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Do we have a less severe form of Kawasaki disease or is it the gammaglobulin effect?
L Abushaban1, A Salama, B Uthaman
1Paediatric Department, Kuwait University, Safat.
Insights
Coronary artery aneurysms (CAA) in Kawasaki disease (KD) were less common and severe in this study, with early IVGG treatment potentially playing a role. Most cases showed regression, but genetic factors may influence incidence across racial groups.
Area of Science:
- Pediatric Cardiology
- Rheumatology
- Immunology
Background:
- Kawasaki disease (KD) is a leading cause of acquired heart disease in children.
- Coronary artery aneurysms (CAA) are a significant complication of KD, potentially leading to long-term cardiovascular issues.
- Understanding the incidence and natural history of CAA in KD is crucial for effective management.
Purpose of the Study:
- To determine the incidence of coronary artery aneurysms (CAA) in children diagnosed with Kawasaki disease (KD).
- To evaluate the echocardiographic findings and clinical outcomes of KD patients with and without CAA.
- To investigate potential factors influencing CAA development and regression in KD.
Main Methods:
- Retrospective review of clinical and echocardiographic data from 135 children with KD treated between 1986 and 1997.
- Echocardiograms performed 2-3 weeks after fever onset to assess coronary artery status.
- Follow-up assessments conducted over 6 months to 1.5 years to monitor coronary artery changes.
Main Results:
- Overall incidence of CAA was 7.4% (10 patients).
- Most CAA cases (80%) regressed completely within 1.5 years; one patient had residual giant CAA.
- Minimal ectasia was observed in 11.85% and resolved within 6 months; 78.5% had normal echocardiograms.
- No patients experienced cardiac failure, arrhythmia, myocardial infarction, or death.
Conclusions:
- Coronary artery abnormalities, including CAA, appear less frequent and severe in this cohort compared to other studies.
- Early administration of intravenous immunoglobulin (IVIG) may contribute to reduced CAA incidence.
- Further genetic studies are warranted to explore racial variations in CAA incidence secondary to KD.
Abstract:
This study was undertaken to evaluate the incidence of coronary artery aneurysms (CAA) in Kawasaki disease (KD). We reviewed the clinical and echocardiographic findings of 135 children who presented to our center with KD between December 1986 and December 1997. The age of onset ranged between 3months to 13 years (median 2 years). The male to female ratio was 1.54:1. All patients received intravenous Gammaglobulin (IVGG) during the acute stage. The echocardiogram, which was done between 2-3 weeks of the onset of fever, was normal in 106 patients (78.5%). Follow-up studies over a period of 6 months to 1 year remained normal. Minimal right or left coronary artery wall ectasia without dilatation or aneurysm formation was seen in 16 (11.85%). Follow-up of these patients showed disappearance of these changes over 6 weeks to 6 months. One patient (0.74%) had generalised dilatation of all the coronary arteries during the acute stage. This has normalized over a period of 9 months. A total of 10 (7.4%) had CAA during the acute stage. On follow-up of 8 of these patients for an average 3 months to 1.5 years all CAA regressed completely. One patient had residual Giant CAA after 1 year follow-up. One patient with CAA was lost to follow-up. One patient (0.74%) had pericardial effusion and another one (0.74%) had mitral incompetence during the acute stage only, both had no coronary involvement. None of our patients had cardiac failure, arrhythmia, myocardial infarction or death. We conclude that coronary artery changes due to KD are less common and less severe in our patients than those seen in other studies. We speculate that this can be related partly to the early administration of IVGG. The difference in incidence of CAA secondary to KD among different racial groups warrants more detailed genetic studies.