Cronkhite-Canada Syndrome (CCS): A Rare Cause of Chronic Diarrhea
Bibliographic record
Abstract
Introduction: CCS is a rare non-hereditary disease which presents as diarrhea, intestinal polyps, alopecia, hyper pigmentation and onychodystrophy. It is associated with high mortality and GI malignancies. We present a patient who was diagnosed with CCS and her management.Figure 1Figure 2Figure 3Case presentation: 55 year old Korean female with history of hypothyroidism presented with complaints of diarrhea with intermittent blood, nausea, abdominal pain and weight loss for 2 months. Associated symptoms were alopecia, loss of finger and toe nails. Labs showed albumin 1.2 g/dL, INR 1.4 and microcytic anemia, otherwise normal liver and serum chemistries. Stool work up was unremarkable. EGD revealed extensive gastroduodenitis with biopsy showing edema and marked mucosal hyperplasia. Biopsy of small bowel showed inflammation and blunting of the villi. On colonoscopy innumerous polyps were seen scattered throughout the colon, many of those were inflammatory with adenomatous change favoring the pathologic diagnosis and clinical presentation of CCS. She was treated with nutritional support and steroids, resulting in complete resolution of her cutaneous symptoms and colonoscopic findings. Discussion: CCS is a rare non-inherited disorder with incidence of 1 in a million, first reported in 1955 by Cronkhite and Canada. They described 2 females who had GI polyps, cutaneous pigmentation, alopecia and onychodystrophy. Although worldwide cases have been reported, the majority are from Japan. Etiology of CCS is still unclear, however in reported cases it can be associated with SLE, vitiligo and hypothyroidism suggesting an autoimmune trigger. Most polyps are inflammatory and non neoplastic but increased incidence of GI malignancy has been reported. Skin and nail changes are thought to be secondary to malabsorption. Nutritional support with corticosteroids remains mainstay therapy with antibiotics, acid suppressive medications and immunosuppressants as secondary treatment. Untreated CCS is associated with high mortality secondary to complications such as GI bleeding, malignant transformation, CHF and sepsis. Conclusion: CCS is a rare entity; clinicians should consider it in patients especially Asians with unexplained diarrhea and ectodermal abnormalities. Given high mortality early diagnosis is important. Diffuse gastrointestinal inflammatory polyposis sparing the esophagus on endoscopy is a hallmark of the disease. Nutritional support with corticosteroids remains the fundamental therapy.
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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) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.002 |
| Science and technology studies | 0.002 | 0.001 |
| Scholarly communication | 0.001 | 0.000 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.003 | 0.000 |
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".