S3073 Cronkhite-Canada Syndrome Presenting as Severe Protein-Losing Enteropathy: A Case Report
Bibliographic record
Abstract
Introduction: Cronkhite-Canada syndrome (CCS) is a rare, non-familial disorder associated with severe diarrhea, weight loss, and gastrointestinal (GI) polyposis. CCS carries a high mortality and malignant transformation rate with death often due to GI bleeding, sepsis, and congestive heart failure. We present a case of CCS manifesting as protein-losing enteropathy causing severe electrolyte disturbances and sarcopenia. Case Description/Methods: A 69-year-old man with sigmoid diverticulosis was hospitalized with 8 weeks of severe bloody diarrhea, unintentional 25-pound weight loss, alopecia, and nail atrophy. Stool studies were negative for infection. Labs revealed hypoalbuminemia (2.0 g/dL) and several electrolyte derangements requiring ongoing intravenous (IV) repletion. Computed tomography abdomen/pelvis with IV contrast showed diffuse pan-colonic and ileal thickening. Sigmoidoscopy showed marked pan-colonic pseudo-polyposis, diffuse mucosal ulcerations, erythema, and friability. Esophagogastroduodenoscopy demonstrated gastroduodenal gastritis and nodular mucosa. Histopathology revealed inflammatory non-dysplastic pseudopolyps with cystically dilated crypts and edematous lamina propria with mononuclear cells and eosinophils, consistent with CCS. The patient began high-dose IV steroids followed by high-dose IV infliximab (IFX, 10 mg/kg) due to lack of clinical improvement and significant hypoalbuminemia. Total parenteral nutrition was initiated due to severe protein-calorie malnutrition and sarcopenia. Patient achieved clinical response to IFX and was discharged on prednisone taper. He achieved steroid-free clinical remission with IFX and discontinued TPN. Discussion: The pathophysiology of CCS is not clear, though associations with immune-mediated reactions, H. pylori infection, psychosocial stress, or gene mutations (PRKDC and APC) have been described. No consensus on treatment of CCS exists, and most patients are initially treated with steroids. With steroid-refractory disease, reports describe use of anti-TNF therapy, H. pylori treatment, mesalamine, tranexamic acid, or surgery. Fortunately, our patient responded well to IFX. Future studies are needed to better understand the pathogenesis of CCS, as well as potential application of newer advanced therapies for inflammatory bowel disease in this setting (Figure 1).Figure 1.: (A-B) Sigmoidoscopy showing marked pan-colonic pseudo-polyposis, diffuse mucosal ulcerations, erythema and friability; (C) Histopathology demonstrating inflammatory non-dysplastic pseudopolyps with cystically dilated crypts and edematous lamina propria with mononuclear cells and eosinophils, consistent withCronkhite-Canada syndrome.
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.004 | 0.002 |
| Meta-epidemiology (broad) | 0.002 | 0.002 |
| Bibliometrics | 0.004 | 0.003 |
| Science and technology studies | 0.004 | 0.002 |
| Scholarly communication | 0.003 | 0.002 |
| Open science | 0.002 | 0.002 |
| Research integrity | 0.008 | 0.004 |
| Insufficient payload (model declined to judge) | 0.003 | 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".