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Background: This study aims to guide the diagnosis and treatment of hepatolenticular degeneration (also named Wilson's disease, WD) and aid multidisciplinary clinicians in making reasonable and personalized treatment regimens.
Objectives: The authors aim to establish a systemic structure for Chinese Multidisciplinary Expert Consensus on Diagnosis and Treatment of Hepatolenticular Degeneration.
Method: We collaborated with experts from relevant branches of the Chinese Medical Association and multiple disciplines, along with statistical experts, to formulate this consensus. It is based on advancements in basic and clinical research on Wilson's disease, both domestically and internationally.
Results: It mainly consists of clinical manifestations, diagnosis, differential diagnosis, management, and prognosis in the context of Multi-Department treatment (MDT) in China.
Conclusion: This Chinese consensus incorporates four decades of institutional experience with thousands of Chinese Wilson's disease (WD) inpatients, as well as decades of international inpatient cases from East to West. It is hoped that this consensus will garner broader attention from clinicians worldwide.
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http://dx.doi.org/10.2174/011570159X349587250311072553 | DOI Listing |
Rev Med Interne
September 2025
Service d'hématologie biologique, CHU d'Amiens-Picardie, Amiens, France; HEMATIM UR4666, université Picardie Jules-Verne, Amiens, France.
The diagnosis of hemolysis is still based on straightforward biochemical parameters: haptoglobin (the most sensitive), lactate dehydrogenase (LDH), and unconjugated bilirubin. Anemia is not always present. Reticulocyte counts typically exceed 120×10/L, except in cases of associated vitamin deficiency or during the very early phase of acute hemolysis.
View Article and Find Full Text PDFFront Endocrinol (Lausanne)
September 2025
Department of Neurology, The First Affiliated Hospital of Anhui University of Chinese Medicine, Hefei, China.
Background: Wilson disease (WD), an inherited copper metabolism disorder, is linked to hepatic injury from copper accumulation-induced dyslipidemia. Children with WD have a high incidence of dyslipidemia, yet personalized risk assessment tools are lacking. This study established a predictive nomogram to provide foundational evidence for early detection in this population.
View Article and Find Full Text PDFAnn Clin Transl Neurol
September 2025
Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.
Objective: Neuroinflammation driven by extracellular copper contributes to neuronal damage in Wilson's disease (WD). This study investigated the relationship between brain metal burden and peripheral neuroinflammation markers in WD.
Methods: We conducted a cross-sectional study involving 89 participants, including patients with WD (n = 63), asymptomatic ATP7B heterozygous carriers (n = 12), and age/sex-matched controls (n = 14).
Curr Med Chem
August 2025
Shanghai Key Lab of Forensic Medicine, Key Lab of Forensic Science, Ministry of Justice, China (Academy of Forensic Science), Shanghai, 200063, China.
Introduction: This paper provides a comprehensive review examining the application of copper radionuclides, particularly 64Cu, in the diagnosis and potential therapy of various brain diseases.
Methods: Two researchers conducted an independent search of the PubMed and Web of Science databases for original research articles published in English. Following a screening process based on titles and abstracts, 42 publications reporting the use of copper radionuclides for diagnosing or treating brain diseases were selected for this review.
The anthelmintic praziquantel (PZQ) has been used for decades as the clinical therapy for schistosomiasis, and remains the only available drug. As a cheap and effective drug therapy for all human disease-causing species, usage of PZQ underpins mass drug administration strategies aimed at eliminating schistosomiasis as a public health problem by 2030. Concern over the potential emergence of resistance to PZQ is therefore warranted, as it would constitute a major threat to this approach.
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