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A comparative analysis in monitoring 24-hour urinary copper in wilson disease: sampling on or off treatment?
Orphanet J Rare Dis
January 2025
Internal Medicine IV, Department of Gastroenterology, University Hospital Heidelberg, INF 410, Heidelberg, 69120, Germany.
Background & Aim: Twenty-four-hour urinary copper excretion (24 h-UCE) is the standard diagnostic tool for dose adjustments in maintenance therapy in Wilson disease (WD) patients. Guidelines lack data if both variants of 24 h-UCE measurement (with or without 48 h of treatment interruption) are equally interpretable.
Methods: Eighty-four patients with a confirmed diagnosis of WD treated with chelators (50% of patients with D-Penicillamine and 50% with trientine) and with pairwise 24-h-UCE values on-therapy and off-therapy were included in the analysis.
Microbe-mediated synthesis of defect-rich CeO nanoparticles with oxidase-like activity for colorimetric detection of L-penicillamine and glutathione.
Nanoscale
January 2025
College of Biotechnology and Pharmaceutical Engineering, State Key Laboratory of Materials-Oriented Chemical Engineering, Nanjing Tech University, Nanjing 211816, P.R. China.
To enhance production efficiency, curtail costs, and minimize environmental impact, developing simple and sustainable nanozyme synthesis methods has been the focus of relevant research. In this report, graphite-coated CeO nanoparticles (CeO NPs) with multiple defects (Ce defects, oxygen vacancies and carbon defects) were synthesized the culture filtrate of the extremely radioresistant bacterium R12 ( R12). The as-prepared CeO NPs exhibit remarkable oxidase (OXD)-like activity, efficiently catalyzing the oxidation of the chromogenic substrate 3,3',5,5'-tetramethylbenzidine (TMB) to form oxTMB, even in the absence of HO.
View Article and Find Full Text PDFIndian Childhood Cirrhosis: Report of 2 Cases With Review of Literature and Implication of Metallothionein Immunohistochemical Expression.
Pediatr Dev Pathol
January 2025
Prism Pediatric Gastro, Ahmedabad, Gujarat, India.
Indian childhood cirrhosis is a chronic liver disease in infants and children. Indian childhood cirrhosis is unique to the Indian subcontinent and occurs from 6 months to 5 years of age. We report 2 cases in a period of 5 years, including 1 male and 1 female.
View Article and Find Full Text PDFNative mercury, hydrargyrism and the birth of occupational toxicology in Colombia.
Environ Geochem Health
January 2025
Public Health Department, School of Medicine, Universidad Industrial de Santander, Bucaramanga, Colombia.
The "La Esperanza" native mercury mine in Aranzazu, (Caldas, Colombia) was active from 1948 until 1975. Before the final closure of the mine, the company began using dimercaprol (BAL, British Anti-Lewisite) and penicillamine for the treatment of hydrargyrism among workers. Mercury poisoning among miners was frequent due to precarious working conditions, inadequate technology, difficult terrain, and the high toxicity of native mercury within the mine.
View Article and Find Full Text PDFNitric Oxide-Releasing Nanoscale Metal-Organic Layer Overcomes Hypoxia and Reactive Oxygen Species Diffusion Barriers to Enhance Cancer Radiotherapy.
Adv Sci (Weinh)
January 2025
Department of Chemistry, The University of Chicago, Chicago, IL, 60637, USA.
Hafnium (Hf)-based nanoscale metal-organic layers (MOLs) enhance radiotherapeutic effects of tissue-penetrating X-rays via a unique radiotherapy-radiodynamic therapy (RT-RDT) process through efficient generation of hydroxy radical (RT) and singlet oxygen (RDT). However, their radiotherapeutic efficacy is limited by hypoxia in deep-seated tumors and short half-lives of reactive oxygen species (ROS). Herein the conjugation of a nitric oxide (NO) donor, S-nitroso-N-acetyl-DL-penicillamine (SNAP), to the Hf secondary building units (SBUs) of Hf-5,5'-di-p-benzoatoporphyrin MOL is reported to afford SNAP/MOL for enhanced cancer radiotherapy.
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