S3900 Cronkhite-Canada Syndrome: A Rare Constellation of Intestinal Polyposis and Dermatologic Abnormalities
Bibliographic record
Abstract
Introduction: Cronkhite-Canada Syndrome (CCS) is a rare acquired syndrome characterized by gastrointestinal polyposis, alopecia, nail atrophy, skin hyperpigmentation, and diarrhea resulting in malabsorption. CCS has an estimated incidence of 1 case per million people, with approximately 500 total reported cases. We report a case of CCS in an elderly woman with chronic diarrhea - 1 of few cases reported in the United States. Case Description/Methods: A 79-year-old Asian woman with hypothyroidism presented with 4 months of profuse watery diarrhea (up to 10 episodes/day), generalized weakness, and a 20-lb unintentional weight loss over 6 months. Laboratory evaluation revealed hemoglobin 8.8 g/dL, platelet count 884K, sodium 120 mmol/L, and potassium 3.4 mmol/L. C. difficile PCR was negative. Esophagogastroduodenoscopy showed enlarged gastric folds with edema, friability, and nodularity. The duodenum was carpeted with polyps, including a large pedunculated lesion in the proximal sweep. Colonoscopy revealed innumerable subcentimeter sessile polyps, colitis, and mucosal erythema. Biopsies were consistent with inflammatory polyps. CEA was mildly elevated (11.2 ng/mL). She tested positive for Helicobacter pylori and was treated accordingly. The patient also exhibited alopecia, hyperpigmented rash, and onychodystrophy. Further workup for malabsorptive and autoimmune etiologies was unremarkable. Based on the constellation of GI and ectodermal findings, CCS was diagnosed. She relocated to Singapore where she was started on azathioprine and prednisolone. Upon return to the U.S., worsening malnutrition, non-healing sacral wounds, and multiple pathologic fractures necessitated TPN. Due to persistent symptoms, escalation to biologics was considered, but she ultimately opted for palliative care. Discussion: The etiology of CCS remains unclear. Physical stress, autoimmune tendencies, H. pylori infection, and allergic response (particularly to oral thyroxine, which our patient was on for hypothyroidism) have been hypothesized to contribute to the development of CCS. Patients with CCS typically present with hyperpigmented skin, alopecia, nail changes, and chronic diarrhea with malnutrition. Endoscopy often shows hamartomatous and inflammatory polyps, giant mucosal folds, and mucosal edema. Treatment involves steroids, immunosuppressives, and biologics, though no standardized regimen exists. Prognosis is poor with a 5-year survival rate under 45%. Early diagnosis and intervention may improve outcomes.
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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 | 0.000 |
| Bibliometrics | 0.002 | 0.002 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.006 | 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".