| ISSN |
2219-2808 (online) |
| Open Access |
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ |
| Copyright |
© The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved. |
| Article Reprints |
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| Permissions |
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| Publisher |
Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA |
| Website |
http://www.wjgnet.com |
| Category |
Pediatrics |
| Manuscript Type |
Retrospective Cohort Study |
| Article Title |
Neither hereditary periodic fever nor periodic fever, aphthae, pharingitis, adenitis: Undifferentiated periodic fever in a tertiary pediatric center
|
| Manuscript Source |
Invited Manuscript |
| All Author List |
Silvia De Pauli, Sara Lega, Serena Pastore, Domenico Leonardo Grasso, Anna Monica Rosaria Bianco, Giovanni Maria Severini, Alberto Tommasini and Andrea Taddio |
| ORCID |
|
| Funding Agency and Grant Number |
| Funding Agency |
Grant Number |
| Institute for Maternal and Child Health IRCCS Burlo Garofolo |
RC36/11 |
|
| Corresponding Author |
Alberto Tommasini, MD, PhD, Adjunct Professor, Medical Assistant, Senior Scientist, Institute of Maternal and Child Health, IRCCS Burlo Garofolo, via dell'Istria 65/1, Trieste 34137, Italy. alberto.tommasini@burlo.trieste.it |
| Key Words |
Hereditary periodic fever syndromes; Therapy; Genetics; Autoinflammatory diseases; Undifferentiated periodic fever |
| Core Tip |
Children with non-infectious recurrent fever more often fall into two diagnostic categories. The first and most common is periodic fever, aphthae, pharingitis, adenitis (PFAPA), the second, far more rare, are hereditary periodic fevers. Very recently a third category has been increasingly recognized, and is that of undifferentiated periodic fevers or undifferentiated periodic fever (UPF). UPF include patients who do not meet the diagnostic criteria for PFAFA or for a monogenic disease. The clinical presentation and management of patients with UPF are poorly defined. In this study, the authors describe a cohort of patients with UPF showing that: (1) The clinical manifestations are on a half way of clinical spectrum between PFAPA and hereditary periodic fever; (2) PRINTO-Eurofever score is not useful to guide treatment choices and does not predict disease course; and (3) Both steroids and colchicine are useful to control symptoms in most cases. The authors conclude that further studies are needed to better define UPF and guide their management in clinical practice. |
| Publish Date |
2018-02-07 01:46 |
| Citation |
De Pauli S, Lega S, Pastore S, Grasso DL, Bianco AMR, Severini GM, Tommasini A, Taddio A. Neither hereditary periodic fever nor periodic fever, aphthae, pharingitis, adenitis: Undifferentiated periodic fever in a tertiary pediatric center. World J Clin Pediatr 2018; 7(1): 49-55 |
| URL |
http://www.wjgnet.com/2219-2808/full/v7/i1/49.htm |
| DOI |
http://dx.doi.org/10.5409/wjcp.v7.i1.49 |
