PMC9099094_01
Histopathologic findings in the liver. (A) Liver tissue showed no significant lobular architecture (hematoxylin and eosin staining). Scale bar represents 50 mum. (B) Cholestasis was observed in the hepatocytes, Kupffer cells, and bile canaliculi at zones 2 and 3 (hematoxylin and eosin staining). Scale bar represents 20 mum. (C) There was no significant fibrosis (Azan staining). Scale bar represents 100 mum. (D-F) Immunohistochemistry for bile salt export pump (D), CD10 (E), and keratin 7 (F). The strong expression of bile salt export pump and CD10 were observed at the canalicular membrane (D,E). The keratin 7 expression was found in the hepatocytes in the periportal areas. The intralobular bile duct arrangement was preserved (F). Scale bars represent 50 mum.
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F1
The liver tissue obtained from a percutaneous liver biopsy showed marked cholestasis in the hepatocytes, Kupffer cells, and bile canaliculi; however, no lobular inflammation, hepatic necrosis, or fibrosis were found (Figures 1A-C). Further pathological assessment and immunohistochemistry studies revealed that bile salt export pump, multidrug resistance protein 3, multidrug resistance-associated protein 2, and CD10 were strongly expressed at the canalicular membrane (Figures 1D-E) and that the intralobular bile duct arrangement was well-preserved (Figure 1F).
Clinical course of the patient. Changes in serum alanine aminotransferase (ALT,  ), total bile acid (TBA,  ), total bilirubin (T-Bil,  ), albumin (Alb,  ), and gamma-glutamyl transpeptidase (GGT,  ) levels. The patient was treated with prednisolone or rifampicin with ursodeoxycholic acid during the attacks. The lowest albumin level during the follow-up period was 3.3 g/dL.
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F2
During each attack, the patient experienced strong digestive symptoms associated with >5 kg of body weight loss and hypoalbuminemia (Figure 2). This treatment rapidly relieved his symptoms and markedly shortened the symptomatic phase compared to prednisolone (Figure 2). Thereafter, the symptomatic phase was markedly shortened (Figure 2).