A fulminant course of Cronkhite-Canada syndrome
Bibliographic record
Abstract
A 57-year-old man suffered from watery diarrhea, weight loss, and abdominal pain for 4 months. Noticeable signs of nail dystrophy (onychomadesis of all nails) were present ([ Fig. 1 a ]) along with alopecia and cutaneous foci of hyperpigmentation ([ Fig. 1 b ]). Fig. 1 Main physical findings. a All 20 nails with onychomadesis about 3 mm from the lunula. Several nail plates have been lost. b Hands with several small foci of skin hyperpigmentation. Upper-gastrointestinal endoscopy revealed a large number of strawberry-like polyps of different size in the stomach ([ Fig. 2 ]). Fig. 2 Endoscopic findings in the stomach. a Polyps of gastric body and antrum. b Detailed view of gastric polyp using zoom and narrow-band imaging mode; note the strawberry-like appearance. Colonoscopy revealed polyposis of the whole colon, including the rectum. The majority of the polyps had a strawberry-like, adenomatous, and hyperplastic appearance ([ Fig. 3 ]). Fig. 3 a, b Endoscopic view of colonic polyps. Histologically, the majority of the polyps were juvenile-like with cystic dilatations of the glands and a benign mucinous epithelium. The glands were filled with a large amount of mucin ([ Fig. 4 ]). Fig. 4 Histology. a Juvenile-like polyp with enlarged mucin glands. b Detailed view of mucinous glandular epithelium. Quality: mobile 360 480 720 Download Video 1 Endoscopy of the stomach. Quality: mobile 360 480 720 Download Video 2 Endoscopy of the colon. Some adenomatous polyps with low-grade dysplasia in the colon were also detected. A subsequent enteroscopy did not find polyps; however, edema and small indentation of the jejunum were present. Immunohistochemistry showed total alactasia and a strong positivity for tumor necrosis factor (TNF) in the macrophages and lymphocytes. Based on both the clinical and endoscopic picture, a diagnosis of Cronkhite-Canada syndrome was established. Despite complex treatment, the clinical course was unfavourable. Since the clinical state of the patient did not improve, anti-TNF-α treatment was considered. TNF-α activity was examined in the small-intestinal mucosa, and the results showed a strong intracellular expression of TNF-α. Unfortunately, an experimental anti-TNF-α treatment could not be introduced, because of the rapid progression of the disease. The patient died 4 months after the diagnosis of Cronkhite-Canada syndrome was established. Cronkhite-Canada syndrome is a rare non-hereditary polyposis syndrome of an unknown, possibly autoimmune etiology. The prognosis is poor and more than 50 % of patients die within 2 – 4 years [ 1 ] [ 2 ]. Cronkhite-Canada syndrome should be considered in all patients with gastrointestinal polyposis together with emergent alopecia and/or nail dystrophy [ 3 ]. Competing interests: None Endoscopy_UCTN_Code_CCL_1AD_2AC
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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) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.002 | 0.001 |
| Science and technology studies | 0.002 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.002 |
| Insufficient payload (model declined to judge) | 0.002 | 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".