Clinical, biochemical and immunological variables were analyzed in 30 patients with orcein-negative (ON) chronic active hepatitis (CAH), 4 patients with ON primary biliary cirrhosis (PBC), 8 patients with orcein-positive (OP; intracellular copper-binding protein seen histologically in liver biopsy specimens) CAH and 15 patients with OP-PBC. A marked elevation of serum bile acids, alkaline phosphatase, leusine aminopeptidase, gammaglutamyl transpeptidase and cholesterol concentrations, and highly pathological BSP Tm values were characteristic for OP-cases. In addition the faecal fat level was increased and bile acids decreased in OP-cases. Serum levels of IgG or IgM and the occurrence of smooth muscle, mitochondrial or glomerular antibodies were identical in ON- and OP-CAH as well as also in ON- and OP-PBC. 49 patients were treated with a combination of prednisone and azathioprine from 4 to 72 months (mean 22). 26 patients with ON-CAH responded biochemically and morphologically to the treatment. No treatment failures were found in ON-CAH. In contrast treatment failure was confirmed in every treated OP-PBC or OP-CAH. The results suggest that orcein-positivity indicates a poor response to prednisone-azathioprine treatment of CAH.
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Front Immunol
December 2024
Institute for Immunodeficiency, Center for Chronic Immunodeficiency, University Medical Center Freiburg, Freiburg, Germany.
Background: Hypomorphic mutations in the () gene cause a glycosylation disorder that leads to immunodeficiency. It is often associated with recurrent infections and atopy. The exact etiology of this condition remains unclear.
View Article and Find Full Text PDFFront Immunol
December 2024
School of Medicine, Shenzhen University, Shenzhen, China.
Front Med (Lausanne)
December 2024
Department of Infectious Diseases, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China.
Objective: To investigate the probability of hepatocellular carcinoma (HCC) in a large number of gray-zone (GZ) patients with chronic hepatitis B (CHB) in clinical practice.
Methods: The patients with CHB who were diagnosed and treated in our hospital from January 2013 to January 2023 were analyzed retrospectively.
Results: According to the different levels of HBeAg, ALT and HBV DNA, GZ patients were divided into four categories: (1) Gray zone A (GZ-A): HBeAg positive, normal ALT level, HBV DNA ≤ 10 IU/ml; (2) Gray zone B (GZ-B): HBeAg positive, ALT>ULN, HBV DNA ≤ 2 × 10 IU/ml; (3) Gray zone C (GZ-C): HBeAg negative, normal ALT level, HBV DNA ≥ 2 × 10 IU/ml; and (4) Gray zone D (GZ-D): HBeAg negative, ALT > ULN, serum HBV DNA ≤ 2 × 10 IU/ml.
Theranostics
January 2025
Department of Nephrology, Jiangsu Province Hospital, The First Affiliated Hospital of Nanjing Medical University, Nanjing Medical University, 300 Guangzhou Road, Nanjing 210029, China.
Chronic kidney disease (CKD) is a global public health issue, with vascular calcification (VC) being a common and deadly complication. Despite its prevalence, the underlying mechanisms of VC remain unclear. In this study, we aimed to investigate whether and how Otubain-2 (OTUB2) contributes to VC.
View Article and Find Full Text PDFCureus
December 2024
Internal Medicine, California Institute of Behavioral Neurosciences and Psychology, Fairfield, USA.
Calciphylaxis is a rare but potentially life-threatening disease that is not yet completely understood. It occurs mainly in patients with chronic kidney disease termed calcific uremic arteriolopathy (CUA) but also affects patients with normal renal function. Although this disease's pathogenesis is unclear, it is associated with the dysregulation of calcium and phosphate and subsequent calcification of peripheral arterioles.
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