Abstract

BackgroundKawasaki disease (KD) is an acute, self-limited febrile illness of unknown cause. Intravenous immunoglobulin (IVIG)-resistance are related to greater risk for permanent cardiac complications. We aimed to determine the correlation between monocytes and the phenotype of KD in relation to IVIG responsiveness in children.Materials and methods The study cohort included 62 patients who were diagnosed with KD, 20 non febrile healthy controls (NFC), and 15 other febrile controls (OFC). In all enrolled patients, blood was taken at least 4 times and laboratory tests were performed. In addition, subtypes of monocytes were characterized via flow cytometry.ResultsThe numbers of intermediate monocytes were significantly lower in IVIG-resistant group compared to IVIG-responsive group before IVIG infusion (p < 0.0001). After infusion, intermediate monocytes decreased in the responsive group, while a trend of increase was observed in the resistant group. Only intermediate monocytes were significant in logistic regression with adjusted OR of 0.001 and p value of 0.03.ConclusionsCD14 + CD16 + intermediate monocyte may play an important role in IVIG responsiveness among KD children. Low starting levels of intermediate monocytes, followed by a dramatic increase post-IVIG infusion during acute phase of KD are associated with IVIG-resistance. Functional studies on intermediate monocyte may help to reveal the pathophysiology.

Highlights

  • Kawasaki disease (KD) is an acute, self-limited febrile illness

  • The numbers of intermediate monocytes were significantly lower in Intravenous immunoglobulin (IVIG)-resistant group compared to IVIGresponsive group before IVIG infusion (p < 0.0001)

  • Intermediate monocytes decreased in the responsive group, while a trend of increase was observed in the resistant group

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Summary

Introduction

Kawasaki disease (KD) is an acute, self-limited febrile illness. It is the most common cause of acquired heart disease in children in developed countries, but the causes of this illness are still unknown [1].If undiagnosed or untreated, permanent cardiac complication such as coronary arterial dilatation (CAL) can occur. Kawasaki disease (KD) is an acute, self-limited febrile illness. It is the most common cause of acquired heart disease in children in developed countries, but the causes of this illness are still unknown [1]. The probability of acquiring this permanent cardiac complication increases if the patient is resistant to intravenous Immunoglobulin (IVIG), which is approximately 10–20 % of the KD population [2, 3]. The inflammatory cascade during the acute phase of KD has been extensively studied and associated with monocyte/macrophage activation [4,5,6,7,8,9] the immune characteristics of IVIG-resistant patients remain. Kawasaki disease (KD) is an acute, self-limited febrile illness of unknown cause. Intravenous immunoglobulin (IVIG)-resistance are related to greater risk for permanent cardiac complications. We aimed to determine the correlation between monocytes and the phenotype of KD in relation to IVIG responsiveness in children

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