Background: Patients with hepatic reticulum degeneration (HLD) may eventually develop complications of cirrhosis with splenomegaly and hypersplenism, requiring splenectomy to alleviate hypersplenism and complete lifelong copper therapy. The purpose of this study is to investigate the effect of splenectomy on liver function in patients with hypersplenism.
Methods: A retrospective systematic analysis was conducted on the liver function indicators of 220 HLD patients who underwent splenectomy from January 2015 to January 2018 before surgery and on days 1, 3, 5, 7, and 14 after surgery. Among them, 30 patients were followed up for 6 months.
Results: The Child score increased on the 1st day after surgery and gradually decreased after the 1st day. The level of alanine aminotransferase (ALT), aspartate aminotransferase (AST), and total bilirubin (TB) increased 5 days after surgery ( < .01) and decreased on the 14th day after surgery ( < .01); the level of albumin (ALB) decreased on the 1st, 3rd, and 5th day after surgery ( < .01) and increased on the 14th day ( < .01). The follow-up results of the patient for 6 months showed that the levels of ALT and AST decreased, while the levels of ALB increased 6 months after surgery.
Conclusion: Splenectomy is proved to be beneficial for the improvement of liver function in HLD patients combined with hypersplenism, which realize a lifelong anti-copper treatment.
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http://dx.doi.org/10.1177/00031348221114041 | DOI Listing |
Signal Transduct Target Ther
January 2025
Department of Hepatobiliary Surgery, Centre for Leading Medicine and Advanced Technologies of IHM, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, Anhui, 230001, China.
CD8T cells within the tumor microenvironment (TME) are often functionally impaired, which limits their ability to mount effective anti-tumor responses. However, the molecular mechanisms behind this dysfunction remain incompletely understood. Here, we identified valosin-containing protein (VCP) as a key regulator of CD8T cells suppression in hepatocellular carcinoma (HCC).
View Article and Find Full Text PDFKidney Int
February 2025
Institute of Physiology, University of Zurich, Zurich, Switzerland; Division of Nephrology, Cliniques universitaires Saint-Luc, UCLouvain Medical School, Brussels, Belgium. Electronic address:
The Kidney Disease: Improving Global Outcomes (KDIGO) 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) represents the first KDIGO guideline on this subject. Its scope includes nomenclature, diagnosis, prognosis, and prevalence; kidney manifestations; chronic kidney disease (CKD) management and progression, kidney failure, and kidney replacement therapy; therapies to delay progression of kidney disease; polycystic liver disease; intracranial aneurysms and other extrarenal manifestations; lifestyle and psychosocial aspects; pregnancy and reproductive issues; pediatric issues; and approaches to the management of people with ADPKD. The guideline has been developed with patient partners, clinicians, and researchers around the world, with the goal to generate a useful resource for healthcare providers and patients by providing actionable recommendations.
View Article and Find Full Text PDFJ Med Genet
January 2025
Division of Clinical and Metabolic Genetics, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Biallelic pathogenic variants in cause a fatal autosomal recessive multisystem disorder characterized by recurrent autoinflammation, hypomyelination, progressive neurodegeneration, microcephaly, failure to thrive, liver dysfunction, respiratory chain defects and accumulation of glycogen in skeletal muscle. No missense variants in have been reported to date.We report a 6-year-old boy with microcephaly, global developmental delays, lower limb spasticity with hyperreflexia, epilepsy, abnormal brain MRI, failure to thrive, recurrent fevers and transaminitis.
View Article and Find Full Text PDFJ Immunother Cancer
January 2025
Department of Oncology, Uppsala University Hospital, Uppsala, Sweden
Background: ATOR-1017 (evunzekibart) is a human agonistic immunoglobulin G4 antibody targeting the costimulatory receptor 4-1BB (CD137). ATOR-1017 activates T cells and natural killer cells in the tumor environment, leading to immune-mediated tumor cell death.
Methods: In this first-in-human, multicenter, phase I study, ATOR-1017 was administered intravenously every 21 days as a monotherapy to patients with advanced, unresectable solid tumors having received multiple standard-of-care treatments.
Food Chem Toxicol
January 2025
Department of Medical Biochemistry, Faculty of Medicine, Aksaray University, Aksaray, TURKEY.
Aim: Tramadol (TRM), a widely used opioid analgesic for moderate to severe pain, is associated with liver and kidney toxicity at high doses or prolonged use. This study investigates the protective role of rosmarinic acid (RA), a natural phenolic compound known for its antioxidant, anti-inflammatory, and cell-protective properties, against TRM-induced hepatorenal toxicity.
Methods: Thirty-five male Wistar rats were divided into five groups: Control, TRM, RA, TRM+RA25, and TRM+RA50.
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