Bibliographic record
Abstract
Case Reports: Non-fatal cases of infantile cardiac glycogenosis are rare. A high index of suspicion is necessary for metabolic cardiomyopathies in atypical presentations of near sudden infant death. A term infant male was born to a healthy non-consanguineous primigravida with normal prenatal ultrasounds. Family history revealed 4 unexplained infant deaths. At 3 months of life he presented with upper respiratory infection, cynosis and stiffening of legs post feed, progressing to obtundation, bradycardia and hypotension requiring 25 min of cardiopulmonary resuscitation (CPR); he was transferred to the intensive care unit (ICU) intubated. Extensive investigations including neuroimaging, electroencephalography and metabolic panel were normal. Echocardiogram demonstrated mild concentric left ventricular hypertrophy (LVH) but normal systolic function. Nasopharyngeal swab was positive for Enterovirus. He was transferred to the ward on day 5 and subsequently had a 2nd episode characterized as sinus bradycardia progressing to 3rd degree heart block. After 3 minutes of CPR and atropine, he converted to sinus rhythm and was transferred back to ICU. The following day he had a similar event progressing to shock; hence he was placed on extracorporeal membrane oxygenation (ECMO). Echocardio showed severe biventricular dilation and dysfunction. Repeat neuroimaging was unremarkable. He was decannulated after 4 days with moderate ventricular dysfunction and dilation. Cardiac biopsy was negative for structural abnormalities; electron microscopy (EM) could not be completed due to insufficient tissue. Considering that the problem was primarily a cardiac rhythm issue, an epicardial dual chamber pacemaker (VVI) was implanted. LV systolic function and dimensions normalized, however moderate concentric LVH persisted at time of discharge at 5 months of age. At 6 months, he was readmitted with a 4th event, having 20 min of CPR at home and regaining hemodynamic stability. Interrogation of the pacemaker revealed bradycardia despite effective VVI backup rate. On day 2 of ICU admission, he had his 5th event requiring E-CPR (ECMO cannulation with ongoing CPR). Repeat cardiac biopsy with EM was completed on support and revealed cardiac glycogenosis. With normal peripheral muscle and liver biopsy, he was deemed a heart transplant candidate. He was changed to a Ventricular assisted device (VAD) and within 32 days successfully bridged to heart transplant. Patient was discharged home within 1 month neurologically intact.
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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) | 0.002 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.003 | 0.002 |
| Science and technology studies | 0.002 | 0.001 |
| Scholarly communication | 0.002 | 0.002 |
| Open science | 0.001 | 0.002 |
| Research integrity | 0.004 | 0.002 |
| Insufficient payload (model declined to judge) | 0.108 | 0.069 |
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".