Evidence map›Paper›PMID 40122279›Full record

ArticleThe Journal of pediatrics2025

Features Associated with Response to Tonsillectomy in Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome in Children.

Kalpana Manthiram, Ana M Ortega-Villa, Sivia Lapidus, Mary Bowes, Tina Romeo, Kathryn Garguilo, Laura Failla, Hemalatha Srinivasalu, Pamela Mudd, Amanda Ombrello and 3 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in The Journal of pediatrics, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
4citing 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

4 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Review
  3. Article
  4. Review
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

13 authors.

Kalpana ManthiramLaboratory of Immune System Biology, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health, Bethesda, MD. Electronic address: kalpana.manthiram@nih.gov.
Ana M Ortega-VillaBiostatistics Research Branch, Division of Clinical Research, NIAID, NIH, Bethesda, MD.
Sivia LapidusJoseph M. Sanzari Children's Hospital, Hackensack University Medical Center, Hackensack, NJ.
Mary BowesLaboratory of Immune System Biology, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health, Bethesda, MD.
Tina RomeoNational Human Genome Research Institute (NHGRI), NIH, Bethesda, MD.
Kathryn GarguiloDivision of Pediatric Infectious Diseases, Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN.
Laura FaillaLaboratory of Immune System Biology, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health, Bethesda, MD.
Hemalatha SrinivasaluDivision of Rheumatology, Department of Pediatrics, George Washington University School of Medicine, Children's National Hospital, Washington, DC.
Pamela MuddDivision of Pediatric Otolaryngology, Children's National Hospital, Washington, DC; Division of Otolaryngology, Department of Surgery, George Washington University School of Medicine and Health Sciences, Washington, DC.
Amanda OmbrelloNational Human Genome Research Institute (NHGRI), NIH, Bethesda, MD.
Karyl BarronNIAID, National Institutes of Health, Bethesda, MD.
Daniel L KastnerNational Human Genome Research Institute (NHGRI), NIH, Bethesda, MD.
Kathryn M EdwardsDivision of Pediatric Infectious Diseases, Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN.

Funding

The Vanderbilt Institute for Clinical and Translational Research (VICTR)UL1TR000445 · NCATS · VANDERBILT UNIVERSITY MEDICAL CENTER · PI BERNARD, GORDON RAPHAEL · 2012 to 2016
$41.4M
Childhood Infections Research ProgramT32AI095202 · NIAID · VANDERBILT UNIVERSITY MEDICAL CENTER · PI JAMES E CASSAT · 2011 to 2026
$4.2M
Conducting Child Health Care Research in Vulnerable PopulationsT32HD060554 · NICHD · VANDERBILT UNIVERSITY MEDICAL CENTER · PI BARKIN, SHARI, WILLIAMS, DEREK J. · 2009 to 2019
$3.8M
Genetic susceptibility loci for PFAPAZIAAI001363 · NIAID · NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES · PI MANTHIRAM, KALPANA · 2022 to 2025
$2.2M
Immunology of Pediatric Tonsil DisordersZIAAI001340 · NIAID · NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES · PI MANTHIRAM, KALPANA · 2021 to 2025
$1.9M
Intramural NIH HHS Z99 AI999999Intramural NIH HHS ZIA AI001340Intramural NIH HHS ZIA AI001363NCATS NIH HHS UL1 TR000445NIAID NIH HHS T32 AI095202NICHD NIH HHS T32 HD060554
6 · The paper itself

Abstract

objectiveTo identify clinical features associated with response to tonsillectomy among children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome and to determine optimal management of children with continued episodes after tonsillectomy. STUDY

designPatients with PFAPA seen at Vanderbilt University Children's Hospital and the National Institutes of Health (NIH) who underwent tonsillectomy were enrolled and queried regarding symptoms before and after surgery.

resultsNinety-seven subjects with PFAPA (43 Vanderbilt, 54 NIH) were followed for a median of 49 months following tonsillectomy. Nearly half of participants reported complete resolution of PFAPA episodes (65% at Vanderbilt and 35% at NIH), while 25% had less severe or frequent episodes, 23% had a period of remission with recurrence, and 4% had no change in episodes. By logistic regression, factors associated with a full response to tonsillectomy were episode resolution with glucocorticoid treatment, presence of exudative pharyngitis, absence of rash, and absence of arthralgia/myalgia during pre-tonsillectomy PFAPA flares. Among those requiring treatment for persistent flares post-tonsillectomy, 15/19 (79%) reported improvement with cimetidine or famotidine prophylaxis.

conclusionTonsillectomy remains effective in improving PFAPA flares in most patients. However, unique episode features prior to tonsillectomy appear to be clinical predictors of response to tonsillectomy. Histamine receptor 2 antagonists like cimetidine were effective prophylactic agents for refractory cases post-tonsillectomy. Patients with incomplete response to tonsillectomy may represent a subset of PFAPA with unique factors affecting their pathogenesis.

Indexed as

FeverLymphadenitisPharyngitisStomatitis, AphthousTonsillectomyAdolescentChildChild, PreschoolFemaleFollow-Up StudiesHumansMaleSyndromeTreatment Outcomeperiodic feverPFAPAtonsillectomy

Identifiers

PMID40122279
PMCPMC12158635

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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.