Evidence map›Paper›PMID 42289664›Full record

ArticleBMC pediatrics2026

Epidemiology and clinical course of PFAPA syndrome in the pediatric population-single center study.

Natalia Gołuchowska, Agata Tomaszewska, Aldona Ząber, Agata Będzichowska, Agata Knapik, Aleksandra Kudiuk, Agnieszka Rustecka, Bolesław Kalicki

Abstract read
In one paragraph

Article in BMC pediatrics, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed, 1 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors.

Natalia GołuchowskaDepartment of Pediatrics, Nephrology and Allergology, Military Institute of Medicine - National Research Institute, Szaserów 128, 04-141, Warsaw, Poland. goluchowskan@gmail.com.
Agata TomaszewskaDepartment of Pediatrics, Nephrology and Allergology, Military Institute of Medicine - National Research Institute, Szaserów 128, 04-141, Warsaw, Poland.
Aldona ZąberDepartment of Pediatrics, Nephrology and Allergology, Military Institute of Medicine - National Research Institute, Szaserów 128, 04-141, Warsaw, Poland.
Agata BędzichowskaDepartment of Pediatrics, Nephrology and Allergology, Military Institute of Medicine - National Research Institute, Szaserów 128, 04-141, Warsaw, Poland.
Agata KnapikFaculty of Medicine, University of Warsaw, Krakowskie Przedmieście 26/28, Warsaw, 00-927, Poland.
Aleksandra KudiukFaculty of Medicine, University of Warsaw, Krakowskie Przedmieście 26/28, Warsaw, 00-927, Poland.
Agnieszka RusteckaDepartment of Pediatrics, Nephrology and Allergology, Military Institute of Medicine - National Research Institute, Szaserów 128, 04-141, Warsaw, Poland.
Bolesław KalickiDepartment of Pediatrics, Nephrology and Allergology, Military Institute of Medicine - National Research Institute, Szaserów 128, 04-141, Warsaw, Poland.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectivePFAPA syndrome is the most common autoinflammatory disease of childhood, with an as yet unknown etiology. It is characterized by recurrent, periodic episodes of fever accompanied by specific clinical features. The aim of this study was to provide a detailed characterization of PFAPA syndrome in a large Polish pediatric cohort.

methodsWe conducted a retrospective study based on data from 47 children with a confirmed diagnosis of PFAPA syndrome. Data were collected using structured questionnaires. Detailed analyses included clinical manifestations, therapeutic approaches, and laboratory findings.

resultsThe median age of the study population was 5 years, with a predominance of males (59.6%). In 91.5% of patients, the first episode of fever occurred before the age of 5 years. Febrile episodes lasted a mean of 5 days, with a median inter-episode interval of 3 weeks. The key clinical manifestations included cervical lymphadenopathy (91.5%), tonsillar exudates (85.1%), pharyngitis (74.5%), and oral aphthae (51.1%). During afebrile intervals, 87.2% of patients were free of symptoms. Laboratory evaluation showed elevated inflammatory markers, with a median C-reactive protein level of 6.9 mg/d (in the 9 patients with available laboratory data), a median erythrocyte sedimentation rate of 35.0 mm/h, and a mean neutrophil count of 11.8 × 10^3/µL. Prednisone treatment was effective in 97.6% of cases. Tonsillectomy was performed in 25.5% of children and resulted in complete resolution of febrile episodes in 83.3% of them.

conclusionsThis study confirms the established epidemiologic and clinical profile of PFAPA syndrome in the pediatric population, highlighting its characteristic manifestations and the high effectiveness of glucocorticoid therapy and tonsillectomy. The findings may contribute to consolidating current knowledge about PFAPA and to reducing the inappropriate use of antibiotics.

Indexed as

FeverHereditary Autoinflammatory DiseasesLymphadenitisPharyngitisStomatitis, AphthousAdolescentChildChild, PreschoolC-Reactive ProteinFemaleHumansInfantLymphadenopathyMaleRetrospective StudiesSyndromeC-Reactive ProteinCervical adenitisPFAPAPharyngitisRecurrent feverStomatitis

Identifiers

PMID42289664
PMCPMC13492027

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.