University of Pennsylvania

education šŸ“ Philadelphia, United States
University of Pennsylvania
3
PFAPA Syndrome Publications
2
PFAPA Syndrome Researchers

Associated Institutions

Children's Hospital of Philadelphia
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Penn Presbyterian Medical Center
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Hospital of the University of Pennsylvania
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Pennsylvania Hospital
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Penn Center for AIDS Research
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Publications

Surgical Treatments for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome: Systematic Review and Network Meta-analysis.

Noy R, Barzilai R, Cohen JT, Gordin A, Zur KB
Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery •

To compare surgical versus medical treatment approaches for periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. PubMed, Embase, Web of Science, and Cochrane. We followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses-Network Meta-analyses checklist. Two authors independently reviewed the studies. Inclusion criteria comprised randomized controlled trials and cohort studies. Non-English studies, along with case series and review articles, were excluded. The primary outcome was the incidence of persistent symptoms following surgery, compared to medical treatment. Secondary outcomes included complications. The search identified 693 publications. After applying the inclusion and exclusion criteria, nine studies were included (three randomized controlled trials and six cohort studies), comprising a total of 691 patients (256 [37%] females, mean age: 2.97 years, and interquartile range: 2.3-3.3). Patients were treated with either tonsillectomy (n = 201), intracapsular tonsillectomy (IT, n = 24), or medical treatment (n = 466). Surgery was associated with a higher likelihood of symptom resolution compared to medical treatment (odds ratio [OR]: 11.7, 95% CI: 2.14-63.94). However, heterogeneity was observed across studies (I = 80.8%, P < .01). A sensitivity analysis was performed, including randomized controlled trials. Both tonsillectomy (OR: 34.15, 95% CI: 3.77-308.95) and IT (OR: 21, 95% CI: 1.5-293.25) were associated with a greater likelihood of symptom resolution. The pooled complication rate was 6.6%, with a higher incidence in patients who underwent tonsillectomy versus IT (46/201 vs 0/24, P = .008). The pooled rate of symptom recurrence was 13.6% in patients following tonsillectomy and 37.5% in IT. Surgical management of PFAPA with tonsillectomy was superior to medical treatment. Tonsillectomy resulted in a lower recurrence rate of symptoms compared to IT, with a comparable incidence of complications to existing literature. However, given the limited number of patients in the IT group, these findings should be interpreted with caution, and further randomized studies are warranted.

Clinical practice guideline: tonsillectomy in children.

Baugh RF, Archer SM, Mitchell RB, Rosenfeld RM, Amin R , et al.
Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery •

Tonsillectomy is one of the most common surgical procedures in the United States, with more than 530,000 procedures performed annually in children younger than 15 years. Tonsillectomy is defined as a surgical procedure performed with or without adenoidectomy that completely removes the tonsil including its capsule by dissecting the peritonsillar space between the tonsil capsule and the muscular wall. Depending on the context in which it is used, it may indicate tonsillectomy with adenoidectomy, especially in relation to sleep-disordered breathing. This guideline provides evidence-based recommendations on the preoperative, intraoperative, and postoperative care and management of children 1 to 18 years old under consideration for tonsillectomy. In addition, this guideline is intended for all clinicians in any setting who interact with children 1 to 18 years of age who may be candidates for tonsillectomy. The primary purpose of this guideline is to provide clinicians with evidence-based guidance in identifying children who are the best candidates for tonsillectomy. Secondary objectives are to optimize the perioperative management of children undergoing tonsillectomy, emphasize the need for evaluation and intervention in special populations, improve counseling and education of families of children who are considering tonsillectomy for their child, highlight the management options for patients with modifying factors, and reduce inappropriate or unnecessary variations in care. The panel made a strong recommendation that clinicians should administer a single, intraoperative dose of intravenous dexamethasone to children undergoing tonsillectomy. The panel made a strong recommendation against clinicians routinely administering or prescribing perioperative antibiotics to children undergoing tonsillectomy. The panel made recommendations for (1) watchful waiting for recurrent throat infection if there have been fewer than 7 episodes in the past year or fewer than 5 episodes per year in the past 2 years or fewer than 3 episodes per year in the past 3 years; (2) assessing the child with recurrent throat infection who does not meet criteria in statement 2 for modifying factors that may nonetheless favor tonsillectomy, which may include but are not limited to multiple antibiotic allergy/intolerance, periodic fever, aphthous stomatitis, pharyngitis and adenitis, or history of peritonsillar abscess; (3) asking caregivers of children with sleep-disordered breathing and tonsil hypertrophy about comorbid conditions that might improve after tonsillectomy, including growth retardation, poor school performance, enuresis, and behavioral problems; (4) counseling caregivers about tonsillectomy as a means to improve health in children with abnormal polysomnography who also have tonsil hypertrophy and sleep-disordered breathing; (5) counseling caregivers that sleep-disordered breathing may persist or recur after tonsillectomy and may require further management; (6) advocating for pain management after tonsillectomy and educating caregivers about the importance of managing and reassessing pain; and (7) clinicians who perform tonsillectomy should determine their rate of primary and secondary posttonsillectomy hemorrhage at least annually. The panel offered options to recommend tonsillectomy for recurrent throat infection with a frequency of at least 7 episodes in the past year or at least 5 episodes per year for 2 years or at least 3 episodes per year for 3 years with documentation in the medical record for each episode of sore throat and 1 or more of the following: temperature >38.3°C, cervical adenopathy, tonsillar exudate, or positive test for group A β-hemolytic streptococcus.

The PFAPA syndrome in oral medicine: differential diagnosis and treatment.

Pinto A, Lindemeyer RG, Sollecito TP
Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics •

Periodic fever syndromes in children can present with orofacial manifestations which contribute to the formulation of a differential diagnosis. Because of the early onset of oral lesions in these syndromes, the oral medicine practitioner may be required to assist in their diagnosis and management. The periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome has been classified as a type of periodic fever syndrome ocurring in early childhood. This article presents a review of the published literature on this entity and summarizes current therapeutic options.