ArticlePediatrics2016
Family History in Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome.
Article in Pediatrics, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 1 of them a synthesis that pooled it.
What it found
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The trial behind it
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Who cites it
29 citing papers in PubMed, 1 synthesis or guideline pooled it, 69 citations in OpenAlex.
- Surgical management for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome: a systematic review and meta-analysis.Jornal de pediatriaPooled it
- Colchicine versus cimetidine: the better choice for Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome prophylaxis, and the role of MEFV gene mutations.Pediatric rheumatology online journal · 2022Trial
- Long-term comorbidity associated with childhood periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: a case-control study.European journal of pediatrics · 2026Article
- Periodic fever, aphthous stomatitis, pharyngitis, adenitis: clinical manifestations, response to steroids, and a practice of online diagnosis and follow-up.Frontiers in immunology · 2026Article
- The etiological spectrum of pediatric FUO and clinical management of PFAPA/SURF: a ten-year retrospective study.Frontiers in immunology · 2026Article
- The first proteomics analysis of tonsils in patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA).Pediatric research · 2025Article
- Features Associated with Response to Tonsillectomy in Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome in Children.The Journal of pediatrics · 2025Article
- Quality of Life and Psychological Functioning in Children with PFAPA Syndrome.Pediatric reports · 2025Article
- Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis Syndrome: An Update.Children (Basel, Switzerland) · 2025Review
- Assessment of recurrent fever among children undergoing tonsillectomy.BMC pediatrics · 2024Article
- Altered serum antibody levels in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis.Archives of rheumatology · 2024Article
- Synchronous disease onset and flares in siblings with PFAPA.Pediatric rheumatology online journal · 2022Article
- Features predicting colchicine efficacy in treatment of children with undefined systemic autoinflammatory disease: A retrospective cohort study.European journal of rheumatology · 2022Article
- Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome: A Single-Center Experience.Turkish archives of pediatrics · 2022Article
- PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort.International journal of general medicine · 2022Article
- Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome in children: a brief literature review.Revista paulista de pediatria : orgao oficial da Sociedade de Pediatria de Sao Paulo · 2022Article
- Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A review.World journal of otorhinolaryngology - head and neck surgery · 2021Review
- Independent risk factors for resolution of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome within 4 years after the disease onset.Clinical rheumatology · 2021Article
- Lifetime prevalence of recurrent aphthous stomatitis and its related factors in Northern Iranian population: The PERSIAN Guilan Cohort Study.Clinical oral investigations · 2021Article
- Familial Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis Syndrome; Is It a Separate Disease?Frontiers in pediatrics · 2021Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
objectiveThe goal of this study was to describe family history and inheritance patterns in patients with periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome.
methodsWe performed a case-control study to compare the family histories of patients with PFAPA recruited from Vanderbilt University Medical Center and matched healthy control subjects from a pediatric primary care practice in Nashville, Tennessee, by using a structured questionnaire. Characteristics of paired case subjects, control subjects, and their family members were compared by using McNemar's test and Wilcoxon signed-rank tests.
resultsEighty PFAPA index case subjects and 80 control subjects were recruited. Eighteen PFAPA case subjects (23%) had ≥1 family member with PFAPA. Parents of PFAPA index case subjects were more likely to have recurrent pharyngitis (36% vs 16%; P < .001) and recurrent aphthous stomatitis (46% vs 28%; P = .002) compared with parents of control subjects. Siblings of case subjects had a higher prevalence of PFAPA (10% vs 2%; P = .04), recurrent pharyngitis (24% vs 10%; P = .03), and recurrent aphthous stomatitis (27% vs 7%; P = .003) compared with siblings of control subjects.
conclusionsA portion of PFAPA case subjects seems to be familial, implying an inherited genetic predisposition to the disorder and/or shared environmental exposures. First-degree relatives (parents and siblings) of patients with PFAPA have a higher prevalence of recurrent pharyngitis and aphthous stomatitis than relatives of control subjects, which suggests that these disorders represent reduced penetrance phenotypes of PFAPA. Further characterization of the genetics and inflammatory profiles of these patients and their relatives is warranted.
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Registered trials
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.