Hepatobiliary and Pancreatic: Multinodular fatty liver associated with porphyria cutanea tarda
Notice bibliographique
Résumé
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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Comment cette classification a été obtenuedéplier
Prédiction distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,000 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule tête enseignante, pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».