Prenatal infantile cortical hyperostosis (Caffey's disease): a ‘hepatic myeloid hyperplasia‐pulmonary hypoplasia sequence’ can explain the lethality of early onset cases
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Bibliographic record
Abstract
BACKGROUND: Infantile cortical hyperostosis (ICH) is benign and self-limiting when it presents near or after birth but is usually lethal when it presents earlier. METHODS: We present the clinical, ultrasonic, radiographic and pathologic findings in an instructive case of early onset prenatal ICH. RESULTS: A 40-year-old G2P1 woman delivered spontaneously at 22 weeks' gestation. Prenatal ultrasounds showed rib and mandibular abnormalities as well as short humeri. Post-mortem radiographs showed asymmetric hyperostosis in long bones, mandible, scapulae and pelvis with sparing of spine, hands, feet and skull. The affected skeleton showed marked bony sclerosis and ballooning of the diaphyses of the long bones with periosteal sclerosis. A complete autopsy showed characteristic histologic findings in affected bones. Previous reports at 20+ weeks have described anasarca, fetal hydrops, hepatomegaly, and pulmonary hypoplasia. In our case, there was no hydrops/anasarca; hepatomegaly, due to massive extramedullary hematopoiesis with marked myeloid hyperplasia, combined with ribcage abnormalities, caused mild pulmonary hypoplasia. CONCLUSION: We hypothesize that early onset fetal ICH is usually lethal because massive hepatic myeloid hyperplasia sequentially causes: (1) hepatomegaly and, in conjunction with rib abnormalities, mild pulmonary hypoplasia, (2) sinusoidal and pre-sinusoidal portal hypertension followed by ascites/hydrops, and (3) ascites/hydrops-induced severe pulmonary hypoplasia.
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Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 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 it