S2916 From Rarity to Recognition: A Case Report of Cronkhite-Canada Syndrome
Bibliographic record
Abstract
Introduction: Cronkhite-Canada Syndrome (CCS) is a rare, non-hereditary disorder characterized by hamatomatous polyposis in the entire GI tract (excluding the esophagus) with alopecia, skin hyperpigmentation, nail dystrophy, abdominal pain, and malabsorption. It is associated with a 5-year mortality up to 55% with no specific reliably effective treatment. Case Description/Methods: A 50-year-old man presented with 7 months of abdominal pain, diarrhea, and pitting with loss of fingernails and toenails. He underwent EGD and colonoscopy which showed numerous polyps in the stomach and colon, initially raising concern for familial adenomatous polyposis. Pathology showed tubular adenoma and inflammatory polyp. Since then, his condition worsened clinically and radiographically until admission in February 2023 for protein-losing enteropathy with anasarca requiring total parenteral nutrition. Repeat upper and lower endoscopy again revealed innumerable polyps in the stomach and colon, with polyp and background mucosa pathology showing hamartomatous and various histological changes of CCS. He underwent genetic testing in March 2023 to exclude genetic syndromes. Unfortunately, his course has been complicated by repeated admissions namely for recurrent Clostridioides difficile infection despite adequate treatment. He has been started on oral prednisone, underwent a bezlotuxomab infusion, and is currently being considered for total colectomy, with possible additional immunosuppression if unresponsive to steroids, once medically stable. Discussion: CCS was first described in 1955 by Cronkhite Jr and Canada. Most cases occur in patients above 50. The exact cause is believed to be multifactorial, with possible autoimmune or inflammatory component due to increased inflammatory markers and identification of non-definitive immune abnormalities. Diagnosis is made with consistent clinical picture, endoscopic findings, and histopathology revealing cystically dilated glands, edematous stroma, and minimal inflammation. Major causes of death include gastrointestinal bleeding, sepsis, and congestive heart failure. Although there is no proven effective treatment, therapy usually consists of nutritional support, steroids, and immunosuppression. Although often non-neoplastic, rare cases of malignant transformation have been reported. Our patient illustrates the need for additional studies investigating the underlying pathophysiology and appropriate management for CCS.
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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 | 0.003 |
| Meta-epidemiology (narrow) | 0.003 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.005 | 0.004 |
| Science and technology studies | 0.005 | 0.002 |
| Scholarly communication | 0.002 | 0.002 |
| Open science | 0.002 | 0.002 |
| Research integrity | 0.007 | 0.004 |
| Insufficient payload (model declined to judge) | 0.005 | 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".