scholarly journals Rare Refractory Kawasaki Disease in an Adolescent Boy With Cardiac and Diffuse Coronary Artery Involvement

Medicina ◽  
2013 ◽  
Vol 49 (7) ◽  
pp. 53
Author(s):  
Rima Šileikienė ◽  
Jolanta Kudzytė ◽  
Antanas Jankauskas ◽  
Liutauras Labanauskas ◽  
Vilma Rakauskienė ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Elderly Age ◽  
Coronary Artery Aneurysms ◽  
Cardiovascular Damage ◽  
Coronary Artery Involvement ◽  
Increased Risk ◽  
Adolescents And Adults ◽  
Appropriate Therapy ◽  
Infants And Young Children

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.

Abstract O.60: Kawasaki Disease Complicated by Coronary Artery Aneurysms: Mortality and 40-year Outcomes

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Cedric Manlhiot ◽  
Andrew M Crean ◽  
Nigel Fernandopulle ◽  
Brendan Lew ◽  
Tanveer H Collins ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
General Population ◽  
Life Expectancy ◽  
Myocardial Infarct ◽  
Coronary Artery Aneurysms ◽  
Cumulative Mortality ◽  
Coronary Artery Involvement ◽  
Coronary Artery Dilatation

Background: Long-term outcomes and life expectancy for children with a previous history of Kawasaki disease (KD), particularly those with coronary artery aneurysms (CAA), remain to be determined. Methods: An inception cohort of all patients with KD assessed at The Hospital for Sick Children in Toronto between 1978 and 2013 was assembled. Patient outcomes were obtained throughout their pediatric and adult clinical follow-up as long as available. Prevalence of outcomes over time was modelled with Kaplan-Meier survival curves. Life tables from Statistics Canada were used to obtain age/gender specific cumulative mortality for the general population. Results: The cohort included 2,623 KD patients, of whom 410 (16%) had coronary artery involvement (215 dilatation, 57 non-giant CAA and 138 giant CAA). Average follow-up for patients with coronary artery involvement was 6.7 years (13.3 years for giant CAA); 57 and 34 patients had at least 15 and 25 years of follow-up, respectively. No patients with coronary artery dilatation or non-giant CAA had revascularization or a myocardial infarct. Freedom from revascularization (14 events) for patients with giant CAA was 90±6%, 87±7% and 80±13% at 10, 20 and 40 years of follow-up. Freedom from myocardial infarct (11 events) was 94±4%, 92±5% and 89±7% at 5, 20 and 40 years. For patients without coronary artery involvement, 3 (0.1%) deaths were recorded, one secondary to complications of macrophage activation syndrome during the acute phase of KD and 2 from cancer. No deaths were noted for patients with coronary artery dilatation or non-giant CAA, although clinical follow-up was more limited. For patients with giant CAA, 3 deaths (2.2%) were noted, 2 related to CAA complications and 1 from non-medical cause. Cumulative mortality for patients with giant CAA was 1.5% at 10 years of age (expected mortality 0.7%, HR: 2.2 (0.3-11.5), p=0.08) and 3.1% at 40 years of age (expected 2.3%, HR: 1.3 (0.4-4.0), p=0.37). Conclusions: Despite risks of myocardial infarction and revascularization, patients with giant CAA had life-expectancy similar to that of the general population up to the fourth decade of life. Additional follow-up will be necessary to determine if these trends continue into later decades.

Abstract O.58: Medium-term Outcomes of Coronary Artery Aneurysms after Kawasaki Disease: A Study from the North American Kawasaki Disease Registry

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Brian W McCrindle ◽  
Cedric Manlhiot ◽  
Kristen Sexson ◽  
Pei-Ni Jone ◽  
Mathew Mathew ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
North American ◽  
Z Score ◽  
Disease Registry ◽  
Coronary Artery Aneurysms ◽  
Luminal Diameter ◽  
The North ◽  
Increased Risk ◽  
Over Time

Background: One of the main impediments to conceiving and planning studies in children with coronary artery aneurysms (CAA) after Kawasaki disease (KD) is the lack of normative data regarding the prevalence of outcomes over time and risk factors. Methods: The North American Kawasaki Disease Registry was used to determine the prevalence of multiple clinically important outcomes of CAA after KD. All analyses were stratified by severity of CAA (small CAA with z-score = 2.5-5, medium with z-score = 5-10 and giant with z-score >10). All analyses were performed using non-parametric survival analysis. Results: n=621 patients submitted to the Registry had complete follow-up data and were included in the analysis (280 [45%] small CAA, 139 [22%] medium and 202 [33%] giant). Time-related freedom from multiple outcomes stratified by type of CAA are reported in the Table. Reduction in z-scores was strongly associated with the initial size of the lesion, with smaller lesions being more likely to decrease to a normal dimension over time. Thrombosis and stenosis were infrequent in patients without giant CAA. For those patients with giant CAA, the risk of thrombosis, myocardial infarction, angiographically-confirmed stenosis and revascularization was substantial and persisted up to 10 years after diagnosis. In addition to larger luminal diameter, other factors associated with increased risk of adverse outcomes included larger CAA longitudinal area and complex CAA (vs. isolated lesions). Conclusions: Only patients with giant CAA are at substantial risk of adverse clinical outcomes; future trials of pharmacological therapy targeting thrombosis and stenosis risk should focus on these patients.

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
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Initial Treatment ◽  
Ivig Treatment ◽  
Cardiac Complications ◽  
Primary Therapy ◽  
Coronary Artery Aneurysms ◽  
Steroid Group ◽  
Increased Risk

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.

Abstract 34: Genome-wide Association Study Identified New Susceptibility Loci To Coronary Artery Aneurysm-related In Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Kyu Yeun Kim ◽  
Mo Kyung Jung ◽  
Yoon-Sun Bae ◽  
Woohyuk Ji ◽  
Dongjik Shin ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Association Study ◽  
Genome Wide Association Study ◽  
Coronary Artery Aneurysm ◽  
Artery Aneurysm ◽  
Genome Wide Association ◽  
Korean Children ◽  
Genome Wide ◽  
Increased Risk

Kawasaki disease (KD) is an acute systemic vasculitis syndrome that predominantly affects children younger than 5 years of age, and may causes serious, sometimes life-threatening, cardiac sequela associated with coronary artery aneurysm (CAA). To identify genetic variants that confers a highly increased risk of coronary artery aneurysm-related in Kawasaki disease. In this study, we carried out genome-wide association study (GWAS) in a Korean children population including 102 CAA-related KD cases and 126 controls. Fifteen genetic loci were found to be significantly correlated with KD risk (P<1.0X10(-7)). Our case-control study revealed that rs4236089 C allele in chloride intracellular channel 5 (CLIC5) gene at 6p21.1 was significantly associated with KD patients with CAA (odds ratio (OR)=4.6, P=7.53X10(-7)). These findings suggest that the CLIC5 gene may play a crucial role in CAA development pathway of KD.

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