Background 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. To date, abdominal involvement at presentation is not recognized as a risk factor for a more severe form of the disease. Objective To evaluate whether presenting abdominal manifestations identify a group at major risk for Intravenous immunoglobulin (IVIG)-resistance and coronary lesions. Methods Retrospective 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. Results 302 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). Conclusions This 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.

Gastrointestinal presentation of kawasaki disease: A red flag for severe disease? / Fabi, Marianna; Corinaldesi, Elena; Pierantoni, Luca; Mazzoni, Elisa; Landini, Chiara; Bigucci, Barbara; Ancora, Gina; Malaigia, Laura; Bodnar, Tetyana; Fazzio, Giorgia Di; Lami, Francesca; Valletta, Enrico; Cicero, Cristina; Biasucci, Giacomo; Iughetti, Lorenzo; Marchetti, Federico; Valin, Paola Sogno; Amarri, Sergio; Brusa, Sandra; Sprocati, Monica; Maggiore, Giuseppe; Dormi, Ada; Lanzoni, Paolo; Donti, Andrea; Lanari, Marcello. - In: PLOS ONE. - ISSN 1932-6203. - 13:9(2018), pp. N/A-N/A. [10.1371/journal.pone.0202658]

Gastrointestinal presentation of kawasaki disease: A red flag for severe disease?

Fazzio, Giorgia Di;Lami, Francesca;Iughetti, Lorenzo;Amarri, Sergio;
2018

Abstract

Background 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. To date, abdominal involvement at presentation is not recognized as a risk factor for a more severe form of the disease. Objective To evaluate whether presenting abdominal manifestations identify a group at major risk for Intravenous immunoglobulin (IVIG)-resistance and coronary lesions. Methods Retrospective 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. Results 302 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). Conclusions This 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.
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Gastrointestinal presentation of kawasaki disease: A red flag for severe disease? / Fabi, Marianna; Corinaldesi, Elena; Pierantoni, Luca; Mazzoni, Elisa; Landini, Chiara; Bigucci, Barbara; Ancora, Gina; Malaigia, Laura; Bodnar, Tetyana; Fazzio, Giorgia Di; Lami, Francesca; Valletta, Enrico; Cicero, Cristina; Biasucci, Giacomo; Iughetti, Lorenzo; Marchetti, Federico; Valin, Paola Sogno; Amarri, Sergio; Brusa, Sandra; Sprocati, Monica; Maggiore, Giuseppe; Dormi, Ada; Lanzoni, Paolo; Donti, Andrea; Lanari, Marcello. - In: PLOS ONE. - ISSN 1932-6203. - 13:9(2018), pp. N/A-N/A. [10.1371/journal.pone.0202658]
Fabi, Marianna; Corinaldesi, Elena; Pierantoni, Luca; Mazzoni, Elisa; Landini, Chiara; Bigucci, Barbara; Ancora, Gina; Malaigia, Laura; Bodnar, Tetyana; Fazzio, Giorgia Di; Lami, Francesca; Valletta, Enrico; Cicero, Cristina; Biasucci, Giacomo; Iughetti, Lorenzo; Marchetti, Federico; Valin, Paola Sogno; Amarri, Sergio; Brusa, Sandra; Sprocati, Monica; Maggiore, Giuseppe; Dormi, Ada; Lanzoni, Paolo; Donti, Andrea; Lanari, Marcello
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11380/1170969
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