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Inability of Asian risk scoring systems to predict intravenous immunoglobulin resistance and coronary lesions in Kawasaki disease in an Italian cohort

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Abstract

Since resistance to intravenous immunoglobulin (IVIG) is associated with coronary lesions (CALs) in Kawasaki disease (KD), it is crucial to identify patients at risk to protect them from coronary involvement. The available risk scores to predict IVIG resistance were developed in Asian populations in whom their effectiveness has been proven, but data on non-Asian children are limited. The aim of this study is to evaluate the ability of the Kobayashi, Egami, and Formosa risk scores to predict IVIG resistance and CALs in Italian patients with KD. A multicenter retrospective analysis involving children with KD diagnosed between 2000 and 2015 was carried out: 257 patients were enrolled (57.9% boys, 89.9% Caucasian); 43 patients were IVIG resistant (16.7%). The scores have low sensitivity and specificity in predicting IVIG resistance: respectively, KS 64% and 62.5%, ES 41.4% and 77.4%, and FS 70.8% and 44.9%. The predictive value of the 3 scores for predicting CALs was also poor.

Conclusion: Kobayashi, Egami, and Formosa Scores are ineffective in predicting IVIG resistance and coronary involvement in a predominantly Caucasian cohort. A specific score system for mostly Caucasian children with KD is needed enable the early identification of those at risk for CALs who could benefit from intensified treatment.

What is Known:

• There are several risk scores developed in the Asian population to early identify patients with KD at risk for immunoglobulin-resistance and thus for coronary lesions.

• Data are scarce on their effectiveness in non-Asian children.

What is New:

• We present a comprehensive analysis of the ability of 3 Asian risk scores in a cohort of mostly Caucasian children to predict immunoglobulin resistance and coronary involvement.

• Low sensitivity and specificity of the Asian scores for immunoglobulin-resistance and coronary lesions suggest the need for criteria specific for different ethnicities.

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Abbreviations

AHA:

American Heart Association

ALT:

Alanine aminotransferase

ASA:

Acetylsalicylic acid

AST:

Aspartate aminotransferase

CALs:

Coronary artery lesions

CRP:

C-reactive protein

ES:

Egami score

ESR:

Erythrocyte sedimentation rate

FS:

Formosa score

Hb:

Hemoglobin

IVIG:

Intravenous immunoglobulin

KS:

Kobayashi score

LDH:

Lactate dehydrogenase

Na:

Sodium

PLT:

Platelet count

RBC:

Red blood cells

SD:

Standard deviation

WBC:

White blood cells

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Author information

Authors and Affiliations

  1. Department of Pediatrics, S.Orsola-Malpighi Hospital, University of Bologna, Via Massarenti 9, 40138, Bologna, Italy

    Marianna Fabi, Laura Andreozzi, Tetyana Bodnar & Marcello Lanari

  2. Department of Pediatrics, Ramazzini Hospital, Carpi, Italy

    Elena Corinaldesi

  3. Pediatric Unit, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, Modena, Italy

    Francesca Lami & Lorenzo Iughetti

  4. Department of Pediatrics, AUSL, Guglielmo da Saliceto Hospital, Piacenza, Italy

    Cristina Cicero

  5. Department of Pediatrics, Ospedale dei Bambini Barilla, University of Parma, Parma, Italy

    Bertrand Tchana

  6. Department of Pediatrics, Maggiore Hospital, Bologna, Italy

    Chiara Landini

  7. Department of Pediatrics, Arcispedale Sant’Anna, Ferrara, Italy

    Monica Sprocati

  8. Department of Pediatrics, Infermi Hospital, Rimini, Italy

    Barbara Bigucci

  9. Department of Pediatrics, Bufalini Hospital, Cesena, Italy

    Claudia Balsamo

  10. Department of Pediatrics, Santa Maria della Scaletta Hospital, Imola, Italy

    Paola Sogno Valin

  11. Department of Pediatrics, IRCCS, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy

    Giorgia Di Fazzio

  12. Department of Pediatrics, G.B.Morgagni - L. Pierantoni Hospital, AUSL della Romagna, Forlì, Italy

    Enrico Valletta

  13. Department of Pediatrics, Santa Maria delle Croci Hospital, AUSL della Romagna, Ravenna, Italy

    Federico Marchetti

  14. Pediatric Cardiology and Adult Congenital Unit, S.Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy

    Andrea Donti

Authors
  1. Marianna Fabi
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  2. Laura Andreozzi
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  3. Elena Corinaldesi
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  4. Tetyana Bodnar
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  5. Francesca Lami
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  6. Cristina Cicero
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  7. Bertrand Tchana
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  8. Chiara Landini
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  9. Monica Sprocati
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  10. Barbara Bigucci
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  15. Enrico Valletta
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  18. Marcello Lanari
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Contributions

Marianna Fabi conceptualized and designed the study and the data collection instruments, drafted the initial manuscript, and supervised the reviewed literature.

Laura Andreozzi carried out the statistical analysis and contributed to the collection of data, and to write the initial draft.

Elena Corinaldesi contributed to the collection of data and supervised the reviewed literature.

Tetyana Bodnar coordinated and supervised data collection, contributed to the critical revision of literature, and critically reviewed the manuscript.

Francesca Lami coordinated and supervised data collection, contributed to the critical revision of literature, and critically reviewed the manuscript.

Andrea Donti coordinated and supervised data collection, contributed to the critical revision of literature, and critically reviewed the manuscript.

Chiara Landini contributed to data acquisition and critically revised the manuscript.

Cristina Cicero contributed to data acquisition and critically revised the manuscript.

Monica Sprocati contributed to data acquisition and critically revised the manuscript.

Barbara Bigucci contributed to data acquisition and critically revised the manuscript.

Claudia Balsamo contributed to data acquisition and critically revised the manuscript.

Giorgia Di Fazzio contributed to data acquisition and critically revised the manuscript.

Federico Marchetti helped to draft the manuscript, contributed to the critical revision of literature, and reviewed the manuscript.

Bertrand Tchana helped to draft the manuscript, contributed to the critical revision of literature, and reviewed the manuscript.

Paola Sogno Valin helped to draft the manuscript, contributed to the critical revision of literature, and reviewed the manuscript.

Enrico Valletta helped to draft the manuscript, contributed to the critical revision of literature, and reviewed the manuscript.

Lorenzo Iughetti helped to draft the manuscript, contributed to the critical revision of literature, and reviewed the manuscript.

Marcello Lanari supervised the reviewed literature, contributed to the interpretation of data, and critically reviewed the manuscript for important content.

All authors approved the final manuscript as submitted and agreed to be accountable for all aspects of the work.

Corresponding author

Correspondence to Marianna Fabi.

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Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

The study was approved by the Central Review Board.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Additional information

Communicated by Peter de Winter

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Fabi, M., Andreozzi, L., Corinaldesi, E. et al. Inability of Asian risk scoring systems to predict intravenous immunoglobulin resistance and coronary lesions in Kawasaki disease in an Italian cohort. Eur J Pediatr 178, 315–322 (2019). https://doi.org/10.1007/s00431-018-3297-5

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  • DOI: https://doi.org/10.1007/s00431-018-3297-5

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