Usefulness and safety of anakinra in refractory Kawasaki disease complicated by coronary artery aneurysm

2018 ◽  
Vol 28 (5) ◽  
pp. 739-742 ◽  
Author(s):  
Marie-Paule Guillaume ◽  
Héloïse Reumaux ◽  
François Dubos

AbstractKawasaki disease is an acute self-limited vasculitis of unknown aetiology. The prognosis depends mainly on coronary damage. There is no consensus regarding optimal adjunctive therapeutics for refractory forms to treatment by intravenous immunoglobulins and corticosteroids. We report the case of an 18-month-old infant with refractory Kawasaki disease complicated by diffuse aneurysms of coronary arteries and successfully treated by anakinra with partial regression of coronary aneurysms

2018 ◽  
Vol 8 (3) ◽  
pp. 752-762 ◽  
Author(s):  
Renee Eigsti ◽  
Dennis J. Firchau ◽  
Marcus B. Nashelsky

Kawasaki disease is an acute vasculitis of unknown etiology that usually occurs in children less than five years old. Coronary artery aneurysm due to arteritis is the most common cardiac complication. Chronic aneurysms are associated with an increased risk of developing luminal thrombosis and ischemic myocardial injury. We present a case of an 18-year-old male with a history of Kawasaki disease complicated by chronic giant aneurysms of the right and left coronary arteries. Serial echocardiographic studies and treadmill electrocardiogram stress tests as recent as 1.5 years before death revealed excellent cardiac function by clinical criteria. The decedent sustained a witnessed collapse after ingesting 3,4-methylenedioxymethamphetamine (MDMA) and ethanol. He was pronounced dead in the emergency department after unsuccessful resuscitative efforts. Autopsy revealed large aneurysms of the three main epicardial coronary arteries with extensive foci of severe stenosis by intimal fibrosis and organizing thrombus. Microscopic examination revealed multifocal severe myocyte hypertrophy. There were remote microinfarcts in the anterior and posterior aspects of the left ventricle and a recent, healing microinfarct in the posterior aspect of the left ventricle. Toxicology examination of postmortem femoral blood revealed MDMA, ethanol, and amiodarone. This case illustrates the residual, lasting effects of cardiac disease due to Kawasaki disease and a potential complication in the setting of use of an illicit stimulant, MDMA, an amphetamine derivative that produces sympathetic activation and cardiovascular effects including tachycardia, vasoconstriction, dysrhythmias, and coronary artery spasm. Kawasaki disease-related abnormalities of the heart likely resulted in a lower threshold for developing a fatal cardiac dysrhythmia under the circumstance of stimulant use.


2020 ◽  
Vol 30 (4) ◽  
pp. 580-581
Author(s):  
Kader Muneer ◽  
Santhosh Narayanan

AbstractKawasaki disease is an acute febrile medium-vessel vasculitis affecting children. Though coronary artery aneurysm is a common complication, stenosis and occlusion are rare. Here, we report a case of Kawasaki disease with concomitant aneurysm and complete occlusion of different coronary arteries in the same patient.


2019 ◽  
Vol 29 (06) ◽  
pp. 828-832 ◽  
Author(s):  
Laxmi V. Ghimire ◽  
Fu-Sheng Chou ◽  
Narayan B. Mahotra ◽  
Sharan P. Sharma

AbstractBackground:Kawasaki disease is an acute vasculitis of childhood and is the leading cause of acquired heart disease in the developed countries.Methods:Data from hospital discharge records were obtained from the National Kids Inpatient Database for years 2009 and 2012. Hospitalisations by months, hospital regions, timing of admission, insurance types, and ethnicity were analysed. Length of stay and total charges were also analysed.Results:There were 10,486 cases of Kawasaki disease from 12,678,005 children hospitalisation. Kawasaki disease was more common between 0 and 5 years old, in male, and in Asian. The January–March quarter had the highest rate compared to the lowest in the July–September quarter (OR=1.62, p < 0.001). Admissions on the weekend had longer length of stay [4.1 days (95 % CI: 3.97–4.31)] as compared to admissions on a weekday [3.72 days (95 % CI: 3.64–3.80), p < 0.001]. Blacks had the longest length of stay and whites had the shortest [4.33 days (95 % CI: 4.12–4.54 days) versus 3.60 days (95 % CI: 3.48–3.72 days), p < 0.001]. Coronary artery aneurysm was identified in 2.7 % of all patients with Kawasaki disease. Children with coronary artery aneurysm were hospitalised longer and had higher hospital charge. Age, admission during weekend, and the presence of coronary artery aneurysm had significant effect on the length of stay.Conclusions:This report provides the most updated epidemiological information on Kawasaki disease hospitalisation. Age, admissions during weekend, and the presence of coronary artery aneurysm are significant contributors to the length of stay.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Kyu Yeun Kim ◽  
Mo Kyung Jung ◽  
Yoon-Sun Bae ◽  
Woohyuk Ji ◽  
Dongjik Shin ◽  
...  

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.


2020 ◽  
Vol 20 (1) ◽  
Author(s):  
I-Hsin Tai ◽  
Pei-Lin Wu ◽  
Mindy Ming-Huey Guo ◽  
Jessica Lee ◽  
Chi-Hsiang Chu ◽  
...  

2008 ◽  
Vol 13 (4) ◽  
pp. 242-250
Author(s):  
Peter N. Johnson ◽  
Robert J. Kuhn

A 3-month-old infant was transferred to our facility with persistent fever and concerns for septic shock. A 2-D echocardiogram revealed multiple coronary aneurysms and axillary and coronary artery thrombi, and a diagnosis of incomplete Kawasaki disease (KD) was established. Aggressive therapies including intravenous immunoglobulins, enoxaparin, abciximab, aspirin, and alteplase were used to decrease the size of the coronary aneurysms and inhibit further thrombus formation. After minimal change in the size of coronary aneurysms and in thrombus formation, clopidogrel was added. Approximately 2 weeks after initiation of these therapies, a decrease in the coronary aneurysm size was noted with no signs of thrombus. This case documents successful use of thrombolytic and combination anti-platelet agents (i.e., clopidogrel, abciximab, and aspirin) in an infant with KD and cardiovascular sequelae.


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