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TERT de novo mutation-associated dyskeratosis congenita and porto-sinusoidal vascular disease: a case report.
J Med Case Rep
January 2025
Department of Hepatic Biliary Pancreatic Medicine, First Hospital of Jilin University, 1 Xinmin Avenue, Changchun, 130021, China.
Background: Dyskeratosis congenita is a rare genetic disease due to telomere biology disorder and characterized by heterogeneous clinical manifestations and severe complications. "Porto-sinusoidal vascular disease" has been recently proposed, according to new diagnostic criteria, to replace the term "idiopathic non-cirrhotic portal hypertension." TERT plays an important role in telomeric DNA repair and replication.
View Article and Find Full Text PDFThe relationship between spontaneous cystic degeneration and pseudocapillarization in sinusoids in the liver of aged Sprague-Dawley rats.
J Toxicol Pathol
January 2025
The Institute of Environmental Toxicology, 4321 Uchimoriya-machi, Joso-shi, Ibaraki 303-0043, Japan.
Cystic degeneration (CD) in the liver is a cyst-like lesion composed of one or more pseudocysts lacking lining cells, occurring spontaneously in rats older than 12 months, with a male predilection. In this study, 32 CDs were identified in 23 out of 104 non-treated, control male Sprague-Dawley rats from two combined chronic toxicity and carcinogenicity studies with agrochemicals. They were examined histologically, histochemically, and immunohistochemically to assess the pathogenesis and pathological significance of CD, focusing on pseudocapillarization in aged rat liver.
View Article and Find Full Text PDFTargeted delivery of polymeric NO-donor micelles to hepatic stellate cells for restoration of liver function and inhibition of hepatic fibrosis.
J Control Release
January 2025
Department of Pharmaceutical Engineering, China Pharmaceutical University, Nanjing 211198, China. Electronic address:
Liver fibrosis is a prevalent liver disease associated with significant morbidity, and the activation of hepatic stellate cells (HSCs) serves as the primary causative factor driving the progression of liver fibrosis. However, capillarization of liver sinusoidal endothelial cells (LSECs) induced by hepatic fibrosis can reduce nitric oxide (NO) production and bioavailability, which consequently loses the ability to retain HSCs dormant, leading to amplified HSCs activation. Herein, an elaborate micelle (VN-M@BN) loaded with benazepril (BN) was constructed by self-assembly of polymeric NO donor, aiming for the controlled release of NO in liver fibrosis lesions thereby impeding the progression of liver fibrosis.
View Article and Find Full Text PDFHepatic encephalopathy in non-cirrhotic portal hypertension.
Metab Brain Dis
January 2025
Institute of Liver and Biliary Sciences, New Delhi, India.
Hepatic encephalopathy (HE) is traditionally associated with hepatic parenchymal diseases, such as acute liver failure and cirrhosis. Its prevalence in non-cirrhotic portal hypertension (NCPH) patients, extrahepatic portal vein obstruction (EHPVO), and non-cirrhotic portal fibrosis (NCPF) is less well described. HE in NCPH allows one to study the effect of portosystemic shunting and ammonia without significant hepatic parenchymal injury.
View Article and Find Full Text PDFClinicopathological features of porto-sinusoidal vascular disorder with a novel GIMAP5 mutation in a pair of twin siblings.
Dig Liver Dis
January 2025
Department of Pathology, Beijing Ditan Hospital, Captial Medical University, Beijing 100015, PR China. Electronic address:
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