 with CAL and 27 without CAL), treated with additional IVGG because of persistent or recrudescent fever after initial IVGG therapy, were studied. Main outcome measures were the presence of CAL by echocardiography and the number of febrile days before and after start of additional IVGG infusion (pre- and post-additional IVGG). RESULTS: In univariate analyses, risk factors for CAL were the number of febrile days pre-additional IVGG, the number of febrile days post-additional IVGG, the number of days that initial IVGG was divided over, the white blood cell count pre- and post-additional IVGG, and the C reactive protein concentration pre-additional IVGG. In a multivariate analysis, the only independent risk factor was the number of febrile days pre-additional IVGG (> or =10 days; odds ratio 7.86; 95% CI 1.44 to 42.8; p = 0.02). CONCLUSIONS: Among KD patients with persistent or recrudescent fever after initial IVGG therapy, administration of additional IVGG before the first 10 febrile days was associated with a decreased prevalence of CAL, when compared with the prevalence in those who were retreated later. An additional IVGG infusion, if administered early, may prevent CAL in initial IVGG non-responders.  相似文献   

6.
Aims: To assess the hypothesis that an additional intravenous gammaglobulin (IVGG) infusion, if administered early, may prevent coronary artery lesions (CAL) in patients with Kawasaki disease (KD) who do not respond to initial IVGG therapy. Methods: Forty four KD patients (17 with CAL and 27 without CAL), treated with additional IVGG because of persistent or recrudescent fever after initial IVGG therapy, were studied. Main outcome measures were the presence of CAL by echocardiography and the number of febrile days before and after start of additional IVGG infusion (pre- and post-additional IVGG). Results: In univariate analyses, risk factors for CAL were the number of febrile days pre-additional IVGG, the number of febrile days post-additional IVGG, the number of days that initial IVGG was divided over, the white blood cell count pre- and post-additional IVGG, and the C reactive protein concentration pre-additional IVGG. In a multivariate analysis, the only independent risk factor was the number of febrile days pre-additional IVGG (⩾10 days; odds ratio 7.86; 95% CI 1.44 to 42.8; p = 0.02). Conclusions: Among KD patients with persistent or recrudescent fever after initial IVGG therapy, administration of additional IVGG before the first 10 febrile days was associated with a decreased prevalence of CAL, when compared with the prevalence in those who were retreated later. An additional IVGG infusion, if administered early, may prevent CAL in initial IVGG non-responders.  相似文献   

7.
Corticosteroids in the treatment of the acute phase of Kawasaki disease.   总被引:11,自引:0,他引:11  
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.  相似文献   

8.
Clinical characteristics to predict the development of coronary artery abnormalities (CAA) in Kawasaki disease (KD) were assessed by reviewing medical records of patients diagnosed with KD at Korea University Medical Center from March 2001 to February 2005. Of the 285 patients diagnosed with KD, 19 developed CAA (6.7%). Compared with the CAA(−) group, the CAA(+) group had a longer duration of fever after intravenous gamma-globulin (IVGG) injection (2.4±2.9 vs. 1.5±1.2 days, p=0.008) and higher C-reactive protein (CRP)(12.3±7.8 vs. 8.7±7.1 mg/dL, p=0.038). In particular, the CAA(+) group tended to have more than 7 days of fever before IVGG and more than 3 days of fever after IVGG (26.3 vs. 5.3%, p<0.001; 26.3 vs. 6.4%, p=0.002). When the IVGG responsiveness was defined by the presence of defervescence within 3 days after IVGG, IVGG-non-responders showed a higher incidence of CAA (22.7 vs. 5.3%, p=0.002). Non-responders had a longer duration of fever after IVGG (5.5±1.9 vs. 1.2±0.6 days, p<0.001) and a significantly increased CRP, AST, ALT and total bilirubin. Multivariate regression analysis for CAA showed that the only factor significantly associated with the development of CAA was total fever that lasted for longer than 8 days (OR=4.052, 95% CI=1.151–14.263, p=0.0293). Conclusively, the most important predictor of CAA in KD is total duration of fever longer than 8 days. Early identification of IVGG non-responders and active therapeutic intervention for fever in KD cases might decrease the incidence of CAA.  相似文献   

9.
The administration of intravenous γ-globulin (IVGG) for Kawasaki disease was investigated throughout Japan in 1993 by obtaining information from pediatric departments in 2652 hospitals that had more than 100 beds. Of 11,221 reported patients, 8958 patients (79.8%) received IVGG treatment. Of all the patients to whom IVGG was administered, the most common total dose was 1000 mg/kg (36.3%) followed by 2000 mg/kg (16.9%) and 1200 mg/kg (16.8%). The treatment was started in 53.8% by day 5 of the illness and in 83.7% by day 7. The proportion of those with cardiac sequelae was higher among patients administered >2000 mg/kg or in those started on IVGG on day 9 of their illness or later. The possible reasons are (1) those who were more severely affected were treated with high-dose IVGG earlier; or (2) IVGG does not effectively prevent cardiac sequelae. We concluded that there is a risk of unfavorable effects with IVGG regarding cardiac sequelae when the IVGG dose is >2000 mg/kg or if IVGG is started on day 9 or later. We believe that only a randomized controlled trial, undertaken prospectively, can adequately address the question of the optimal use of IVGG.  相似文献   

10.
目的探讨10d内已退热的川崎病(KD)患儿应用丙种球蛋白(IVGG)治疗的必要性以及不同剂量IVGG治疗对KD预后的影响。方法研究对象为1999-10—2005-10山东省菏泽市立医院收治的56例KD患儿,所有患儿均为10d内退热后确诊且无冠脉病变。按IVGG治疗剂量分成3组,A组(11例)用1g/kg,B组(26例)用2g/kg,C组(19例)未使用,余治疗相同。对其冠状动脉损害(CAL)情况进行对比。结果病程14~21d时发生CAL例数:A组2例(18·18%),B组4例(15·38%),C组16例(84·21%),A、B组比较差异无显著性意义(P>0·05);A、B组与C组之间差异有非常显著性意义(P<0·01)。随访0·5年CAL例数:A组1例(9·09%),B组1例(3·85%),C组11例(57·89%),A、B组比较差异无显著性意义(P>0·05),而A、B组与C组之间差异有非常显著性意义(P<0·01)。结论10d内一经确诊的KD无论是否已退热均应给予IVGG治疗,对已退热且无冠脉损害的患儿应用总量1g/kg IVGG治疗可以达到满意的效果。  相似文献   

11.
Pure red cell aplasia (PRCA) is a rare disorder of erythrocyte production which is believed to have an autoimmune basis in most cases. Diamond-Blackfan anemia (DBA) is one type of congenital PRCA. Since PRCA has been reported to respond to intravenous γ-globulin (IVGG) therapy, we administered IVGG to a 2 year old girl with DBA resistant to corticosteroids and observed slight therapeutic effect.  相似文献   

12.
In a retrospective study, 121 children with Kawasaki disease (KD) were investigated to determine (i) the incidence of myocardial damage using the antimyosin antibody (AMA) titer; (ii) the differences in the electrocardiograms between the AMA-positive and -negative patients; and (iii) the effect of treatment with intravenous gamma globulin (IVGG) on the AMA. Comparisons were made with 117 normal children (controls). Patients with KD showed a significantly higher mean AMA titer and more patients were positive for AMA than the controls. The AMA titer in the KD group was not related to the presence of coronary artery lesions. Electrocardiograms obtained during the acute and the convalescent stage of KD revealed that patients positive for AMA had a significantly lower voltage of T wave in lead V6 at week four than at week two of illness, whereas patients negative for AMA showed no T wave change after week two. The group treated with IVGG showed a significantly lower AMA titer than that not given IVGG. These observations suggest that myocardial damage occurs in some patients with KD which is unrelated to the presence of coronary artery lesions and that the treatment with IVGG reduces the AMA titer in patients with KD.  相似文献   

13.
We compared the efficacy of oral administration of pentoxifylline (PTX) and intravenous infusions of gamma globulin (IVGG) combination therapy with that of IVGG in reducing the frequency of coronary-artery lesions (CAL) in children with Kawasaki disease (KD), in a randomized trial. All patients with KD received acetylsalicylic acid (30 mg/kg per day), until the 30th day, after the onset of fever, followed by daily acetylsalicylic acid at a dose of 3-5 mg/kg per day there-after, and intravenous IVGG, 200 mg/kg per day, for 5 consecutive days. In addition, patients randomly assigned to PTX and IVGG combination therapy groups received oral PTX at a dosage of 10 mg/kg per day (low-dose) or 20 mg/kg per day (high-dose), in three divided doses until the 30th day. Patients with KD were all free from CAL prior to treatment. We assessed the presence of CAL by two-dimensional echocardiography which was also done prior to treatment and then twice a week after hospital admission. We detected CAL in 3 of 18 patients (16.7%) in the IVGG therapy group, as compared with 2 of 18 patients (11.1%) in the low-dose PTX and IVGG combination therapy group. There were no significant differences between the two groups. In the next study, we detected CAL in 3 of 21 patients (14.3%) in the IVGG therapy group, as compared with none of 22 patients (0%) in the high-dose PTX and IVGG combination therapy group (2 = 6.4, P < 0.02). No adverse side-effects were observed in 79 patients with KD.  相似文献   

14.
OBJECTIVE: We evaluated the efficacy of intravenous gamma-globulin (IVGG) administration for children with Kawasaki disease to establish whether additional, more advanced therapy is needed in intractable cases. STUDY DESIGN: A total of 193 children with Kawasaki disease were studied retrospectively. Patients were admitted 3 to 7 days after the onset of the disease, and IVGG was administered. Laboratory measurements including white blood cell (WBC), neutrophil, and platelet counts and C-reactive protein (CRP) and albumin concentrations were determined before and 2 to 3 days after IVGG treatment. The progression of coronary artery lesions (CALs) was monitored by serial echocardiography until 30 days after treatment. RESULTS: Of 193 children, 24 (12.2 %) had CALs including transient dilatation. In contrast to the other measurements, the WBC count increased in 21 of 24 (87.5%) children with CALs after IVGG therapy. The patients with increased neutrophil count and CRP concentration after IVGG therapy also had CAL formation at a high rate (78.3% and 66.7%, respectively). Among children with normal coronary arteries, elevations of the WBC and neutrophil counts and CRP concentration were observed after IVGG therapy in only 3, 6, and 8 patients, respectively (specificity: 98.2%, 97.0%, and 95.3%, respectively). Furthermore, multiple logistic regression indicated that these variables were useful predictors of CALs in KD. CONCLUSION: Though the introduction of IVGG therapy has improved the prognosis of Kawasaki disease, approximately 10% of patients still develop CALs. The need for more aggressive therapy in IVGG-resistant cases can be recognized early by increases in the WBC and neutrophil counts and serum CRP concentration after IVGG administration.  相似文献   

15.
BACKGROUND: A fever lasting for at least 5 days is an essential characteristic of the original diagnostic criteria of Kawasaki disease (KD). However, it is not difficult for an experienced physician to confirm the diagnosis of KD before the fifth day of fever. The aim of this study is to investigate the effect of intravenous gamma globulin therapy (IVGG) in KD initiated before the fifth day of illness. METHODS: A total of 125 patients treated with IVGGwere divided into group A (IVGG was initiated before the fifth day of illness, n= 46) and group B (IVGG was initiated at the fifth day or after, n= 79). Patients' characteristics,laboratory findings, treatments and outcomes were compared between the groups. RESULTS: White blood cell count value, C-reactive protein and Harada's score showed no difference between the groups. A significantly higher average value of alanine aminotransferase(ALT) was observed in group A. Although the treatments were identical in both groups, the average duration of fever from the initial day of IVGG in group A was significantly longer than in group B. The incidence of aneurysm in group A was significantly higher than that in group B. Stepwise regression analysis using aneurysm as a dependent variable revealed that group A and ALT were significant. CONCLUSIONS: Patients diagnosed with KD before the fifth day of illness showed a poor response to IVGG. This observation might be related to high ALT values. Further examination concerning the modification of treatment in such patients is necessary.  相似文献   

16.
A multicenter randomized controlled study was carried out to assess the effectiveness of different, doses and kinds of γ-globulin in Kawasaki disease. Gamma globulin lowered the incidence of coronary artery abnormalities. The effect of γ-globulin was dose dependent. The intact type was more effective than the pepsin treated type. To establish the indications for γ-globulin, a study was made of patients who received neither γ-globulin nor indomethacin and who, within nine days of onset of illness, satisfied at least four of the following criteria: (1) WBC: more than 12,000/mm; (2) platelet count: less than 35×104γmm; (3) CRP: more than 3 +; (4) Hct: less than 35%; (5) albumin: less than 3.5 g/dl (6) age: 12 months or less; (7) male sex. This prospective study is continuing. Of 143 children, 73.4% received γ-globulin, and only two demonstrated small dilatations of the coronary arteries in children who did not receive γ-globulin. These guidelines seem satisfactory to establish the indications for γ-globulin in Kawasaki disease.  相似文献   

17.
A 14-month-old girl presented with a 4-d history of fever and generalized exanthema. Four characteristic symptoms of incomplete Kawasaki disease (KD) were present on admission (fever, rash, non-purulent conjunctival injection, oropharyngeal changes) and then followed by oedema of the hands and feet and mild plantar desquamation. The typical laboratory features of KD, such as elevated erythrocyte sedimentation rate, leukocytosis, thrombocytosis, and positive C-reactive protein were also seen. Ultrasound examination of the mediastinum revealed the presence of a lymph node, 30 mm in diameter, below the tracheal carina. Thoracic CT scan confirmed the mediastinal lymph node. The patient was treated with aspirin and intravenous γ -globulin. Ultrasound study of the mediastinum, which was carried out 6 weeks after hospital discharge, showed that the lymph node had disappeared. This case illustrates that lymph nodes other than cervical lymphadenopathy should be sought when the diagnosis of classical or atypical KD is suspected.  相似文献   

18.
The administration of iv gamma globulin (IVGG) for Kawasaki disease was investigated throughout Japan in 1993 by obtaining information from the pediatric departments of 2652 hospitals with more than 100 beds. A total of 1826 hospitals (68.9%) responded, reporting on 11 221 patients who were diagnosed during the survey period from January 1991 to December 1992. There were 8958 patients (79.8%) who received IVGG treatment. The most common treatment modality was 200mg/kg (29.6%), followed by 400mg/kg (18.7%) and 300mg/kg (12.9%), all for 5 days. The distributions of total dose were: 1000 mg/kg or less, 45.7%; 1001-1500 mg/kg, 27.3%; and over 1500 mg/kg, 23.8%. For all patients to whom IVGG was administered, treatment was started in 53.8% by day 5 of illness and in 86.1 % by day 7. The proportion of those with cardiac sequelae was higher in patients who were treated with IVGG, possibly due to the fact that those who were more severely affected were more likely to be treated with IVGG. Epidemiology, gamma globulin treatment, Japan, Kawasaki disease  相似文献   

19.
The effects of intravenous gammaglobulin (IVGG) on changes in the peripheral blood mononuclear cell subsets during acute Kawasaki disease (KD) were studied by a random selection trial of IVGG plus Aspirin (group G) compared to Aspirin alone (group A). Group G received IVGG with 200 mg/kg per day × 5 dose. The absolute counts of peripheral blood mononuclear cell subsets were assayed by a fluorescence-activated cell sorter using monoclonal antibodies of Leu series. Before therapy, patients in each treatment group had increased counts of CD14+ macrophage/monocytes compared to healthy childhood controls (P<0.01). After IVGG treatment, group G underwent a greater decrease in their CD14+ macrophage/monocyte counts (P<0.01) than group A. The changes of CD3+ T cells, Leu 7+ NK/K cells and CD19+ B cells in the peripheral blood mononuclear cell subsets with treatment in group G, were similar to those in group A. These results suggest the possibility that IVGG therapy is effective in KD by modulating macrophages/monocytes.  相似文献   

20.
Coronary angiography and right ventricular endomyocardial biopsy were performed in 36 children during convalescence (days of illness 23–86; mean = 41.5 days) following acute Kawasaki disease. Treatment of the acute stage was not randomized; it consisted of aspirin alone in 14 subjects (during the years 1980-88), and gammaglobulin and aspirin in 22 subjects (1984-91). The dosage of aspirin was 30 mg/kg orally during the acute febrile stage and 5–10 mg/kg orally after lysis of fever. The dosage of gammaglobulin was 200 mg/kg × 5 days in 19 patients, 200 mg/kg × 3 days in one patient, 200 mg/kg × 1 day + 400 mg/kg × 4 days in one patient and 200 mg/kg × 4 days + 400 mg/kg × 4 days in one patient. Among the 14 patients treated with aspirin alone, large coronary aneurysms were noted in four, moderate aneurysms in three and transient aneurysms in one. Among 22 patients treated with gammaglobulin, only five had aneurysms and these were transient. The histopathological (HP) score based on myocardial disarrangement, degeneration and hypertrophy; interstitial edema, large mononuclear cell infiltration and fibrosis; and endocardial abnormalities was higher in the aspirin group than in gammaglobulin group. Moderate to severe HP. changes were noted in five subjects in the aspirin group, while moderate HP changes were found in only two subjects in the gammaglobulin group. Gammaglobulin therapy not only reduced the incidence of the coronary arterial lesions but also reduced the severity of myocardial damage from moderate or severe to mild in Kawasaki disease.  相似文献   

设为首页 | 免责声明 | 关于勤云 | 加入收藏

Copyright©北京勤云科技发展有限公司  京ICP备09084417号