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Overview of the rarest causes of fever in newborns: handy hints for the neonatologist

Journal of Perinatology volume 41, pages 372–382 (2021)Cite this article

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Abstract

Neonatal causes of fever are a major source of concern for clinicians. If fever is combined with organ-specific sterile inflammatory manifestations the suspicion of autoinflammatory disorders should be considered, and the list of such conditions starting in the neonatal period includes chronic infantile neurological cutaneous articular syndrome, mevalonate kinase deficiency, deficiency of the interleukin-1 receptor antagonist, otulipenia, STING-associated vasculopathy with onset in infancy and Blau syndrome. Other causes of noninfectious fever that can rarely occur in newborns are Kawasaki disease, Behçet’s disease, and hemophagocytic lymphohistiocytosis. Diagnosis of these exceptionally rare disorders is challenging for neonatologists. An early recognition of these complex diseases might lead to use more specific or rational drugs preventing permanent consequences. This review focuses on the rarest causes of fever occurring in the neonatal age with the aim of portraying many protean clinical pictures associated with fever and reviewing the potential available treatments.

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  1. These authors contributed equally: Domenico Umberto De Rose, Maria Coppola

Authors and Affiliations

  1. Department of Life Sciences and Public Health, Fondazione Policlinico Universitario “A. Gemelli” IRCCS, Rome, Italy

    Domenico Umberto De Rose, Maria Coppola, Francesca Gallini, Luca Maggio, Giovanni Vento & Donato Rigante

  2. Department of Medical and Surgical Neonatology, “Bambino Gesù” Children’s Hospital IRCCS, Rome, Italy

    Domenico Umberto De Rose

  3. Università Cattolica del Sacro Cuore, Rome, Italy

    Francesca Gallini, Luca Maggio, Giovanni Vento & Donato Rigante

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DUDR and MC conceptualized the study, reviewed articles, and wrote the first draft of the paper; FG, LM, and GV critically revised the paper; DR contributed to the design of this study and critically revised the paper. All authors read and approved the final version of the paper.

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Correspondence to Domenico Umberto De Rose.

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De Rose, D.U., Coppola, M., Gallini, F. et al. Overview of the rarest causes of fever in newborns: handy hints for the neonatologist. J Perinatol 41, 372–382 (2021). https://doi.org/10.1038/s41372-020-0744-8

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