{ "vercy": "1.0-draft", "publication": { "status": "research-draft", "adjudicationStatus": "unreviewed", "publishableCanonical": false, "generatedAt": "2026-09-12T12:01:13Z", "providers": [ "Codex" ], "breadth": "recalled by Codex without web access - no source was read", "missingProviders": [], "pass": 2, "cost": { "grok": { "seconds": 31.3, "error": "Reading additional input from stdin...\nOpenAI Codex v0.153.4\n--------\nworkdir: R:\\02_PROJECTS\\02_Meta_Models_Platforms\\Ver.cy\\current\\thing-registry-backlog\nmodel: gpt-6-astra\nprovider: openai\napproval: never\nsandbox: read-only\nreasoning effort: none\nreasoning summaries: none\nsession id: 01a0957d-2b2b-76c2-a30d-5b85fefe588f\n--------\nuser\nDescribe what is already known about one registered thing. Answer as JSON only, no prose around it.\n\nThing: periodic fever syndrome\nSense to describe: (none recorded)\nDomain code: XCT.STA\nAlso known as: (none)\n\n\nContext for this batch of 1001 things:\n# Batch 0", "usd": 0.0, "recall": true }, "codex": { "seconds": 70.2, "error": "Reading additional input from stdin...\nOpenAI Codex v0.153.4\n--------\nworkdir: R:\\02_PROJECTS\\02_Meta_Models_Platforms\\Ver.cy\\current\\thing-registry-backlog\nmodel: gpt-6-astra\nprovider: openai\napproval: never\nsandbox: read-only\nreasoning effort: none\nreasoning summaries: none\nsession id: 01a0957d-2b3b-79a0-9d6f-6eeab2e95051\n--------\nuser\nYou are drafting a Vercy meta-model for one registered thing. Answer as JSON only, no prose around it.\n\nThing: periodic fever syndrome\nRegistry id: vr.tr.periodic-fever-syndrome\nPlane / domain: XCT / XCT.STA\nRegistry definition: (none recorded)\nNames folded in" } }, "enrichment": { "pass": 3, "engine": "claude", "producedAt": "2026-10-06T12:00:00Z", "ingestedAt": "2026-10-06T08:09:42+00:00", "fields": [ "agent_conduct", "ethics", "failure_modes_and_hazards", "owners", "standards_and_regulation" ] }, "gate": { "passed": true, "version": "1.0", "at": "2026-10-07T10:10:30+00:00", "evidence": [ "completeness 1.00", "sense: Wikidata description", "2 live sources", "review by codex: pass" ] } }, "metaModel": { "id": "THING-Q3278145", "registryId": "vr.tr.periodic-fever-syndrome", "name": "periodic fever syndrome", "version": "0.1.0-research.1", "entryKind": "thing", "family": "Thing Registry", "domain": [ "XCT.STA" ], "status": "research-draft" }, "canonicalUrl": "https://ver.cy/models/thing/q3278145/", "model": { "registry_id": "vr.tr.periodic-fever-syndrome", "name": "periodic fever syndrome", "purpose": "Enable an agent to describe, distinguish and track a periodic fever syndrome while preserving uncertainty about its subtype, diagnostic basis and implications for care.", "definition": "Periodic fever syndrome denotes a group of disorders characterised by recurrent episodes of fever and systemic inflammation, usually involving innate immune dysregulation, with inherited and non-inherited forms and variable inflammation between attacks.", "scope_statement": "This model owns the syndrome-level representation of vr.tr.periodic-fever-syndrome in XCT / XCT.STA: recurrent fever patterns, associated inflammatory features, diagnostic evidence, subtype relationships and longitudinal disease state, distinct from the person, individual attack and care encounter.", "in_scope": [ "Working definition and boundaries of the periodic fever syndrome umbrella", "Attack timing, duration, associated manifestations and intercritical state", "Evidence supporting or challenging an autoinflammatory explanation", "Subtype attribution, family history and interpretation of genetic evidence", "Versioned diagnostic or classification criteria and terminology mappings", "Longitudinal activity, complications and clinician-directed management constraints" ], "out_of_scope": [ "The patient's complete medical and demographic record", "Individual fever episodes as independently managed clinical events", "Infectious, malignant, autoimmune and other alternative conditions as full disease models", "Laboratory assays and genetic variants as independently specified things", "Drug specifications, prescribing protocols and treatment encounters", "Separate registry publications for subtypes without an explicit granularity decision" ], "distinguishing_features": [ "Determine whether fever recurs in discrete attacks with a characterisable interval state, rather than representing one prolonged febrile illness.", "Compare manifestations across attacks for a reproducible syndrome pattern; regular timing alone does not establish the diagnosis.", "Record evidence for and against recurrent infection and other explanations before attributing repeated fever to a periodic fever syndrome.", "Use associated manifestations, onset, family history and interpreted genetic findings to distinguish candidate subtypes; do not require a genetic result for the umbrella label.", "Distinguish syndrome identification from an unexplained recurrent-fever symptom label by recording the clinician's attribution and the criteria or reasoning supporting it." ], "characteristics": [ { "name": "Syndrome attribution", "kind": "state", "unit_or_values": "suspected | clinician-established | disputed | historical | unresolved", "why_it_matters": "Separates diagnostic confidence from the presence of recurrent fever." }, { "name": "Subtype attribution", "kind": "relation", "unit_or_values": "candidate or established subtype, supporting evidence, attribution date and responsible clinician", "why_it_matters": "Preserves clinically meaningful distinctions within an umbrella registry entry." }, { "name": "Age at first compatible attack", "kind": "measurement", "unit_or_values": "months or years, with onset-date uncertainty", "why_it_matters": "Supports comparison with subtype-specific presentations without treating onset age as sufficient evidence." }, { "name": "Attack duration", "kind": "measurement", "unit_or_values": "hours or days per observed attack; range and observation period", "why_it_matters": "Makes the temporal phenotype comparable across episodes." }, { "name": "Attack recurrence", "kind": "measurement", "unit_or_values": "attacks per observation period and onset-to-onset interval in days, with variability", "why_it_matters": "Distinguishes frequent, irregular and approximately regular recurrence without assuming clockwork periodicity." }, { "name": "Fever documentation", "kind": "measurement", "unit_or_values": "temperature in degrees Celsius, measurement site, method, time and reported versus measured status", "why_it_matters": "Separates documented fever from subjective reports and measurement differences." }, { "name": "Attack-associated manifestations", "kind": "category", "unit_or_values": "observed, absent, unknown or unassessed manifestations, including oral, pharyngeal, lymph-node, skin, abdominal, chest, joint and eye features", "why_it_matters": "Captures combinations that can help distinguish candidate syndromes." }, { "name": "Intercritical inflammatory state", "kind": "state", "unit_or_values": "symptom and laboratory status between attacks, with dates and reference ranges", "why_it_matters": "Prevents symptom-free intervals from being equated automatically with absence of inflammation." }, { "name": "Inflammatory marker profile", "kind": "measurement", "unit_or_values": "marker-specific value, laboratory unit and reference range, linked to attack phase and treatment exposure", "why_it_matters": "Allows inflammatory evidence to be interpreted in its temporal context." }, { "name": "Familial and molecular evidence", "kind": "relation", "unit_or_values": "pedigree observations and genetic reports with gene, variant, zygosity, interpretation, laboratory and report date", "why_it_matters": "Keeps family resemblance and molecular evidence distinct from a confirmed causal diagnosis." }, { "name": "Longitudinal burden", "kind": "measurement", "unit_or_values": "attack days, missed school or work days, functional impact and assessment period", "why_it_matters": "Represents disease burden beyond the number of recorded visits." } ], "affordances": [ "Assemble an attack timeline and compare manifestations across attacks and intervals.", "Compare documented evidence with an explicitly sourced and versioned subtype criteria set.", "Identify missing observations or unresolved alternative explanations for clinician review.", "Track disease activity, functional burden and relevant complication surveillance over time.", "Link clinician-authorised management plans to their subtype assumptions and reassessment conditions.", "Map the syndrome to terminology systems while retaining release versions and mapping uncertainty." ], "agent_conduct": { "may": [ "Explain periodic fever syndromes in general terms, citing medical sources.", "Advise people with recurrent unexplained fevers to see a clinician." ], "must_not": [ "Diagnose a periodic fever syndrome in a specific person.", "Recommend starting, stopping or changing treatment.", "Disclose a named person's diagnosis or genetic test results without consent." ], "requires_human": [ "Any decision on genetic testing or treatment, especially for a child." ] }, "ethics": { "considerations": [ "Rare conditions are often missed for years, which affects patients and families.", "Genetic findings can affect relatives, insurance and employment." ], "affected_parties": [ "Patients and families", "Children", "Relatives with shared genes" ] }, "owners": { "steward": "The patient's treating clinician; each person answers for their own health records.", "master_systems": [ "Rare disease registries" ] }, "failure_modes_and_hazards": [ "Untreated inflammation in some forms can lead to organ damage such as amyloidosis.", "Delayed diagnosis leads to repeated unnecessary treatments." ], "standards_and_regulation": [ "International Classification of Diseases, World Health Organization." ], "what_it_is_for": [ "Periodic fever syndromes are encountered when recurrent fever and inflammatory episodes prompt clinical assessment over time.", "The term supports documenting attack patterns, associated symptoms and family history while distinguishing possible subtypes and alternative explanations." ], "facets_not_applicable": { "recognition": "A periodic fever syndrome has no defining visual appearance and requires clinical evidence across episodes to distinguish it from other causes of recurrent fever." } }, "sources": [ { "id": "wikidata", "title": "Wikidata item Q3278145: periodic fever syndrome", "url": "https://www.wikidata.org/wiki/Q3278145", "url_status": "live", "what_it_supports": "identity and sense of the item", "checked_by": "wikidata-api" }, { "id": "wikipedia-en", "title": "Wikipedia: Periodic fever syndrome", "url": "https://en.wikipedia.org/wiki/Periodic_fever_syndrome", "url_status": "live", "what_it_supports": "general description of the item", "checked_by": "wikidata-api sitelink" } ], "structure": { "bundles": [ { "id": "syndrome-boundary", "name": "Syndrome boundary and attribution", "description": "Defines what the umbrella label means in this record and how it relates to narrower syndromes and symptom labels.", "rationale": "Periodic fever syndrome can be used at different levels of specificity, so its intended meaning must remain explicit.", "layers": [ { "id": "umbrella-meaning", "name": "Umbrella meaning", "description": "Establishes the inclusion boundary of the registered thing.", "findings": [ { "id": "recurrent-fever-versus-syndrome", "name": "Recurrent fever versus syndrome", "description": "Record the evidence that supports a syndrome attribution beyond recurrent fever alone.", "questions": [ { "text": "Which sourced definition governs the use of periodic fever syndrome here?", "kind": "definition", "id": "recurrent-fever-versus-syndrome-q01" }, { "text": "What evidence supports this syndrome label rather than an unresolved recurrent-fever symptom label?", "kind": "boundary", "id": "recurrent-fever-versus-syndrome-q02" } ] } ] }, { "id": "subtype-and-coding", "name": "Subtype and coding", "description": "Maintains narrower clinical attributions and versioned terminology relationships.", "findings": [ { "id": "qualified-subtype-mapping", "name": "Qualified subtype mapping", "description": "Represent subtype and coding assignments with their scope, source and certainty rather than assuming a single universal code.", "questions": [ { "text": "Is the attribution to an umbrella syndrome, a named subtype or an unresolved group of candidates?", "kind": "boundary", "id": "qualified-subtype-mapping-q01" }, { "text": "Which ICD or SNOMED CT edition, release and jurisdiction support each mapping, and is it exact or broader than the clinical attribution?", "kind": "provenance", "id": "qualified-subtype-mapping-q02" } ] } ] } ] }, { "id": "attack-phenotype", "name": "Attack and interval phenotype", "description": "Describes the repeating clinical pattern and what happens between attacks.", "rationale": "Temporal structure and associated manifestations are central to recognising and differentiating periodic fever presentations.", "layers": [ { "id": "attack-timing", "name": "Attack timing", "description": "Captures fever documentation, attack boundaries and recurrence variability.", "findings": [ { "id": "observed-recurrence-pattern", "name": "Observed recurrence pattern", "description": "Derive the temporal phenotype from dated observations while retaining missing data and uncertain attack boundaries.", "questions": [ { "text": "How were fever, attack onset and attack resolution measured or reported?", "kind": "measurement", "id": "observed-recurrence-pattern-q01" }, { "text": "What are the observed attack durations and onset-to-onset intervals, and how much do they vary?", "kind": "measurement", "id": "observed-recurrence-pattern-q02" } ] } ] }, { "id": "manifestations-and-intervals", "name": "Manifestations and intervals", "description": "Separates recurring attack features from persistent symptoms or inflammation.", "findings": [ { "id": "phase-linked-clinical-pattern", "name": "Phase-linked clinical pattern", "description": "Link manifestations and laboratory results to attack phase instead of flattening them into a timeless symptom list.", "questions": [ { "text": "Which oral, pharyngeal, lymph-node, skin, serosal, joint or eye manifestations recur together during documented attacks?", "kind": "measurement", "id": "phase-linked-clinical-pattern-q01" }, { "text": "Between attacks, which symptoms and inflammatory markers normalise, persist or remain unmeasured?", "kind": "measurement", "id": "phase-linked-clinical-pattern-q02" } ] } ] } ] }, { "id": "diagnostic-evidence", "name": "Diagnostic evidence and competing explanations", "description": "Makes clinical reasoning, criteria use and unresolved alternatives inspectable.", "rationale": "Repeated fever and inflammatory findings require interpretation; neither establishes a particular syndrome by itself.", "layers": [ { "id": "criteria-basis", "name": "Criteria basis", "description": "Records the intended use and applicability of each criteria set.", "findings": [ { "id": "versioned-criteria-assessment", "name": "Versioned criteria assessment", "description": "Distinguish diagnostic reasoning from research classification and preserve assessed, absent and unknown criteria.", "questions": [ { "text": "Which issuing body, publication and revision define the criteria used, and are they diagnostic or classification criteria?", "kind": "provenance", "id": "versioned-criteria-assessment-q01" }, { "text": "Which criteria are met, not met or unassessed, and does the criteria set apply to this age group and presentation?", "kind": "boundary", "id": "versioned-criteria-assessment-q02" } ] } ] }, { "id": "alternative-explanations", "name": "Alternative explanations", "description": "Tracks evidence for other causes of recurrent or apparently periodic fever.", "findings": [ { "id": "differential-resolution", "name": "Differential resolution", "description": "Record clinician assessment of alternatives, including recurrent infection and cyclic neutropenia, without equating an absent test with exclusion.", "questions": [ { "text": "Which infectious, haematological, malignant, autoimmune or other explanations were considered, and what evidence supports their current status?", "kind": "provenance", "id": "differential-resolution-q01" }, { "text": "What change in the fever pattern or associated findings requires renewed clinical assessment of those alternatives?", "kind": "action", "id": "differential-resolution-q02" } ] } ] } ] }, { "id": "aetiology-and-context", "name": "Aetiology, inheritance and population context", "description": "Represents proposed causal attribution and the limits of familial, molecular and epidemiological evidence.", "rationale": "An umbrella syndrome label must not imply that every presentation has the same mechanism, inheritance or population distribution.", "layers": [ { "id": "familial-molecular-evidence", "name": "Familial and molecular evidence", "description": "Connects family patterns and genetic reports to qualified subtype hypotheses.", "findings": [ { "id": "causal-evidence-interpretation", "name": "Causal evidence interpretation", "description": "Retain the distinction between a detected variant, its laboratory classification and its clinical explanatory value.", "questions": [ { "text": "What family history and molecular findings support the proposed subtype or inheritance pattern?", "kind": "provenance", "id": "causal-evidence-interpretation-q01" }, { "text": "How were negative results, uncertain variants, test limitations and phenotype compatibility interpreted by the responsible clinician?", "kind": "boundary", "id": "causal-evidence-interpretation-q02" } ] } ] }, { "id": "onset-and-population", "name": "Onset and population context", "description": "Places onset and epidemiological claims within explicitly defined populations.", "findings": [ { "id": "contextualised-occurrence", "name": "Contextualised occurrence", "description": "Keep population-level occurrence estimates separate from individual diagnostic evidence.", "questions": [ { "text": "What was the age at first compatible attack, and how reliable is that onset history?", "kind": "measurement", "id": "contextualised-occurrence-q01" }, { "text": "For any prevalence claim, which subtype definition, population, geography, study year and ascertainment method were used?", "kind": "provenance", "id": "contextualised-occurrence-q02" } ] } ] } ] }, { "id": "course-and-management", "name": "Course, consequences and management state", "description": "Tracks longitudinal burden and the evidence underlying clinician-directed actions.", "rationale": "Recognition alone is insufficient: an agent must preserve the distinction between current activity, cumulative consequences and an authorised care plan.", "layers": [ { "id": "longitudinal-course", "name": "Longitudinal course", "description": "Tracks changes in attacks, interval inflammation and functional impact.", "findings": [ { "id": "activity-and-consequences", "name": "Activity and consequences", "description": "Record observed course and subtype-relevant complications without assigning a uniform progression to the umbrella condition.", "questions": [ { "text": "Over a defined interval, how have attack frequency, attack days, interval inflammation and daily functioning changed?", "kind": "measurement", "id": "activity-and-consequences-q01" }, { "text": "Which complications, including AA amyloidosis where relevant, warrant surveillance for the attributed subtype, and what source supports that relevance?", "kind": "provenance", "id": "activity-and-consequences-q02" } ] } ] }, { "id": "management-and-reassessment", "name": "Management and reassessment", "description": "Links recorded interventions and response assessments to clinical authority and subtype assumptions.", "findings": [ { "id": "qualified-management-response", "name": "Qualified management response", "description": "Represent treatment intent, observed response and reassessment conditions without treating response as automatic diagnostic proof.", "questions": [ { "text": "What clinician-authorised plan applies to attacks, prevention and monitoring, and which subtype attribution supports it?", "kind": "action", "id": "qualified-management-response-q01" }, { "text": "How were benefit, adverse effects and nonresponse assessed, and what findings trigger review of the diagnosis or plan?", "kind": "action", "id": "qualified-management-response-q02" } ] } ] } ] }, { "id": "evidence-and-external-alignment", "name": "Evidence and external alignment", "description": "What the world already says about this thing, gathered so the model can be checked against it.", "rationale": "A model that cannot be lined up against existing standards, identifiers and practice cannot be adopted by anyone who already uses them.", "layers": [ { "id": "reported-evidence", "name": "Reported evidence", "description": "Findings from the breadth pass, kept separate from the structural claims.", "findings": [ { "id": "evidence-confidence-notes", "name": "Check these first", "description": "Recalled without web access and unsourced; every item is a lead to verify.", "evidence": [ "The unqualified registry label is ambiguous between the broad periodic-fever grouping and hereditary periodic fever syndromes; confirm the intended scope.", "CAPS can produce chronic or cold-triggered inflammation rather than regularly spaced fever attacks, so inclusion depends on the grouping used.", "This is recall without source verification; check current classification versions, coding assignments and syndrome-specific criteria before publication." ], "questions": [ { "id": "evidence-confidence-notes-q01", "text": "Which of these check these first hold for the sense of periodic fever syndrome this model covers, and on what evidence?", "kind": "provenance" } ] }, { "id": "evidence-kinds", "name": "Kinds and varieties", "description": "Recalled without web access and unsourced; every item is a lead to verify.", "evidence": [ "Familial Mediterranean fever (FMF)", "Tumour necrosis factor receptor-associated periodic syndrome (TRAPS)", "Mevalonate kinase deficiency (MKD), including the hyperimmunoglobulinaemia D syndrome phenotype", "Cryopyrin-associated periodic syndromes (CAPS)", "Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)" ], "questions": [ { "id": "evidence-kinds-q01", "text": "Which of these kinds and varieties hold for the sense of periodic fever syndrome this model covers, and on what evidence?", "kind": "provenance" } ] }, { "id": "evidence-identifiers", "name": "Identifiers and schemes", "description": "Recalled without web access and unsourced; every item is a lead to verify.", "evidence": [ { "scheme": "ICD-10", "value_or_pattern": "Category and subcategory codes; assignment depends on the specific syndrome and coding edition", "note": "Verify the WHO edition or national clinical modification before assigning a code to this umbrella term." }, { "scheme": "OMIM", "value_or_pattern": "Six-digit phenotype identifiers", "note": "Applicable to individual inherited syndromes; phenotype records and gene records are distinct." }, { "scheme": "Orphanet", "value_or_pattern": "ORPHA followed by a numeric identifier", "note": "Rare-disease identifiers distinguish specific syndromes and disease groupings." } ], "questions": [ { "id": "evidence-identifiers-q01", "text": "Which of these identifiers and schemes hold for the sense of periodic fever syndrome this model covers, and on what evidence?", "kind": "provenance" } ] }, { "id": "evidence-standards-and-regulation", "name": "Standards and regulation", "description": "Recalled without web access and unsourced; every item is a lead to verify.", "evidence": [ "Eurofever/PRINTO classification criteria for hereditary recurrent fevers and PFAPA; these support classification and are not interchangeable with clinical diagnosis.", "European Alliance of Associations for Rheumatology (EULAR) recommendations for familial Mediterranean fever management." ], "questions": [ { "id": "evidence-standards-and-regulation-q01", "text": "Which of these standards and regulation hold for the sense of periodic fever syndrome this model covers, and on what evidence?", "kind": "provenance" } ] }, { "id": "evidence-real-world-use", "name": "Real-world use", "description": "Recalled without web access and unsourced; every item is a lead to verify.", "evidence": [ "Organising the differential diagnosis of recurrent unexplained fever.", "Selecting syndrome-specific clinical assessment and, where appropriate, genetic testing.", "Guiding syndrome-specific treatment and monitoring.", "Identifying inherited disease relevant to family counselling.", "Classifying participants for autoinflammatory disease registries and studies." ], "questions": [ { "id": "evidence-real-world-use-q01", "text": "Which of these real-world use hold for the sense of periodic fever syndrome this model covers, and on what evidence?", "kind": "provenance" } ] }, { "id": "evidence-measurements", "name": "Typical measurements", "description": "Recalled without web access and unsourced; every item is a lead to verify.", "evidence": [ { "quantity": "Duration of a typical untreated FMF attack", "typical_range": "Approximately 12-72", "unit": "hours" }, { "quantity": "Duration of a typical PFAPA fever episode", "typical_range": "Approximately 3-7", "unit": "days" }, { "quantity": "Systemic inflammatory activity", "typical_range": "C-reactive protein and serum amyloid A commonly rise during attacks; persistent elevation between attacks varies by syndrome and disease control", "unit": "mg/L" } ], "questions": [ { "id": "evidence-measurements-q01", "text": "Which of these typical measurements hold for the sense of periodic fever syndrome this model covers, and on what evidence?", "kind": "provenance" } ] }, { "id": "evidence-failure-modes-and-hazards", "name": "Failure modes and hazards", "description": "Recalled without web access and unsourced; every item is a lead to verify.", "evidence": [ "Mistaking infection, malignancy or another inflammatory disorder for a periodic fever syndrome.", "Delayed recognition causing repeated investigations or unnecessary antibiotic treatment.", "Persistent inflammation causing AA amyloidosis in susceptible syndromes, particularly inadequately controlled FMF.", "Treating a genetic variant of uncertain significance as proof of disease, or treating a negative genetic test as exclusion.", "Assuming all syndromes have clocklike attacks or completely symptom-free intervals." ], "questions": [ { "id": "evidence-failure-modes-and-hazards-q01", "text": "Which of these failure modes and hazards hold for the sense of periodic fever syndrome this model covers, and on what evidence?", "kind": "provenance" } ] }, { "id": "evidence-regional-variation", "name": "Regional variation", "description": "Recalled without web access and unsourced; every item is a lead to verify.", "evidence": [ "FMF is more prevalent in several populations with ancestry around the eastern Mediterranean, but occurs worldwide.", "Access to specialist assessment and genetic testing affects recognition and reported disease frequency." ], "questions": [ { "id": "evidence-regional-variation-q01", "text": "Which of these regional variation hold for the sense of periodic fever syndrome this model covers, and on what evidence?", "kind": "provenance" } ] }, { "id": "evidence-neighbours", "name": "Neighbouring kinds and how to tell them apart", "description": "Recalled without web access and unsourced; every item is a lead to verify.", "evidence": [ { "name": "Recurrent fever", "difference": "A symptom pattern with many possible causes; periodic fever syndromes are particular disease entities within that differential." }, { "name": "Cyclic neutropenia", "difference": "Serial blood counts demonstrate recurring neutrophil nadirs associated with fever, oral ulceration and infections." }, { "name": "Recurrent infection", "difference": "Clinical and microbiological evidence supports repeated infectious episodes rather than a primary autoinflammatory disorder." }, { "name": "Autoinflammatory disease", "difference": "A broader category that also includes diseases dominated by persistent inflammation or manifestations without recurrent fever." }, { "name": "Fever episode", "difference": "An individual event in the disease course; the syndrome is the underlying condition and is distinct from an encounter for its care." } ], "questions": [ { "id": "evidence-neighbours-q01", "text": "Which of these neighbouring kinds and how to tell them apart hold for the sense of periodic fever syndrome this model covers, and on what evidence?", "kind": "provenance" } ] } ] } ] } ] }, "openQuestions": [ "Does the registry intend this entry to cover both hereditary and non-hereditary periodic fever syndromes, including PFAPA, or a narrower concept?", "Does an existing Vercy world model already own this umbrella concept or any proposed subtype representation?", "Which authoritative definitions and current criteria sets should anchor the umbrella and its subtype relationships?", "Which ICD-10, ICD-11 and SNOMED CT mappings are valid for the intended scope and relevant releases?", "Which subtype-specific evidence supports prevalence estimates, complication surveillance and longitudinal state definitions, and where is evidence insufficient?" ], "statistics": { "bundles": 6, "layers": 11, "findings": 19, "questions": 29 } }