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Giant aneurysms: A gender-specific complication of Kawasaki disease?
Sanne M Dietz1, Irene M Kuipers2, Carline E A Tacke1
1Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands.
Insights
Male gender, young age, and IVIG retreatment increase the risk of giant coronary artery aneurysms in Kawasaki disease. Early and repeated echocardiography is crucial for monitoring disease progression.
Area of Science:
- Pediatric Cardiology
- Vasculitis Research
- Medical Diagnostics
Background:
- Kawasaki disease (KD) is a pediatric vasculitis with unknown etiology.
- Coronary artery aneurysms (CAA) are a primary complication, with giant CAA representing the most severe form.
- Identifying risk factors for giant CAA is critical for timely intervention.
Purpose of the Study:
- To evaluate patient characteristics associated with the development of giant CAA in Kawasaki disease.
- To identify predictors of giant CAA using z-score analysis.
- To differentiate risk factors for giant CAA from other CAA sizes.
Main Methods:
- Retrospective cohort study of 301 Kawasaki disease patients.
- Analysis of echocardiographic data to categorize CAA size (none, small, medium, giant).
- Multivariable, multinomial logistic regression to identify risk factors for giant CAA.
Main Results:
- Male gender, age under 1 year, and IVIG re-treatment were significantly associated with increased risk of giant CAA.
- 95% of patients with giant CAA were male.
- Giant CAA often continued to increase in size within the first 40 days.
Conclusions:
- Male gender, younger age, and IVIG re-treatment are key risk factors for giant CAA.
- Giant CAA may represent a distinct, treatment-resistant subgroup of KD.
- Repeated echocardiography is recommended in the first two months to monitor CAA growth.
Background:
Kawasaki disease (KD) is a pediatric vasculitis of unknown origin. Its main complication is the development of coronary artery aneurysms (CAA) with giant CAA at the end of the spectrum.
Methods:
In this cohort study, we evaluated the association between patient characteristics and the development of giant CAA based on z-scores. Multivariable, multinomial logistic regression analysis was used to identify variables associated with giant CAA.
Results:
A total of 301 KD patients, comprising 216 patients without enlargement, 45 with small-sized, 19 with medium-sized, and 21 with giant CAA with all echocardiographies at our center were retrospectively included. Remarkably, 95% of patients with giant CAA were boys. In addition to 'no/late intravenous immunoglobulin (IVIG) treatment', 'male gender' (OR 16.23, 95% CI 1.88-140.13), 'age<1 year' (OR 7.49, 95% CI 2.29-24.46), and 'IVIG re-treatment (9.79, 95% CI 2.79-34.37)' were significantly associated with an increased risk of giant CAA, with patients without enlargement as reference. Compared to patients with medium-sized CAA, 'IVIG re-treatment' was significantly associated with giant CAA. The majority of giant CAA continued to increase in size during the first 40 days.
Conclusions:
We identified risk factors associated with an increased risk of giant CAA. The difference in variables between the giant CAA group and the other CAA subgroups suggests a separation between patients with the treatment-resistant giant CAA and the other IVIG-responsive patients, in which gender may be factored as a most relevant genetic trait. The increase in size during the first 2 months indicates the need for repeated echocardiography.
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