S2545 Battlefield of Polyps: Challenges of the Rare Cronkhite-Canada Syndrome
Bibliographic record
Abstract
Introduction: Hamartomatous polyps are benign, disorganized growth of tissues found in the gastrointestinal tract. Commonly associated syndromes are juvenile polyposis (JPS), Peutz-Jegher syndrome (PJS), and Cowden Syndrome (CS). Cronkhite Canada syndrome (CCS) is an extremely rare gastrointestinal polyposis syndrome with high morbidity and mortality. Case Description/Methods: An 87-year-old man presented with epigastric discomfort, postprandial fullness, unintentional weight loss, and diarrhea. He had a past history of metachronous colon cancer (status post right cecal resection followed by right hemicolectomy almost 20 years ago), duodenal adenoma, hyperplastic gastric polyps, and a recurrent ventral hernia. His presenting labs were significant for a positive FIT test, chronically elevated CEA level stable at 3.5 due to his history of colon cancer, and a mid-line ventral hernia containing fat on abdominopelvic ultrasound. An EGD showed biopsy-proven gastric cardiac hyperplastic polyps and a giant gastric fold at lesser curvature negative for malignancy (Figure 1). A colonoscopy revealed multiple colonic polyps and a biopsy-proven moderately differentiated adenocarcinoma of the ascending colon. Patient underwent ventral hernia repair and subtotal colectomy with ileosigmoid and small bowel anastomosis. Patient continued to have diarrhea despite a bowel regimen including loperamide, diphenoxylate/atropine, and cholestyramine along with fibers and tincture of opium resulting in further weight loss. A repeat EGD and colonoscopy 1 year later showed a significant burden of hamartomatous gastric, duodenal, and rectal polyps, for which he underwent extensive genetic testing which was negative. Patient was diagnosed with Cronkhite Canada syndrome based on ageusia, hair thinning, onychodystrophy, gastrointestinal hamartomatous polyps, diarrhea, weight loss, and hypoalbuminemia. Discussion: The rarity of Cronkhite Canada Syndrome (CCS) makes it a challenging diagnosis. IgG4 was negative in our patient and its absence does not rule out CCS as reported in previous cases. Although evidence-based effective therapies are lacking, we treated our patient symptomatically based on case studies with steroids and azathioprine, with resultant improvement in diarrhea. Nutritional support and TPN were initiated for debilitating malnutrition, weight loss, and electrolyte abnormalities. Close surveillance is integral to managing patient's symptoms and therapies due to the high morbidity of this syndrome.Figure 1.: Endoscopy images.
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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) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.002 | 0.001 |
| Science and technology studies | 0.002 | 0.001 |
| Scholarly communication | 0.001 | 0.002 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.004 | 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".