The level of awareness of the medical community with Marshall syndrome (PFAPA)
DOI:
https://doi.org/10.15574/SP.2024.6(142).3541Keywords:
Marshall syndrome, PFAPA syndrome, diagnostics, treatmentAbstract
Syndrome of periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA - periodic fever, aphthous stomatitis, pharyngitis, adenitis), known as Marshall syndrome (SM.). In Ukraine, with its child population of about 7.6 million. it should prognostically be established in at least several thousand cases, but reports of such children are only sporadic, attesting to diagnostic problems.
Aim - to evaluate the level of knowledge of doctors of certain specialties regarding the diagnosis of SM and finding a way to improve the efficiency of its management.
Materials and methods. An anonymous survey of 157 residents and interns of the Ternopil region of Ukraine was conducted regarding the diagnosis of SM and its management using a written questionnaire followed by statistical processing. Exclusion criteria were the absence of answers or the number of answers to at least one of the questions asked was greater than the suggested number. Therefore, 139 questionnaires were taken into analysis, among which 56 (40.29%) were provided by specialist doctors and 83 (59.71%) by intern doctors.
Results. The analysis of the answers showed difficulties not only with establishing the correct diagnosis, but also with its diagnosis and treatment tactics both among specialists with experience and among interns. About quarter of the residents (13/56 (23.21%)) and only 8/83 (9.64%) of the interns decided on the treatment tactics of the patient in accordance with the described clinical picture and the specified diagnosis, which testified to a lack of understanding of the pathogenetic bases of SM. Despite the fact that the clinical symptoms of MS most interns (67/83; 80.72%) are known and they recognized it based on the description provided by the questionnaire. The biggest difficulties arose in both groups when solving the issue of planning preventive measures in the inter-attack period.
Conclusions. Thus, the analysis of the conducted survey revealed a lack of knowledge about MS, its diagnosis, treatment and rehabilitation. Autoinflammatory diseases need their place in modern programs of university training and especially postgraduate education in the continuous professional development system in order to direct the vector of medical opinion clearly in the direction of the correct clinical diagnosis.
The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institutions. The informed consent of the patient was obtained for conducting the studies.
No conflict of interests was declared by the authors.
References
Amarilyo G, Rothman D, Manthiram K, Edwards KM, Li SC, Marshall GS et al. (2020, Apr 15). Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group. Pediatr Rheumatol Online J. 18(1): 31. https://doi.org/10.1186/s12969-020-00424-x; PMid:32293478 PMCid:PMC7157990
Boyarchuk O, Lewandowicz-Uszyńska A, Kinash M, Haliyash N, Sahal I, Kovalchuk T. (2018). Physicians' awareness concerning primary immunodeficiencies in the Ternopil Region of Ukraine. Pediatria Polska. Polish Journal of Paediatrics. 93(3): 221-228. https://doi.org/10.5114/polp.2018.77435
Boyarchuk O, Volyanska L, Kosovska T, Lewandowicz-Uszynska A, Kinash M. (2018, Dec). Awareness of primary immunodeficiency diseases among medical students. Georgian Med News. (285): 124-130.
Burbela E, Volianska L, Boyarchuk O. (2021). Clinical features and diagnosis of PFAPA syndrome: approach of the primary care physician. Pediatria Polska - Polish Journal of Paediatrics. 96(3): 168-172. https://doi.org/10.5114/polp.2021.109301
Forsvoll J, Kristoffersen EK, Oymar K. (2013, Feb). Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study. Acta Paediatr. 102(2): 187-192. Epub 2012 Nov 27. https://doi.org/10.1111/apa.12069; PMid:23106338
Foster KJ, Rodrigues J, Lee J, Powell S, Azmeh R et al. (2018). Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome at a Single Children's Hospital. Int J Aller Medications. 4: 034. https://doi.org/10.23937/2572-3308.1510034
Goel N, Kashyap V, Saini I, Sharma S, Singh A. (2023). Periodic Fever in Children: A Report of Three Unusual Cases. J Compr Ped. 14(2): e130982. https://doi.org/10.5812/compreped-130982
Harel L, Hashkes PJ, Lapidus S, Edwards KM, Padeh S, Gattorno M, Marshall GS. (2018, Feb). The First International Conference on Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome. J Pediatr. 193: 265-274.e3. Epub 2017 Dec 12. https://doi.org/10.1016/j.jpeds.2017.10.034; PMid:29246466
Hausmann J, Dedeoglu F, Broderick L. (2023, Jun). Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome and Syndrome of Unexplained Recurrent Fevers in Children and Adults. J Allergy Clin Immunol Pract. 11(6): 1676-1687. Epub 2023 Mar 21. https://doi.org/10.1016/j.jaip.2023.03.014; PMid:36958521
Hofer M. (2020, Jun). Why and How Should We Treat Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome? Paediatr Drugs. 22(3): 243-250. https://doi.org/10.1007/s40272-020-00393-4; PMid:32342289
Mamchych TI, Olenko AIa, Osypchuk MM, Shportiuk VH. (2006). Statystychnyi analiz danykh z paketom STATISTICA. Drohobych: Vidrodzhennia: 208. ISBN 966-538-161-X.
Marshall GS, Edwards KM, Butler J, Lawton AR. (1987, Jan). Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr. 110(1): 43-46. https://doi.org/10.1016/S0022-3476(87)80285-8; PMid:3794885
Rigante D, Vitale A, Natale MF, Lopalco G, Andreozzi L, Frediani B et al. (2017, Feb). A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome. Clin Rheumatol. 36(2): 463-468. Epub 2016 Jun 1. https://doi.org/10.1007/s10067-016-3317-7; PMid:27251674
Veres T, Amarilyo G, Abu Ahmad S, Abu Rumi M, Brik R, Hezkelo N et al. (2022) Familial Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis Syndrome; Is It a Separate Disease? Front. Pediatr. 9: 800656. https://doi.org/10.3389/fped.2021.800656; PMid:35310141 PMCid:PMC8929572
Wang A, Manthiram K, Dedeoglu F, Licameli GR. (2021, Jun 27). Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A review. World J Otorhinolaryngol Head Neck Surg. 7(3): 166-173. https://doi.org/10.1016/j.wjorl.2021.05.004; PMid:34430824 PMCid:PMC8356195
Więsik-Szewczyk E, Wolska-Kuśnierz B, Jahnz-Różyk K. (2019). Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome persisting to adulthood - an example of a diagnostic and therapeutic challenge. Reumatologia. 57(5): 292-296. Epub 2019 Oct 31. https://doi.org/10.5114/reum.2019.89523; PMid:31844343 PMCid:PMC6911254
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