Abstract

BackgroundKawasaki disease (KD) is a febrile systemic vasculitis of unknown etiology and the main cause of acquired heart disease among children in the developed world. To date, abdominal involvement at presentation is not recognized as a risk factor for a more severe form of the disease.ObjectiveTo evaluate whether presenting abdominal manifestations identify a group at major risk for Intravenous immunoglobulin (IVIG)-resistance and coronary lesions.MethodsRetrospective study of KD patients diagnosed between 2000 and 2015 in 13 pediatric units in Italy. Patients were divided into 2 groups according to the presence or absence of abdominal manifestations at onset. We compared their demographic and clinical data, IVIG-responsiveness, coronary ectasia/aneurysms, laboratory findings from the acute and subacute phases.Results302 patients (181 boys) were enrolled: 106 patients with, and 196 patients without presenting abdominal features. Seasonality was different between the groups (p = 0.034). Patients with abdominal manifestations were younger (p = 0.006) and more frequently underwent delayed treatment (p = 0.014). In the acute phase, patients with abdominal presentation had higher platelet counts (PLT) (p = 0.042) and lower albuminemia (p = 0.009), while, in the subacute phase, they had higher white blood cell counts (WBC) and PLT (p = 0.002 and p < 0.005, respectively) and lower red blood cell counts (RBC) and hemoglobin (Hb) (p = 0.031 and p 0.009). Moreover, the above mentioned group was more likely to be IVIG-resistant (p < 0.005) and have coronary aneurysms (p = 0.007). In the multivariate analysis, presenting abdominal manifestations, age younger than 6 months, IVIG- resistance, delayed treatment and albumin concentration in the acute phase were independent risk factors for coronary aneurysms (respectively p<0.005, <0.005, = 0.005 and 0.009).ConclusionsThis is the first multicenter report demonstrating that presenting gastrointestinal features in KD identify patients at higher risk for IVIG-resistance and for the development of coronary aneurysms in a predominantly Caucasian population.Clinical trial registration8/20014/O/OssN.

Highlights

  • In the multivariate analysis, presenting abdominal manifestations, age younger than 6 months, intravenous immunoglobulin (IVIG)- resistance, delayed treatment and albumin concentration in the acute phase were independent risk factors for coronary aneurysms. This is the first multicenter report demonstrating that presenting gastrointestinal features in Kawasaki disease (KD) identify patients at higher risk for IVIG-resistance and for the development of coronary aneurysms in a predominantly Caucasian population

  • Kawasaki disease (KD) is a febrile systemic vasculitis of unknown etiology which usually affects children younger than 5 years of age, and it is the main cause of acquired heart disease in the developed world

  • Conflicting data exist regarding the risk of coronary artery lesions (CALs) in patients with various clinical presentations [2,3], incomplete [2, 4] and atypical forms of KD [5] seem to be related to a higher risk of coronary involvement [6]

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Summary

Introduction

Kawasaki disease (KD) is a febrile systemic vasculitis of unknown etiology which usually affects children younger than 5 years of age, and it is the main cause of acquired heart disease in the developed world. It mainly affects small and medium-sized arteries, leading to coronary artery lesions (CALs) in up to 25% of untreated cases [1]. The aim of our study was to evaluate whether gastrointestinal symptoms at presentation, regardless of their magnitude, can identify a group at higher risk for IVIGresistance and CALs. Kawasaki disease (KD) is a febrile systemic vasculitis of unknown etiology and the main cause of acquired heart disease among children in the developed world. Abdominal involvement at presentation is not recognized as a risk factor for a more severe form of the disease

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