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Bilateral Renal Fungal Bezoars and Perinephric Abscess in an Infant With Arthrogryposis-Renal Dysfunction-Cholestasis Syndrome: A Clinico-pathologic Case Report.
Pediatr Dev Pathol
January 2025
Département d'Anatomie et Cytologie pathologiques, Hôpital Menzel Bourguiba, Menzel Bourguiba, Tunisia.
The patients with Arthrogryposis-Renal dysfunction-Cholestasis (ARC) syndrome have genetic susceptibility to the opportunistic infections due to the involvement of VPS33B (vacuolar protein sorting 33 homolog B) in phagolysosome fusion in macrophages. Detailed pathologic studies in ARC patients are missing in literature due to the lack of autopsy. We described the first autopsy case of ARC syndrome in a 2-month-old male infant.
View Article and Find Full Text PDFA machine learning based algorithm accurately stages liver disease by quantification of arteries.
Sci Rep
January 2025
Shuguang Hospital, Shanghai University of Traditional Chinese Medicine, 528 Zhangheng Road, Pudong New Area, Shanghai, 201203, China.
A major histologic feature of cirrhosis is the loss of liver architecture with collapse of tissue and vascular changes per unit. We developed qVessel to quantify the arterial density (AD) in liver biopsies with chronic disease of varied etiology and stage. 46 needle liver biopsy samples with chronic hepatitis B (CHB), 48 with primary biliary cholangitis (PBC) and 43 with metabolic dysfunction-associated steatotic liver disease (MASLD) were collected at the Shuguang Hospital.
View Article and Find Full Text PDFIncreased PD-1 expression in livers associated with PD-1-antibody-induced hepatotoxicity.
BMC Immunol
January 2025
Department of Oncology and Hematology, Oulu University Hospital, Oulu, Finland.
Vanishing bile duct syndrome (VBDS) is a serious drug induced liver injury characterized by chronic cholestasis and loss of intrahepatic bile ducts. VBDS has been reported also following checkpoint inhibitor treatment. We compared CD3 + , CD4 + , CD8 + , CD20 + , CD57 + , PD-1 + and PD-L1 + lymphocyte infiltrates in liver biopsies of patients that encountered VBDS (n = 2) or hepatotoxicity (n = 3) after pembrolizumab (n = 4) or nivolumab (n = 1) treatment with samples from normal liver (n = 10), non-alcohol steatohepatitis (NASH, n = 10), primary biliary cholangitis (PBC, n = 10) or pembrolizumab-treated patients without adverse events (n = 2).
View Article and Find Full Text PDFMortality in Autoimmune Liver Disease in Sweden: A Population-Based Cohort Study of 9,654 Patients.
Liver Int
February 2025
Department of Medicine, Huddinge, Karolinska Institute, Stockholm, Sweden.
Background/aims: Epidemiological data on mortality in autoimmune liver diseases (AILDs) are scarce. We examined all-cause and cancer-related mortality in individuals with AILD from Sweden.
Methods: We identified 9654 individuals with AILD (3342 with autoimmune hepatitis (AIH), 3751 with primary biliary cholangitis (PBC), and 2561 with primary sclerosing cholangitis (PSC)) using national Swedish registries between 2001 and 2020.
Recent advances in the management of pediatric cholestatic liver diseases.
J Pediatr Gastroenterol Nutr
January 2025
Department of Pediatrics, Children's Hospital of Colorado, University of Colorado, Aurora, Colorado, USA.
Pediatric cholestatic liver diseases are rare conditions that can result from multiple specific underlying etiologies. Among the most common etiologies of pediatric cholestatic liver diseases are biliary atresia, Alagille syndrome (ALGS), and inherited disorders of bile acid transport. These diseases are characterized by episodic or chronic unremitting cholestasis.
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