Abstract 200: Afebrile Kawasaki Disease Beyond Diagnostic Guideline Evolving Coronary Artery Complication

Kawasaki disease (KD) is diagnosed with clinical features. Presence of at least five days of fever is major criteria. Herein we report a 7 month -old boy diagnosed as afebrile kawasaki disease who initially presented with inflammation at the Bacille Calmette-Guerin (BCG) inoculation site (BCGitis) with multiple erythematous papular rash, followed by desquamation of finger tips at 11 day of illness. Laboratory test showed elevated ALT (110 U/L) and otherwise showed no specific finding. Clinical feature disappeared spontaneously except BCGitis. The patient did not fulfill the diagnostic criteria but progressive coronary arterial dilatation was noticed. Left coronary artery (LMCA) was dilated from Z-score 1.6 (3 day of illness) to 2.8 (11 day of illness). After treated with intravenous immunoglobulin (2g/kg), BCGitis disappeared and follow up echocardiogram showed normalized LMCA lesion (Z-score 1.0 at 17 day of illness). BCGitis was considered to be pathognomonic feature of this patient. Diagnostic algorithm and guidelines are useful tool for the incomplete KD patients but clinicians should also be cautious for the patient with BCGitis even they are excluded by diagnostic guideline.

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Z-score is a possible predictor of the risk of coronary artery lesion development in patients with Kawasaki disease in Japan

European Journal of Pediatrics ◽

10.1007/s00431-021-04006-1 ◽

2021 ◽

Author(s):

Takayuki Suzuki ◽

Nobuyuki Kakimoto ◽

Tomoya Tsuchihashi ◽

Tomohiro Suenaga ◽

Takashi Takeuchi ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Coronary Artery Lesion ◽

Z Score ◽

Lesion Development

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Sequential follow-up results of catheter invention for coronary artery lesions after Kawasaki disease: quantitative coronary artery. Angiography and intravascular ultrasound imaging

ACC Current Journal Review ◽

10.1016/s1062-1458(02)01073-5 ◽

2003 ◽

Vol 12 (1) ◽

pp. 91

Author(s):

M. Ishii ◽

T. Ueno ◽

H. Ikeda

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Intravascular Ultrasound ◽

Ultrasound Imaging ◽

Coronary Artery Lesions ◽

Intravascular Ultrasound Imaging ◽

Coronary Artery Angiography

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Abstract 221: Use of Statins in Patients with Coronary Artery Aneurysms: A Pilot Study from the North American Kawasaki Disease Registry

Circulation ◽

10.1161/circ.131.suppl_2.221 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

Michael Khoury ◽

Michael A Portman ◽

Cedric Manlhiot ◽

Anne Fournier ◽

Rejane F Dillenburg ◽

...

Keyword(s):

Coronary Artery ◽

Pilot Study ◽

Kawasaki Disease ◽

Linear Regression ◽

North American ◽

Z Score ◽

Disease Registry ◽

Coronary Artery Aneurysms ◽

The North ◽

Significant Difference

Background: Statins have been considered as therapy for children with coronary artery aneurysms (CAA) after Kawasaki disease (KD), due to potential beneficial pleiotropic effects which might influence chronic vascular processes and inflammation. Methods: The North American Kawasaki Disease Registry was queried to identify patients who have received statins in the first 6 months following the convalescent phase of KD. Each identified patient was matched by age, gender and CAA z score to 3 patients who were statin-naïve (controls). Linear regression models adjusted for repeated measures and maximum coronary involvement were used to determine an association of statin use with longitudinal changes in coronary artery diameter z-score. Kaplan-Meier analysis was used to compare freedom from angiographically-confirmed stenosis or interventions. Results: Of 29 patients with KD and CAA (maximum coronary artery z-score >10) who received statins at any time (of n=621, 5%), 10 (9 males) patients were started within 6 months of the acute KD episode. The mean age at KD was 6.3±3.4 years (5.4±3.5 for controls, p=0.57). Mean maximum CAA z-score was 36±14 (vs. 29±16, p=0.20); 90% of statin patients and 87% of matched controls had CAAs in 3 or more branches. Linear regression analysis of 442 serial echocardiograms showed that maximum CAA z-score decreased by -1.5 (95%CI: -2.7; -0.4) SD/year (p=0.008) for control patients compared to -2.9 (95%CI: -4.4; -1.4) SD/year (p<0.001) for statin treated patients. The difference between the rate of change of CAA z-score for statin vs. control patients did not reach statistical significance (controls vs. statins: +1.4 SD/year, 95%CI: -0.6; +3.4, p=0.18). n=7 patients (3 on statin, 4 controls) developed stenosis or had revascularization, with no significant difference between groups (HR for statin group: 2.2 (0.4-11.4), p=0.41). Conclusions: This underpowered pilot study suggests that equipoise likely exists with regards to statin therapy in children with KD and CAA, and that a formal registry-nested trial might be considered.

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Abstract 28: African American Race is Not Protective Against Coronary Artery Involvement in Kawasaki Disease

Circulation ◽

10.1161/circ.131.suppl_2.28 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

Emily P Williams ◽

Michael S Kelleman ◽

William T Mahle

Keyword(s):

African American ◽

Coronary Artery ◽

Kawasaki Disease ◽

Reference Group ◽

Artery Aneurysm ◽

Z Score ◽

Strict Adherence ◽

Hispanic Patients ◽

American Children ◽

African American Race

It has been previously reported that African American race may be protective against coronary artery aneurysm development in Kawasaki Disease (KD). We aimed to test this with our own cohort of KD patients from a large pediatric cardiology practice. Data from 250 subjects diagnosed with KD and followed as outpatients with surveillance echocardiography over a two-year period were analyzed. Twelve patients were excluded due to incomplete records or an unconfirmed diagnosis. Race designated by parent was recorded. Charts were reviewed for any coronary involvement (ectasia or aneurysm) and coronary Z-score greater than 2.5 at the time of diagnosis and at subsequent follow-up visits. Odds rations were calculated comparing each racial group to others for any coronary involvement and for coronary Z-score > 2.5. Of 238 included patients, 44.5% were African American, 37.4% were non-Hispanic white, 10.5% were Hispanic, and 7.6% identified with other racial designations. Approximately 21.9% of African American patients had any coronary involvement and 9.5% had a coronary Z-score > 2.5. Approximately 21.4% of non-Hispanic whites had any coronary involvement and 13.5% of non-Hispanic whites had a coronary Z-score > 2.5. Twenty-eight percent of Hispanic patients had any coronary involvement and 12% had a coronary Z-score > 2.5%. Of patients that identified with other racial designations, 38.9% had coronary involvement and 22.2% had a coronary Z-score > 2.5. No statistically significant odds ratios were identified. Relative to reference group (non-Hispanic whites) African American patients had nearly identical rates of 1) any coronary involvement, or 2) coronary Z-score > 2.5. KD occurs commonly in African-American children. Given equal risk for late coronary sequelae vigilance and strict adherence to consensus guidelines is essential.

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