'Little Leopard ' Syndrome Description of 3 Cases and Review of 24
Bibliographic record
Abstract
3 cases and review of 24. Three cases of the 'Little Leopard ' syndrome are described; its features are short stature, lentigines, electrocardiographic and ocular defects, pulmonary infundibular stenosis, abnormal genitalia, mental retardation, and deafness. The published material is reviewed, and the cardiac and electro-cardiographic abnormalities are described in detail. The word 'leopard ' was first applied as a mnemonic by Gorlin, Anderson, and Blaw (1969) to the syndrome of multiple lentigines, electro-cardiographic conduction abnormalities, ocular hypertelorism, pulmonary stenosis and other congenital heart defects, abnormalities of genitalia and reproductive physiology, retarded growth, and deafness. The condition is inherited as an auto-somal dominant of variable penetrance. Lentigines are small dark-brown spots which may be present at birth or appear shortly after. Their pathognomonic feature is the combination of a saw-tooth appearance of the dermal epidermal junction combined with a single layer of pigment cells which contrasts with the nests of the cells that occur in a naevus. They usually appear earlier than freckles, contain a greater number of melano-cytes per unit of skin area, and do not increase in number with exposure to sunlight. This paper reports 3 cases, 2 seen at The Hospital for Sick Children, Toronto, and one at the Radcliffe Infirmary, Oxford, together with a review of the literature, in which it appears that while a number of the children are retarded physically, a significant proportion are also retarded mentally. We have therefore altered the meaning of the 'r ' in 'leopard' to represent retarded mentally.
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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 | 0.001 |
| Meta-epidemiology (narrow) | 0.002 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.006 | 0.003 |
| Science and technology studies | 0.001 | 0.002 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.002 | 0.001 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.006 | 0.004 |
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".