Abstract 125: Steroid Treatment in the Acute Phase of Kawasaki Disease in Mexican Children. Are they Useful?

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Miguel Garcia-Dominguez ◽  
Luis M Garrido-Garcia

Background: Intravenous immunoglobulin (IVIG) and aspirin is the standard initial therapy in the treatment of Kawasaki disease (KD), which is proven to decrease the incidence of coronary artery aneurysms from 25% to less than 5%. There is increasing evidence for steroid therapy as adjunctive primary therapy with IVIG especially in those patients who are at increased risk of coronary artery aneurysms and in patients with risk of IVIG resistance. However, clinical trials evaluating the use of corticosteroids plus IVIG have produced confusing results. Objective: To evaluate the clinical efficacy and safety of steroids plus intravenous immunoglobulin (IVIG) combination therapy (IVIG+S) for the initial treatment of patients with KD to prevent coronary artery aneurysms (CAA) compared with the standard treatment with intravenous immunoglobulin plus aspirin (IVIG+A) in a Children’s Hospital in Mexico City. Material and Methods: An observational, comparative, retrospective and case-control study of all patients treated with IVIG for KD in our Institution from August 1995 to May 2014. The clinical presentation, laboratory results and coronary artery abnormalities in the IVIG+S and the IVIG+A groups were analyzed and compared. Results: We studied 295 patients with KD treated with IVIG, 136 (46.1%) received IVIG+A treatment and 159 (53.9%) received IVIG+S treatment. We didn’t found adverse reactions in the patients treated with steroids. The IVIG+S group were older 43.25 ± 43.04 than the non-steroid group 32.07 ± 24.51 (p < 0.008). Steroids were commonly use in incomplete cases (p < 0.059) and in patients with cardiac complications at diagnosis: pericardial effusion (p < 0.056) and pericarditis (p < 0.013). The steroid group has slightly more days of fever after the IVIG treatment 1.27 ± 1.51 days vs. 0.93 ± 0.924 days (p < 0.028). We found no difference in the development of CAA in both groups. (p = 0.221) Conclusions: There were no differences in the development of CAA with the use of steroids in the initial treatment of KD. Nevertheless steroids were used more commonly in incomplete forms of KD and in more severely affected patients, which could reflect the lack of difference in the development of CAA in both groups.

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Ju Young Kim ◽  
Hyun Jung Kim

Kawasaki disease is an acute febrile illness that usually occurs in children younger than 5 years of age. The use of intravenous immunoglobulin (IVIG) within the first 10 days of illness has been shown to reduce the incidence of coronary artery aneurysms significantly. The relative roles of repeated doses of intravenous immunoglobulin (IVIG) are controversial in refractory Kawasaki disease (KD). Most experts recommend the second retreatment with IVIG, 2 g/kg in refractory KD. However, the dose-response effect of the third or fourth IVIG was uncertain. Although there have been a significant number of reports on new therapeutic options for refractory KD, such as steroid, infliximab, methotrexate, and other immunosuppressants, their effectiveness in reducing the prevalence of coronary artery aneurysms was unproven. We present here KD patient with small coronary artery aneurysm who is resistant to the third IVIG and steroid pulse therapy but showed improvement immediately after the infusion of the 4th IVIG on fever day 18.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
lijian xie ◽  
Cuizhen Zhou ◽  
Renjian Wang ◽  
Tingting Xiao ◽  
Jie Shen ◽  
...  

Introduction: The incidence of Kawasaki disease (KD) in China is increasing for years. The current coronary artery lesion (CAL) incidence is 5-10% in KD with intravenous immunoglobulin (IVIG) treatment. And the 10-20% KD patients still exhibit IVIG resistance. However, little clinical evidence on the occurrence of either CAL or IVIG resistance for big KD sample study in China during the past decade. Objective: In order to find clinical risk factors of CAL and IVIG resistance of KD in China. Methods: We retrospectively analyzed the clinical manifestations, laboratory results, treatment and complications of cardiac vascular of 602 KD cases from 2007 to 2012 admitted at Shanghai Children’s Hospital. The SAS 9.2 edition was used for statistical analysis. The mean ± standard deviation or the median were used for measurements. Case numbers and percentages were used for the count number. The t-test and the Mann-Whitney test were both used for mean comparisons. Single factor and multi-factor logistic regression analyses were used to analyze the risk factors. Results: 1. The KD gender male to female ratio was 1.85: 1. The KD median age was 2.0 years old (one month to 11.7 years old). 20.1% cases (121 of 602) exhibited CAL. There was no difference of CAL incidence between the gender (p=0.09). 2. The incidence of bright red cracked lips (p=0.001), peeling of the skin of the toes (p=0.021) and perianal skin peeling (p=0.031) are less in group with CAL. 3. Among the 602 cases, there were 525 cases that were sensitive to IVIG therapy. 100 of those cases had CAL with an incidence of 19.1%. Among the 26 IVIG resistance cases, there were 9 cases with CAL with an incidence of 34.6%, which was higher than the IVIG sensitive group (p=0.05). 4. ESR (p=0.014), CRP (p=0.017), PLT (p=0.003) and Hb (p=0.032) were much higher in the IVIG resistance group than the IVIG sensitive group, even though the IVIG resistance group started the IVIG treatment earlier (p=0.003). 5. Logistic regression analysis was conducted to show that GPT≥80IU/L was the independent risk factor of IVIG resistance, risk ratio was 2.945 (p=0.012) . Conclusion: This research suggests that risk factors of clinical evidence for IVIG resistance and CAL in KD.


2020 ◽  
Vol 8 ◽  
Author(s):  
Fan Yan ◽  
Huayong Zhang ◽  
Ruihua Xiong ◽  
Xingfeng Cheng ◽  
Yang Chen ◽  
...  

Background: In the latest 2017 American Heart Association guidelines for Kawasaki disease (KD), there are no recommendations regarding the early administration of intravenous immunoglobulin (IVIG). Therefore, the purpose of this systematic review and meta-analysis was to investigate the effects of early IVIG therapy on KD.Methods: We searched databases including the PubMed, Medline, the Cochrane Library, and the Clinicaltrials.gov website until July 2019.Results: Fourteen studies involving a total of 70,396 patients were included. Early treatment with IVIG can lead to an increased risk of IVIG unresponsiveness [OR 2.24; 95% CI (1.76, 2.84); P = 0.000]. In contrast to the studies performed in Japan [OR 1.27; 95% CI (0.98, 1.64); P = 0.074] that found no significant difference in coronary artery lesions (CAL) development, studies conducted in China [OR 0.73; 95% CI (0.66, 0.80); P = 0.000] and the United States [OR 0.50; 95% CI (0.38, 0.66); P = 0.000] showed a reduced risk in the occurrence of CAL with early IVIG treatment.Conclusions: At present, the evidence does not support the treatment with IVIG in the early stage of the onset of KD. But, early IVIG treatment could be a protective factor against the development of CAL, which needs to be further clarified.


2015 ◽  
Vol 100 (4) ◽  
pp. 366-368 ◽  
Author(s):  
Sarah Davies ◽  
Natalina Sutton ◽  
Sarah Blackstock ◽  
Stuart Gormley ◽  
Clive J Hoggart ◽  
...  

The Kobayashi score (KS) predicts intravenous immunoglobulin (IVIG) resistance in Japanese children with Kawasaki disease (KD) and has been used to select patients for early corticosteroid treatment. We tested the ability of the KS to predict IVIG resistance and coronary artery abnormalities (CAA) in 78 children treated for KD in our UK centre. 19/59 children were IVIG non-responsive. This was not predicted by a high KS (11/19 IVIG non-responders, compared with 26/40 responders, had a score ≥4; p=0.77). CAA were not predicted by KS (12/20 children with CAA vs 25/39 with normal echo had a score ≥4; p=0.78). Low albumin and haemoglobin, and high C-reactive protein were significantly associated with CAA. The KS does not predict IVIG resistance or CAA in our population. This highlights the need for biomarkers to identify children at increased risk of CAA, and to select patients for anti-inflammatory treatment in addition to IVIG.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Mallory L Downie ◽  
Cedric Manlhiot ◽  
Tanveer H Collins ◽  
Nita Chahal ◽  
Rae S Yeung ◽  
...  

Introduction: While the risk is reduced, patients may develop coronary artery aneurysms (CAA) after Kawasaki disease (KD) despite receiving intravenous immunoglobulin (IVIG) within 10 days of onset of symptoms. Risk factors for CAA may differ compared to those patients with delayed or no treatment. Methods: Patients diagnosed with KD between 1990 and 2013 were included. Patients with maximum coronary artery z-scores >5 were classified as having CAA. Separate multivariable regression models were used to determine factors associated with CAA for those with vs. without prompt treatment. Results: Of 1,358 patients included, 83% were treated with IVIG within 10 days and 5.4% developed CAA. Patients who had delayed (>10 days) or no IVIG treatment were at increased odds of developing CAA (OR: 3.1, p<0.001). From 1990-2013, the proportion of patients treated promptly increased (OR: 1.05/year, p=0.006) while the total duration of fever decreased (EST: -0.10 (0.03) days/year, p=0.001). These trends were associated with a shift such that a greater proportion of the patients who developed CAA actually had been treated promptly (from <25% in 1990 to >70% in 2013, OR: 1.1/year, p=0.01). For patients with prompt treatment with IVIG, factors associated with increased odds of CAA were: longer duration of fever prior to treatment (OR: 1.2/day, p=0.04), age <1 year old (OR 3.9, p=0.001), higher pre-IVIG white blood cell count (OR: 1.05/x10 9 /L, p=0.007), lower hemoglobin (OR: 1.4/g/L, p=0.004) and non-response to the initial IVIG treatment (OR: 2.5, p<0.001). For patients with delayed or no treatment, factors associated with increased odds of CAA were: males (OR: 5.4, p=0.009), age <1 year old (OR: 29.9, p<0.001), lower red blood cell count (OR: 2.5/-0.5 x10 12 /L, p=0.01) and higher platelet count at diagnosis (OR: 1.4/100x10 12 /L, p=0.001). Additionally, delayed treatment with IVIG did not reduce the risk of CAA (OR: 1.9, p=0.28), and total duration of fever was not associated with CAA for this group (OR: 1.04/day, p=0.16). . Conclusions: Factors associated with the development of CAA are generally similar for those treated promptly vs. those with delayed or no treatment. For those with delayed diagnosis, treatment with IVIG does not appear to be effective to prevent CAA.


Medicina ◽  
2013 ◽  
Vol 49 (7) ◽  
pp. 53
Author(s):  
Rima Šileikienė ◽  
Jolanta Kudzytė ◽  
Antanas Jankauskas ◽  
Liutauras Labanauskas ◽  
Vilma Rakauskienė ◽  
...  

Kawasaki disease is an acute multisystemic vasculitis occurring predominantly in infants and young children and rarely in adolescents and adults. At elderly age, Kawasaki disease may remain unrecognized with a subsequent delay in appropriate therapy and an increased risk of coronary artery aneurysms. We report a case of intravenous immunoglobulin- and aspirin-resistant Kawasaki disease and severe cardiovascular damage in an adolescent boy. The article discusses major issues associated with the management of refractory Kawasaki disease.


2018 ◽  
Vol 23 (suppl_1) ◽  
pp. e29-e30
Author(s):  
Steffany Poupart ◽  
Audrey Dionne ◽  
Cathie-Kim Le ◽  
Léamarie Meloche-Dumas ◽  
Jean Turgeon ◽  
...  

Abstract BACKGROUND Kawasaki disease (KD) is a paediatric systemic vasculitis that can be associated with concomitant viral or bacterial infections. Patients with persistent or recurrent fever 36 hours after the end of intravenous immunoglobulin (IVIG) are considered to be resistant to treatment and are at increased risk for coronary complications. OBJECTIVES However, it is unknown how concomitant infection influences the response to IVIG treatment. The aim of this study was to determine the impact of concurrent infection on the prevalence of IVIG resistance and coronary outcome. DESIGN/METHODS Retrospective study of 154 children (mean age at diagnosis: 3.4 ± 2.8 years) diagnosed with KD, between 2008–2016 in a tertiary paediatric university hospital, of which 59 (38%) had concomitant infection. RESULTS Delay in diagnosis (>10 days of fever) was similar between patients with and without concomitant infection (7% vs 7%, p=0.89). Patients with concomitant infection were more likely to have fever 48 hours after initial treatment (36% vs 20%, p=0.05) and to be treated with a second dose of IVIG (33% vs 18%, p=0.04). Patients with infection had higher C-reactive protein at the time of diagnosis (148 vs 112 mg/L, p=0.04), which persisted after IVIG administration (111 vs 59 mg/L at 48 hours, p=0.003). However, there was no statistically significant difference in the prevalence of coronary artery (CA) complications (coronary artery Z-score > 2.5) between patients with and without concomitant infection (36% vs 39%, p=0.68). CONCLUSION Children with KD and concomitant infection are more likely to have persistent fever and elevated inflammatory markers after treatment requiring a second dose of IVIG. Nevertheless, this is not associated with an increased risk of CA complications. Larger scale studies are needed to help distinguish IVIG resistance from infection in children with persistent fever and guide management of this population. Table/Chart or graphic upload


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