Based on 28 histologically well-established cases of chronic non-suppurative destructive cholangitis, clinico-pathological aspects of differential diagnosis and staging are reported. In only half of the cases could the diagnosis be made on the first liver biopsy. In 14 cases, two or more liver biopsy specimens were necessary for diagnosis. The most frequent and most difficult differential diagnostic issue was to distinguish between chronic non-suppurative destructive cholangitis and chronic aggressive hepatitis of viral or autoimmune origin. The histochemical demonstration of copper by the rhodanine method was of particular value for differentiating between chronic non-suppurative destructive cholangitis and chronic aggressive hepatitis as well as for diagnosis of mixed types. In two cases of mixed type, the HBs-Antigen in ground glass hepatocytes and copper-associated protein in periportal hepatocytes could be demonstrated simultaneously with Shikata's Orcein-staining. Compared with the previously used system (Scheuer 1967) the staging concept proposed by Ludwig et al. (1978) has proved to be more useful and easier to apply. This system permits recognition of the stage on routinely obtained specimens regardless of specific differential diagnostic features. Most liver biopsy specimens were assigned to stage III. Features considered characteristic of the earlier phases (inflammatory bile duct destruction and granulomas) frequently coexist with more advanced lesions in late stage.
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Introduction: Primary Biliary Cholangitis (PBC) is a chronic autoimmune liver disease causing bile duct destruction and inflammation, impacting patient quality of life (QoL) due to variable symptoms. Digital symptom-tracker apps may improve patient care through enhanced monitoring. This feasibility study reassessed symptom burden in PBC patients using a tailored symptom-tracker app, focusing on its usability, effectiveness, and impact on management and QoL.
View Article and Find Full Text PDFHepatology
January 2025
Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, 20090 Pieve Emanuele, Milan, Italy.
Primary Biliary Cholangitis (PBC) is a chronic cholestatic liver disease characterized by the progressive destruction of intrahepatic bile ducts, leading to fibrosis, and potentially cirrhosis. PBC has been considered a prototypical autoimmune condition, given the presence of specific autoantibodies and the immune response against well-defined mitochondrial autoantigens. Further evidence supports the interaction of immunogenetic and environmental factors in the aetiology of PBC.
View Article and Find Full Text PDFHepatol Commun
January 2025
Department of Biomedical and Pharmaceutical Sciences, College of Pharmacy, University of Rhode Island, Kingston, Rhode Island, USA.
Diagnostics (Basel)
November 2024
Parkview Cancer Institute, Advanced Interventional Endoscopy & Endoscopic Oncology (IOSE) Division, GI Oncology Program, 11104 Parkview Circle, Suite 310, Fort Wayne, IN 46845, USA.
Front Pediatr
November 2024
Department of Infectious Diseases, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China.
Background: Langerhans cell histiocytosis (LCH) is a systemic neoplasia with diverse clinical manifestations, predominantly affecting bone and skin. However, in children, LCH presenting primarily with cholestasis is rare.
Case Summary: We present the case of a 22-month-old boy who was admitted to our hospital with a history of intermittent fever and abdominal distension for over 2 months, and jaundice for over 1 month.
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