A40: Periodic Fever Syndromes in Canada: Results from a National Surveillance Program
Bibliographic record
Abstract
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.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.001 |
| Bibliometrics | 0.003 | 0.007 |
| Science and technology studies | 0.002 | 0.000 |
| Scholarly communication | 0.001 | 0.000 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.002 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".