Hepatobiliary and Pancreatic: Multinodular fatty liver associated with porphyria cutanea tarda
Bibliographic record
Abstract
A 61-year-old woman was investigated because of an episode of pain in the right upper quadrant of her abdomen that radiated into the mid-back. Her past history included a cholecystectomy and hysterectomy and, 11 years previously, she had an episode of blistering on her hands that was diagnosed as porphyria cutanea tarda. A sibling had also been diagnosed with porphyria cutanea tarda. Screening blood tests including liver function tests were within the reference range while an abdominal ultrasound study raised the possibility of fatty infiltration with liver nodules. A computed tomography scan showed hypoattenuating masses throughout both lobes of the liver (Figure 1). The masses did not show hypervascularity on arterial-phase imaging. A magnetic resonance imaging scan also showed multinodular fatty infiltration throughout the liver (Figure 2) with no enhancement in the post-gadolinium sequences. A liver biopsy performed under ultrasound guidance showed macrovesicular steatosis with an increase in hepatic iron (siderosis). Porphyria cutanea tarda is caused by a deficiency in hepatic uroporphyrinogen decarboxylase. This results in the accumulation of porphyrins in the skin and the clinical manifestations of photodermatitis, blistering and hypertrichosis. Only a minority of patients (20%) have a family history of this disorder. In the majority, the inheritance is either poorly understood or the disease is acquired because of factors such as alcohol abuse and hormonal therapy. On liver biopsy, up to 80% of patients have iron overload and some patients (15%) have cirrhosis. Causes for iron overload include alcohol abuse and heterozygosity for haemochromatosis genes such as C282Y. Porphyria cutanea tarda has also been associated with multinodular fatty liver in two case reports. Both patients appeared to have an acquired form of porphyria cutanea tarda because of chronic liver disease. In the patient described above, multinodular fatty liver occurred in the presence of an inherited form of the disorder. When liver biopsies are performed in patients with porphyria cutanea tarda, a characteristic feature is that of bright red/orange fluorescence (Maltese cross) when specimens are examined under ultraviolet light. However, these crystals are water-soluble and are not observed in routine specimens stained with hematoxylin and eosin. Contributed by
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".