Abstract
Kawasaki syndrome (KS) is the most relevant cause of heart disease in children living in developed countries. Intravenous immunoglobulin (IVIG) has a preventive function in the formation of coronary artery abnormalities and a poor strictly-curative action in established coronary damage. More than two decades ago, the Harada score was set to assess which children with KS should be subject to administration of IVIG, evaluating retrospectively a large cohort of patients with regard to age, sex and laboratory data. Nowadays, high dose IVIG is administered to all children with a confirmed diagnosis of KS, but a tool for predicting non-responsiveness to the initial infusion of IVIG has not been found. The prediction of IVIG resistance is a crucial issue, as recognising these high-risk patients should consent the administration of an intensified initial treatment in combination with IVIG in order to prevent coronary injuries. Few reports have focused on factors, referring to both clinical parameters and laboratory data at the onset of KS, in order to predict which patients might be IVIG non-responsive. We have analysed three different risk scores which were formulated to predict IVIG resistance in Japanese children with typical KS, but their application in non-Japanese patients or in those with incomplete and atypical patterns of the disease has been studied in a fragmentary way. Overall, our analysis showed that early and definite ascertainment of likely IVIG non-responders who require additional therapies reducing the development of coronary artery involvement in children with KS is still a challenge.
Highlights
Almost half a century has passed since Kawasaki syndrome (KS) was first reported by Tomisaku Kawasaki as an enigmatic disease affecting children, and, today, KS still remains uncannily dangerous due to the intrinsic risk of damaging the vascular system, mostly the coronary arteries, in 25% of untreated patients
The Harada score (1991) was extrapolated by a multicentre study carried out to evaluate the effectiveness of different doses and kinds of intravenous globulin (IVIG) in 865 naïve patients with Kawasaki syndrome, analysed retrospectively with regard to laboratory data, age, and sex: the score was an attempt to establish a set of criteria for starting treatment with intravenous immunoglobulin (IVIG)
The first of these, reported by Asai in 1983, tried to determine an indication for cardiac catheterization during a period in which echocardiography was not routinely used in Japan in the management of KS [12]. Another scoring model was created by Nakano et al [13] in 1986, starting from clinical and early laboratory findings from 78 children who were hospitalized since the 4th day of fever to the 7th: they found that age at onset, C-reactive protein (CRP), and baseline platelet count were useful items in the differentiation of patients at higher risk of coronary artery abnormalities (CAA)
Summary
Almost half a century has passed since Kawasaki syndrome (KS) was first reported by Tomisaku Kawasaki as an enigmatic disease affecting children, and, today, KS still remains uncannily dangerous due to the intrinsic risk of damaging the vascular system, mostly the coronary arteries, in 25% of untreated patients. Prompt treatment with intravenous immunoglobulin (IVIG) has been shown to resolve all manifestations of KS and to significantly decrease the risk of development of coronary artery abnormalities (CAA) [5]. Many recent data suggest that treatment and prognostic issues are dissociated with the etiology of KS, though the extent of acute phase response and a younger age at onset are probably related to patients’ responsiveness to IVIG [8]. The main aim of this review was to analyse the three more recent risk scores which were formulated to predict IVIG resistance in Japanese populations of children with typical KS, and to evaluate their pertinence in non-Japanese patients or in those with incomplete and atypical patterns of the disease
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