IBD in Camouflage or Are There Similarities?: A Case of Cronkhite-Canada Syndrome
Bibliographic record
Abstract
First described in 1955, Cronkhite Canada Syndrome (CCS) is a rare non-familial polyposis syndrome with approximately 500 cases to date. With a five-year mortality of 50%, it is crucial to correctly and quickly identify the syndrome's primary features of alopecia, onychodystrophy, cutaneous hyperpigmentation, and intestinal malabsorption. Unfortunately, even when diagnosed, treatment is challenging as the etiology is unknown though possibly immune-mediated. Antibiotics, steroids, and immunosuppressants have all shown varying degrees of success. A 74-year-old Filipino woman with a history of hypothyroidism presented to our Inflammatory Bowel Disease (IBD) Clinic for a second opinion. Seven months prior, she experienced onset of watery, non-bloody diarrhea with initial diagnosis of ulcerative colitis. After this diagnosis, she responded to treatment with prednisone, but failed maintenance therapy with oral mesalamine. She then received two doses of infliximab without response and was referred to our IBD clinic. She reported continued watery, non-bloody diarrhea with significant weight loss. Physical exam revealed alopecia, onychodystrophy, and lower extremity edema with cutaneous hyperpigmentation. Her labs revealed hyponatremia, severe hypoalbuminemia, c-reactive protein (CRP) of 55.3 mg/L (0-8.2 mg/L), and a hemoglobin of 10.1 g/dL which prompted hospital admission.Inpatient EGD and colonoscopy revealed diffuse polyposis throughout the stomach, duodenum, and colon. Biopsies revealed diffuse inflammatory hyperplastic polyps with increased IgG4 positive cells (up to 22 per high power field). Based on this clinical picture, she was diagnosed with CCS. She was placed on full bowel rest and given total parenteral nutrition. She also received IV corticosteroids in conjunction with azathioprine and infliximab. We achieved good clinical response and her CRP normalized. CCS is a rare non-familial polyposis syndrome with characteristic physical exam findings, diffuse polyposis, debilitating diarrhea, and significant 5-year mortality. Treatment is challenging and focuses primarily on nutritional support and immunosuppressant medications. In this particular case we chose to proceed with low dose azathioprine and infliximab based on success of this regimen in past case reports published. We will plan to achieve steroid free remission by optimizing infliximab based on levels and providing nutritional support.2073_A Figure 1. Onychodystrophy2073_B Figure 2. Diffuse polyposis on colonoscopy2073_C Figure 3. Diffuse inflammatory hyperplastic polyps with increased IgG4 cells
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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) | 0.002 | 0.002 |
| Meta-epidemiology (broad) | 0.002 | 0.001 |
| Bibliometrics | 0.004 | 0.004 |
| Science and technology studies | 0.004 | 0.003 |
| Scholarly communication | 0.002 | 0.002 |
| Open science | 0.002 | 0.003 |
| Research integrity | 0.007 | 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".