Limited GPA and Alpha-1 Antitrypsin Deficiency in a Pediatric Patient
Bibliographic record
Abstract
Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis affecting small to medium-sized blood vessels. The most frequent presenting symptoms are nonspecific constitutional symptoms, followed by pulmonary, renal, and upper respiratory tract symptoms1. Alpha-1-antitrypsin (A1AT) deficiency is the most common genetic cause of liver disease in children2. We present a case of concurrent GPA and A1AT deficiency in a pediatric patient. Consent for this case report was obtained from the patient’s family. Because this is a case report, a waiver from ethics review was granted by the Hamilton Integrated Research Ethics Board. A previously healthy 3-year-old female was referred to pediatric rheumatology for subglottic stenosis. She had presented numerous times to the emergency department over several months with recurrent episodes of cough, nasal congestion, shortness of breath, and stridor. Oral dexamethasone produced dramatic symptomatic improvement. Unfortunately, she experienced ongoing congestion and stridor between steroid courses. History of presenting illness was negative for rash, joint pain/swelling, hematuria, or constitutional symptoms. Past medical history was unremarkable. Family history was positive for childhood asthma in her mother, and systemic lupus erythematosus in … Address correspondence to Dr. M. Batthish, Division of Rheumatology, Department of Pediatrics, McMaster University, 1280 Main St. West, Hamilton, Ontario L8N 3Z5, Canada. E-mail: batthim{at}mcmaster.ca
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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 | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.002 | 0.001 |
| Scholarly communication | 0.001 | 0.002 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.002 |
| Insufficient payload (model declined to judge) | 0.004 | 0.001 |
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".