ABSTRACT
A 33-year-old woman with a history of photosensitivity, persistent abdominal pain, and liver dysfunction was admitted to our department because of abdominal pain and progression of liver dysfunction. On admission, levels of protoporphyrin and coproporphyrin within erythrocytes were markedly increased. Autofluorescent erythrocytes were also detected, leading to a diagnosis of erythropoietic protoporphyria. A liver biopsy specimen revealed cirrhosis with dark brown granules filling hepatocytes, bile canaliculi, and bile ductules. Transfusion of washed erythrocytes, hemodialysis, and administration of cholestyramine and beta-carotene transiently improved levels of porphyrins and liver function. The patient died of rupture of esophageal varices followed by multiple organ failure. However, the treatments were believed to have extended survival.
Subject(s)
Liver Cirrhosis/etiology , Liver Failure/etiology , Multiple Organ Failure/etiology , Porphyria, Hepatoerythropoietic/complications , Porphyria, Hepatoerythropoietic/therapy , Adult , Autopsy , Biopsy, Needle , Disease Progression , Drug Therapy, Combination , Esophageal and Gastric Varices/etiology , Fatal Outcome , Female , Humans , Liver Cirrhosis/pathology , Liver Cirrhosis/therapy , Liver Failure/pathology , Liver Function Tests , Porphyria, Hepatoerythropoietic/pathology , Renal Dialysis , Rupture, SpontaneousABSTRACT
Magnetic resonance imaging (MRI) and computed tomography (CT) were performed in four patients with thorotrastosis. On CT scan, Thorotrast (thorium oxide) deposition was shown as high-density areas in the liver and spleen and the abdominal lymph nodes. These deposits were not found on MRIs. Splenic volume was significantly small due to atrophy. The contrast-noise ratio in the spleen on T1-weighted images was significantly lower. Thorotrast deposition does not affect MRI appearance; therefore it may be useful for the early detection of malignant tumors as a complication of thorotrastosis.
