A40: Periodic Fever Syndromes in Canada: Results from a National Surveillance Program
Notice bibliographique
Résumé
Background/Purpose: To estimate the incidence of periodic fever syndromes in the Canadian paediatric population, to describe the patterns of presentation, and to raise awareness in the medical community. Methods: This study was initiated though the Canadian Pediatric Surveillance Program (CPSP), and is being carried out over a three year period which will conclude in September 2014. The case definition includes patients less than 18 years of age presenting with a newly diagnosed periodic fever syndrome. Conditions under surveillance included Familial Mediterranean fever (FMF), Tumor necrosis factor receptor‐associated periodic syndrome (TRAPS), Hyperimmunoglobulin D syndrome (HIDS), Cryopyrin‐associated periodic syndromes (CAPS), Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA), and Undefined periodic fever syndromes. For each condition the study protocol outlined established clinical and/or genetic criteria for diagnosis. Participating pediatricians and pediatric subspecialists across the country are sent monthly reporting forms. Detailed questionnaires are completed by physicians who report a recent diagnosis. Submissions are screened for accuracy and confirmed cases then entered into a database. Results: During the 20 month period from September 2011 to December 2012 a total of 167 cases were reported. Of these cases, detailed questionnaires were completed on 126, with the remainder pending at the time of this report. 114 cases have been confirmed as meeting the criteria for one of the Periodic Fever Syndromes under surveillance, 55% of which had had genetic testing completed as part of their evaluation. Twelve cases were discarded. Amongst the 114 confirmed cases, PFAPA was most frequent (51%), followed by Undefined Periodic fever syndrome (37%). Also seen were FMF (11%), and one case of HIDS (1%). There were no cases of TRAPS, or CAPS, reported during the study period to date. Amongst the 42 Undefined Periodic Fever syndrome cases the reporting physicians described 11 as being suspected PFAPA, 5 suspected FMF, 3 suspected CAPS, and 1 suspected HIDS. In most cases this was because genetic testing was still pending at the time of reporting. The overall male to female ratio was 1.1:1. Amongst Undefined cases there was a male predominance with a male to female ratio of 1.6:1, whereas the male to female ratio was 0.6:1 for FMF. An equal number of males and females were reported for PFAPA. The most frequent fever associated manifestations for PFAPA included pharyngitis (79%), cervical lymphadenopathy (66%), stomatitis (59%), and fatigue (53%). For FMF most frequent were abdominal pain (69%), arthralgia (54%), pharyngitis (46%), and headache (46%). For Undefined cases most frequent manifestations were fatigue (45%), pharyngitis (38%), abdominal pain (36%), and lymphadenopathy (33%). Conclusion: Although rare periodic fever syndromes are identified across Canada each year. PFAPA, Undefined fever syndromes, and FMF are most frequently seen. We hope to have a more complete picture of the full spectrum and incidence of these autoinflammatory diseases in the Canadian population as genetic test results become available, and additional cases are identified through the end of the study in September 2014.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,001 | 0,003 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,001 |
| Bibliométrie | 0,003 | 0,007 |
| Études des sciences et des technologies | 0,002 | 0,000 |
| Communication savante | 0,001 | 0,000 |
| Science ouverte | 0,001 | 0,001 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,002 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».