Article
Medicine, Research & Experimental
Uche C. Ezeh, Philip J. Kahn, Max M. April
Summary: This study presented two children diagnosed with PFAPA syndrome and treated with ITA. The procedure was successful in eliminating symptoms and had no complications in the one-year follow-up.
Article
Pediatrics
Gulcin Otar Yener, Ilke Aktas, Ceren Altintas Mese, Mustafa Cakan
Summary: The study aimed to investigate the treatment modalities for periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and examine the efficacy and safety of colchicine in PFAPA patients. It also explored the impact of MEFV gene sequence variants on the clinical course and response to colchicine. The findings suggest that colchicine is an effective and safe treatment for PFAPA, regardless of the presence of MEFV gene mutations.
EUROPEAN JOURNAL OF PEDIATRICS
(2023)
Review
Rheumatology
Kalpana Manthiram
Summary: This review summarizes recent findings on PFAPA syndrome, including the discovery of genetic susceptibility loci associated with PFAPA syndrome and the efficacy of tonsillectomy in treating recurrent stereotypical fever. These findings highlight the heterogeneous phenotypes related to PFAPA that may be linked to common genetic susceptibility or response to therapy.
CURRENT OPINION IN RHEUMATOLOGY
(2023)
Article
Anesthesiology
Christoph Czarnetzki, Eric Albrecht, Jules Desmeules, Christian Kern, Jean-Baptiste Corpataux, Sylvain Gander, Sander M. J. van Kuijk, Martin R. Tramer
Summary: Dexamethasone is widely used for the prevention of postoperative nausea and vomiting (PONV), but its efficacy for the treatment of established PONV is limited.
EUROPEAN JOURNAL OF ANAESTHESIOLOGY
(2022)
Article
Pediatrics
Mor Broide, Yoel Levinsky, Rotem Tal, Liora Harel, Shoval Shoham, Sabreen Abu Ahmad, Yonatan Butbul Aviel, Gil Amarilyo
Summary: Objective Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. Some patients with PFAPA experience more frequent attacks, less than 2 weeks, known as extreme PFAPA (ePFAPA). This study aims to characterize the demographic and clinical features of ePFAPA and compare them to non-extreme PFAPA (nPFAPA) patients.
PEDIATRIC RHEUMATOLOGY
(2023)
Article
Pediatrics
Kristen L. Dammeyer, Amanda Schneider, Max M. April, Philip J. Kahn
Summary: This is the first report of siblings with near simultaneous onset of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) followed by synchronous disease flares. The flares were characterized by fever, aphthous ulceration, cervical lymphadenitis, and the absence of infection. Tonsillectomy resulted in dramatic resolution of episodes in the older sibling.
PEDIATRIC RHEUMATOLOGY
(2022)
Article
Rheumatology
Mariko Hara, Noriko Morimoto, Takahisa Watabe, Naho Morisaki, Kenji Matsumoto
Summary: This study evaluated the clinical factors associated with the outcome of tonsillectomy in children with PFAPA syndrome. Results showed that patients with late onset and headache responded well to the surgery.
INTERNATIONAL JOURNAL OF RHEUMATIC DISEASES
(2023)
Article
Pediatrics
Elif Kilic Konte, Fatih Haslak, Mehmet Yildiz, Neslihan Gucuyener, Ipek Ulkersoy, Aybuke Gunalp, Esma Aslan, Amra Adrovic, Sezgin Sahin, Kenan Barut, Ozgur Kasapcopur
Summary: This study aimed to investigate the demographic and clinical characteristics of children with both FMF and PFAPA syndromes and their response to colchicine and tonsillectomy. The results showed that patients with tonsillopharyngitis, aphthous stomatitis, and PFAPA family history were more likely to be colchicine-resistant and tonsillectomy responsive, while those with exon 10 MEFV gene mutations were more prone to have a favorable response to colchicine.
EUROPEAN JOURNAL OF PEDIATRICS
(2023)
Article
Pediatrics
Takuma Ohnishi, Satoshi Sato, Yoji Uejima, Yutaka Kawano, Eisuke Suganuma
Summary: This study retrospectively investigated the clinical characteristics and treatment efficacy of Japanese children with PFAPA syndrome. The results showed that pharyngitis is the most frequent symptom associated with fever, and cimetidine is a safe and effective initial treatment option.
PEDIATRICS INTERNATIONAL
(2022)
Article
Dermatology
Thomas Bettuzzi, Marie Jachiet, Emilie Sbidian, Laure Frumholtz, Florence Cordoliani, Luc Mouthon, Francois Chasset, Romain Paule, Jean-David Bouaziz, Loic Guillevin, Benjamin Terrier, Selim Aractingi, Nicolas Dupin, Alexis Regent
Summary: This study retrospectively analyzed the treatment efficacy and safety in patients with cutaneous polyarteritis nodosa. The results showed that colchicine and glucocorticoids + azathioprine were effective treatment options, while patients with peripheral sensory neuropathy had a lower treatment response.
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2022)
Review
Medicine, General & Internal
Stefano Avondo, Alessandro Andreis, Matteo Casula, Giuseppe Biondi-Zoccai, Massimo Imazio
Summary: Recurrence is a common complication following acute pericarditis, with available treatments including aspirin, NSAIDs, colchicine, glucocorticoids, immunosuppressive agents, immunoglobulins, and anti-IL1 agents. Correct pharmacological management is essential in preventing recurrences, with colchicine being the mainstay of treatment and anti-IL1 agents as a valuable option for refractory cases of recurrent pericarditis.
Review
Pharmacology & Pharmacy
Nicholas C. Schwier, Janice Tsui, Jordan A. Perrine, Corey M. Guidry, Julia Mathew
Summary: Management of pericarditis has significantly updated since the publication of the 2015 European Society of Cardiology Guidelines, with an increasing use of pharmacotherapies for various causes of pericarditis. High-dose anti-inflammatory therapies should be used to manage symptoms, while colchicine is recommended as adjuvant therapy to lower the risk of recurrence and improve morbidity. Glucocorticoids and immunotherapies are reserved as second- and third-line treatments, respectively, with limited evidence supporting their use in certain populations. Pharmacovigilance strategies are essential for balancing efficacy and safety in managing pericarditis.
Article
Multidisciplinary Sciences
Avishai Maliah, Roma Parikh, Oshrat E. Tayer-Shifman, Oded Kimhi, Raz Gepstein, Tami Halperin, Yair Levy, Carmit Levy, Yael Pri-Paz Basson, Shaye Kivity
Summary: This study assessed the impact of prolonged steroid treatment on the T-cell and humoral response to the mRNA vaccine in patients with systemic autoimmune rheumatic disease. The results showed that high-dose long-term steroid treatment weakened the CD4 T-cell response to the SARS-CoV-2 spike peptide, while the humoral response was not affected. This suggests that high-dose steroid treatment inhibits the T-cell response to the vaccine.
SCIENTIFIC REPORTS
(2022)
Article
Genetics & Heredity
Karin Rydenman, Carina Sparud-Lundin, Anna Karlsson-Bengtsson, Stefan Berg, Anders Fasth, Per Wekell
Summary: This study investigated the impact of PFAPA syndrome on the families of affected children and the health-related quality of life (HRQOL) of children with the syndrome. The results showed that PFAPA syndrome has a negative impact on the families and tonsillectomy can improve the HRQOL of affected children.
ORPHANET JOURNAL OF RARE DISEASES
(2023)
Review
Medicine, General & Internal
Carina Wagner, Mirko Griesel, Agata Mikolajewska, Maria-Inti Metzendorf, Anna-Lena Fischer, Miriam Stegemann, Manuel Spagl, Avinash Anil Nair, Je L. Erson Daniel, Falk Fichtner, Nicole Skoetz
Summary: This study aims to evaluate the effectiveness and safety of systemic corticosteroids in the treatment of people with COVID-19, explore equity-related aspects in specific populations, and keep up with the evolving evidence base using a living systematic review approach.
COCHRANE DATABASE OF SYSTEMATIC REVIEWS
(2022)