The 100 top-cited articles in the field of Wilson’s disease from 1990 to 2022: A bibliometric study

Tang, Zhenchu; Lan, Ziwei; Li, Jing; Zhang, Hedong; Gao, Shan

doi:10.1016/j.heliyon.2023.e17785

Cited by 3 publications

(2 citation statements)

References 33 publications

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“…Although more than 900 ATP7B mutations have been described in the literature over the last 2 decades, the mechanisms involved in the development of WD are not yet fully understood. 17,34 Interestingly, the group from Austria claims that age and gender actually influence the WD phenotype more than the ATP7B genotype does. 32 Kayser-Fleischer rings were detected in 78.3% of patients but were absent in 2 (1.1%) patients with the neurologic form.…”

Section: Discussionmentioning

confidence: 99%

Wilson disease: the diagnostic challenge and treatment outcomes in a series of 262 cases

Deguti,

Araujo,

Terrabuio

et al. 2024

Arq Neuropsiquiatr

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Background Wilson disease (WD) is an autosomal recessive disorder that leads to organ toxicity due to copper overload. Early diagnosis is complicated by the rarity and diversity of manifestations. Objective To describe the diagnostic features and response to treatment in our cohort of WD patients. Methods This was a retrospective analysis of 262 WD patients stratified by clinical presentation, complementary exams, ATP7B genotyping, and response to treatment. Results Symptoms occurred at an average age of 17.4 (7–49) years, and patients were followed up for an average of 9.6 (0–45) years. Patients presented mainly with hepatic (36.3%), neurologic (34.7%), and neuropsychiatric (8.3%) forms. Other presentations were hematologic, renal, or musculoskeletal, and 16.8% of the patients were asymptomatic. Kayser-Fleischer rings occurred in 78.3% of the patients, hypoceruloplasminemia in 98.3%, and elevated cupruria/24h in 73.0%, with an increase after D-penicillamine in 54.0%. Mutations of the ATP7B gene were detected in 84.4% of alleles. Brain magnetic resonance imaging showed abnormalities in the basal ganglia in 77.7% of patients. D-penicillamine was the first choice in 93.6% of the 245 patients, and 21.1% of these patients were switched due to adverse effects. The second-line therapies were zinc and trientine. The therapeutic response did not differ significantly between the drugs (p = 0.2). Nine patients underwent liver transplantation and 82 died. Conclusion Wilson disease is diagnosed at a late stage, and therapeutic options are limited. In people under 40 years of age with compatible manifestations, WD could be considered earlier in the differential diagnosis. There is a need to include ATP7B genotyping and therapeutic alternatives in clinical practice.

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Section: Discussionmentioning

confidence: 99%

Wilson disease: the diagnostic challenge and treatment outcomes in a series of 262 cases

Deguti,

Araujo,

Terrabuio

et al. 2024

Arq Neuropsiquiatr

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“…Critical analysis of the highly cited publications (HCP) provides insights into the nature, status, and impact of any given research field (Gaviria-Marin et al 2019). It also presents an overview of the benchmark publications (Tang et al 2023), which have attracted the interest of researchers due to the significant breakthroughs. Intrinsically, researchers can effectively track the current research trends, controversial findings, and innovative methodologies and discern future directions in any given scientific research (Patel et al 2022).…”

Section: Highly Cited Publicationsmentioning

confidence: 99%

Low-Grade Waste Heat Recovery and Utilisation: A Bibliometric Analysis and Literature Review

Nyakuma

2024

Preprint

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This study examines the research landscape on the recovery and utilisation of low-grade waste heat (RU/LGWH) through bibliometric analysis and literature review based on publications indexed in Scopus from 2008 to 2022. During this period, a total of 135 publications and 4,759 citations were recorded in RU/LGWH research. Publications trends showed that high publication rates and scientific interest in the topic were largely occasioned by efforts to achieve humanity’s quest for cleaner sources of energy and net zero emissions. The most prolific researchers, affiliations and nations are Sheng Yang, Central South University and China, respectively. The observed dominance of Chinese-based researchers and institutions is ascribed to research funding available from funding organisations/programmes such as the National Natural Science Foundation, among others, that account for 6 out of the top 10 funders of RU/LGWH research. High rates of collaboration between countries (RC = 69.23%) compared to authors (RC = 54.05%) were observed during social network analysis. Hotspots analysis revealed that RU/LGWH research has been majorly focused on three core areas: Energy Recovery and Management (ERM), Economics and Efficiency Assessment (EEA), and Exergy and Performance Analysis (EPA). As such, the various stakeholders have devoted significant resources to exploring and exploiting the technological feasibility, economic viability, and thermodynamic performance of LGWH. Despite the enormous progress recorded, selected grey areas still exist, and we opine the prospects of the field greatly lie in finding solutions to these research gaps.

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Knowledge mapping of macrophage and its role in aneurysm from 1999 to 2022: A bibliometric analysis

Tang,

Gao,

Shi

et al. 2024

Heliyon

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The 100 top-cited articles in the field of Wilson’s disease from 1990 to 2022: A bibliometric study

Cited by 3 publications

References 33 publications

Wilson disease: the diagnostic challenge and treatment outcomes in a series of 262 cases

Wilson disease: the diagnostic challenge and treatment outcomes in a series of 262 cases

Low-Grade Waste Heat Recovery and Utilisation: A Bibliometric Analysis and Literature Review

Knowledge mapping of macrophage and its role in aneurysm from 1999 to 2022: A bibliometric analysis

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