Variation in the management of Kawasaki disease

2019 ◽  
Vol 105 (10) ◽  
pp. 1004-1006 ◽  
Author(s):  
Audrey Dionne ◽  
David Burgner ◽  
Sarah De Ferranti ◽  
Davinder Singh-Grewal ◽  
Jane Newburger ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Initial Treatment ◽  
Coronary Aneurysm ◽  
Medical Specialty ◽  
Primary Treatment ◽  
Management Options ◽  
Necrosis Factor Alpha ◽  
Coronary Aneurysms ◽  
Factor Alpha ◽  
Collaborative Efforts

Intravenous immunoglobulin (IVIG) reduces coronary aneurysms in patients with Kawasaki disease (KD), but additional management options remain challenging, with no generalisable evidence-based recommendations. We performed a survey of 724 physicians from 73 countries to assess variation in practice. IVIG was the preferred initial treatment by 659 (91%) of respondents. Criteria for adjunctive primary treatment varied considerably and definitions of IVIG resistance varied markedly by geographical continent, Human Development Index tiers and medical specialty. A second dose of IVIG was used most often for patients with coronary aneurysm non-responsive to initial treatment (572, 79%), but corticosteroids (379, 52%) and tumour necrosis factor alpha inhibitors (208, 29%) were also frequently used. Our findings highlight the need for international collaborative efforts to optimise management of patients with KD worldwide.

Abstract 219: Long - Term Prognosis Of Patients With Kawasaki Disease Complicated By Significant Coronary Aneurysm (diameter ≥ 6 mm)

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Ji Seok Bang ◽  
Gi Beom Kim ◽  
Bo Sang Kwon ◽  
Eun Jung Bae ◽  
Chung Il Noh ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Coronary Artery Bypass ◽  
Artery Bypass ◽  
Coronary Aneurysm ◽  
Coronary Intervention ◽  
Surgical Interventions ◽  
Coronary Aneurysms ◽  
The Mean

Background: Some patients with Kawasaki disease (KD) develop large coronary aneurysms (diameter ≥ 6mm) and coronary stenosis, leading to ischemic heart disease. This study determined the long-term outcome for patients with Kawasaki disease complicated by significant coronary aneurysm. Methods and Results: From the database in Seoul National University Children’s Hospital between December 1986 and December 2013, medical records of 83 patients (61 men and 22 women) with large coronary aneurysms (diameter ≥ 6mm) were retrospectively reviewed. Information on patient demographics, catheter or surgical interventions, and most recent status was collected. From these data, we calculated the survival rate, cumulative coronary intervention rate, coronary artery bypass graft surgery rate. The mean age at onset was 4.28 ± 2.62 years, and the mean observational period was 13.2 ± 6.5 years. The maximum coronary artery internal diameter ranged from 6.1 to 25mm (median 9mm). Giant coronary aneurysm was 57 patients (68.7%) and large coronary aneurysm (6-8mm) was 26 patients (31.3%). Coronary aneurysms had progressed to coronary artery stenosis and/or complete occlusion in 42 patients (50.6%). The overall freedom from coronary intervention, coronary artery bypass surgery, or coronary thrombi was respectively 85.5%, 85.5%, and 90.3%. Catheter and surgical coronary interventions (median 1 intervention; range 1 to 5 interventions) were performed in 20 patients (24.1%) at 9 month to 18 years after onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8mm vs ≥8mm). Myocardial infarction occurred in 8 (8.98 %) patients. During this study period, 1 patient died and 98% survival rates was seen in our patients group. Conclusion: The long-term survival of patients with Kawasaki disease complicated by large coronary aneurysm is good even though a fourth of patients underwent multiple catheter or surgical interventions. Further research should focus on the indications for and effectiveness of percutaneous and surgical coronary interventions in large coronary aneurysm (≥ 6mm).

scholarly journals Combination Thrombolytic and Anti-Platelet Therapies in an Infant with Incomplete Kawasaki Disease and Coronary Aneurysms

2008 ◽  
Vol 13 (4) ◽  
pp. 242-250
Author(s):  
Peter N. Johnson ◽  
Robert J. Kuhn
Keyword(s):  
Septic Shock ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Thrombus Formation ◽  
Coronary Aneurysm ◽  
Intravenous Immunoglobulins ◽  
Minimal Change ◽  
Incomplete Kawasaki Disease ◽  
Persistent Fever ◽  
Coronary Aneurysms

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.

Thrombolysis in the treatment of patients with Kawasaki disease

1993 ◽  
Vol 3 (3) ◽  
pp. 207-215 ◽  
Author(s):  
Atsuko Suzuki ◽  
Kamiya Tetsuro ◽  
Yasuo Ono ◽  
Yoshihisa Kinoshita
Keyword(s):  
Kawasaki Disease ◽  
Myocardial Ischemia ◽  
Thrombolytic Therapy ◽  
Coronary Aneurysm ◽  
Coronary Arteriography ◽  
The Other ◽  
Myocardial Imaging ◽  
Coronary Aneurysms ◽  
Large Coronary Arteries

AbstractThrombolysis was used in 13 procedures to treat eight patients with massive thrombosis of coronary aneurysms due to Kawasaki disease. The patients have now been followed for periods ranging from 2.5 years to 7.4 years subsequent to treatment. The procedures included intracoronary or intravenous infusion of urokinase and intravenous administration of tissue plasminogen activator. Thallium myocardial imaging, coronary arteriography and echocardiography were used for evaluation of the efficacy of the therapy and for follow-up studies. The latest time of treatment was 17 months after the onset of the disease. No new formation of massive thrombus was observed in any of the aneurysms. In five patients, eight of 13 procedures were judged to be successful. In the four patients with symptoms of myocardial ischemia, thrombolysis was undertaken immediately after the onset of symptoms. In the other patient, thrombolysis was attempted 36 days after the onset and produced remarkable improvement in the degree of ischemia. In the other four patients with no symptoms of ischemia, the therapy was undertaken when thrombus was detected in a coronary aneurysm. Aortocoronary bypass surgery was undertaken subsequent to the thrombolytic therapy in three patients, but proved satisfactory in only one patient. At follow-up, five patients have shown improvement in the degree of myocardial ischemia after treatment. Our results suggest that careful observation is needed to detect formations of thrombus in large coronary arteries for at least 1½ years after the onset of Kawasaki disease. Even if repeated thrombolytic therapy is required, it seems to be effective in checking the progression of ischemic heart disease.

“Giant within a giant”: a case of typical Kawasaki disease with a giant coronary aneurysm and a large coronary thrombus – a rare case report

2017 ◽  
Vol 28 (1) ◽  
pp. 147-149
Author(s):  
Sandeep Rajasekharan ◽  
Suneesh Kalliath ◽  
Sajeev C. Govindan
Keyword(s):  
Case Report ◽  
Kawasaki Disease ◽  
Rare Case ◽  
Coronary Aneurysm ◽  
Giant Coronary Aneurysm ◽  
Coronary Thrombus ◽  
Coronary Aneurysms ◽  
Arterial Thrombus ◽  
Rare Case Report

AbstractKawasaki disease is a febrile vasculitis affecting young children, which may lead to coronary aneurysms. Echocardiography, although sensitive in detecting coronary aneurysms, has a limited role in diagnosing coronary thrombus. Here we report the case of a 10-year-old boy who presented with typical features of Kawasaki disease with giant coronary aneurysms. His follow-up echocardiogram revealed coronary arterial thrombus.

Extremely refractory Kawasaki disease with disseminated intravascular coagulation

2017 ◽  
Vol 27 (5) ◽  
pp. 1004-1007
Author(s):  
Young Kwon Koh ◽  
Jae Hee Lee ◽  
Yeong Bong Park
Keyword(s):  
Kawasaki Disease ◽  
Disseminated Intravascular Coagulation ◽  
Rare Complication ◽  
Coronary Aneurysm ◽  
Pulse Therapy ◽  
Fresh Frozen Plasma ◽  
Intravascular Coagulation ◽  
Coronary Aneurysms ◽  
Fresh Frozen ◽  
Intravenous Methylprednisolone Pulse

AbstractDisseminated intravascular coagulation is a rare complication of Kawasaki disease and appears in <0.1% of Kawasaki disease patients. We report a case of refractory Kawasaki disease complicated with disseminated intravascular coagulation and giant coronary aneurysm. A 5-month-old boy presented with Kawasaki disease with coagulopathy. Although the coagulopathy improved after fresh-frozen plasma and antithrombin-III administration, the fever persisted despite two rounds of intravenous immunoglobulin, along with intravenous methylprednisolone pulse therapy and infliximab administration. Despite all efforts to treatment, the patient had giant coronary aneurysms and died suddenly.

Primary adjunctive corticosteroid therapy is associated with improved outcomes for patients with Kawasaki disease with coronary artery aneurysms at diagnosis

2020 ◽  
pp. archdischild-2020-319810 ◽  
Author(s):  
Kevin G Friedman ◽  
Kimberlee Gauvreau ◽  
Annette Baker ◽  
Mary Beth Son ◽  
Robert Sundel ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Corticosteroid Therapy ◽  
Initial Treatment ◽  
Treatment Resistance ◽  
Primary Treatment ◽  
Z Score ◽  
Coronary Artery Aneurysms ◽  
Reactive Protein ◽  
Randomised Controlled

ObjectivePatients with Kawasaki disease (KD) with coronary artery enlargement at diagnosis are at the highest risk for persistent coronary artery aneurysms (CAAs) and may benefit from primary adjunctive anti-inflammatory therapy beyond intravenous immunoglobulin (IVIG). We evaluate the effect of primary adjunctive corticosteroid therapy on outcomes in patients with CAA at diagnosis.DesignSingle-centre, retrospective review.PatientsPatients with KD diagnosed within 10 days of fever onset and with baseline CA z-score ≥2.5.InterventionsPrimary treatment with IVIG (n=162) versus IVIG plus corticosteroids (n=48).Main outcome measuresTreatment resistance (persistent fever >36 hours after initial treatment), CAA regression rate.ResultsOf the 92 patients with KD who received corticosteroids at our institution from 2012 to 2019, 48 met the inclusion criteria for primary adjunctive therapy. The corticosteroid group was younger and had larger baseline CAAs compared with historical controls. Demographics and laboratory values were otherwise similar between groups. The corticosteroid group had a less treatment resistance (4% vs 30%, p=0.003) and a greater improvement in C reactive protein. After adjusting for baseline CA z-score, age and baseline bilateral versus unilateral CAA, the corticosteroid group had a higher odds of (OR 2.77 (1.04, 7.42), p=0.042) and a shorter time to CAA regression (HR 1.94 (1.27, 2.96), p=0.002).ConclusionPrimary adjunctive corticosteroid therapy is associated with decreased initial treatment resistance, greater improvement in inflammatory markers and higher likelihood of CAA regression in patients who have CAA at diagnosis. Multi-centre, randomised controlled trials are needed to confirm the benefits of corticosteroids in patients with CAA at diagnosis and to compare corticosteroids with other adjunctive therapies.

A girl with extremely refractory Kawasaki disease: an instructive case with unusual course and outcome

2011 ◽  
Vol 22 (4) ◽  
pp. 461-465 ◽  
Author(s):  
Kossiva Lydia ◽  
Karanassios Evangelos ◽  
Papadopoulos George ◽  
Karavanaki Kyriaki
Keyword(s):  
Kawasaki Disease ◽  
Standard Treatment ◽  
Tumour Necrosis Factor Alpha ◽  
Unknown Aetiology ◽  
Therapeutic Approaches ◽  
Coronary Artery Aneurysms ◽  
Necrosis Factor Alpha ◽  
Factor Alpha ◽  
Instructive Case ◽  
Necrosis Factor

AbstractKawasaki disease constitutes an acute febrile vasculitis of unknown aetiology. It is considered the most common cause of acquired cardiac failure in children. Although standard treatment comprises intravenous immunoglobulin and aspirin, some children exhibit refractory disease, necessitating the use of alternative therapies such as corticosteroids and anti-tumour necrosis factor-alpha. For these cases, few controlled data are available. This report focuses on an extremely refractory classical Kawasaki disease with coronary artery aneurysms and ongoing inflammation. We discuss the therapeutic approaches and the potential pitfalls undertaken, which led to an unfavourable clinical outcome.

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