A report on four new cases of nephrogenic fibrosing dermopathy in chronic hemodialysis patients
Bibliographic record
Abstract
Nephrogenic fibrosing dermopathy (NFD) is a rare clinical entity affecting patients with renal failure, often on chronic dialysis or after transplantation. The patient profile at risk for this debilitating condition is undefined. Lack of awareness of the condition has hampered epidemiologic work in identifying the etiology. We present four chronic hemodialysis (HD) patients who developed this disease. The patients' ages ranged from 26 to 75 years, and they had received HD from between 20 months and 10 years before the diagnosis of NFD. Two patients had a history of renal transplantation. All patients had progressive thickening and woody induration of the skin associated with contractures, leading to difficult ambulation, and permanent disability within weeks of the diagnosis. In one case, the diaphragm, psoas muscle, and pericardium were involved. The latter is likely the first report of pericardial involvement of NFD. In all four patients, the skin findings were restricted to the extremities, sparing the trunk and face. Skin biopsy findings included thickened dermis with particularly thickened collagen bundles, fibroblast proliferation, minimal mucin deposition, and nearly absent inflammation. The pathologic findings were distinct from scleromyxedema and scleroderma. We found no laboratory evidence of autoimmune disease or thyroid dysfunction to account for alternate etiologies. CD34-positive cells were documented in the skin biopsies as well as in the diaphragm, psoas muscle, and pericardial tissue of the concerned case. NFD is a novel fibrosing disorder of progressively debilitating nature which needs further clinical characterization and recognition to guide investigation of its pathogenesis.
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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) | 0.000 | 0.001 |
| 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 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 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".