Background: Pharmacological therapy is currently the main treatment method for patients with Wilson disease (WD). We aimed to evaluate the efficacy and safety of the common treatment regimens in these patients.Methods: We conducted a systemic review and meta-analysis by searching multiple databases for studies from inception to October 2021. Outcomes of interest were the improved rate and safety of d-penicillamine and zinc salts treatment in WD patients. Two independent reviewers performed the study selection and data extraction.Results: Sixteen studies were included in this meta-analysis. The pooled improved rate for all included symptomatic WD patients was 78.0% (95% CI: 70.8%–85.2%). In symptomatic hepatic WD patients, there is no difference in the treatment efficiency of d-penicillamine and zinc salts (RR: 0.98, 95% CI: 0.86%–1.12%; p = 0.765). In neurological WD patients, the pooled improved rate of those who received d-penicillamine and zinc salts was 56.3% (95% CI: 37.5%–75.1%) and 80.2% (95% CI: 67.2%–93.2%), respectively. The incidence of adverse effects (RR: 2.42, 95% CI: 1.20%–4.88%; p = 0.014) and neurological deterioration (RR: 1.96, 95% CI: 1.31%–2.93%; p = 0.001) in all symptomatic WD patients treated with d-penicillamine was both higher than that of patients treated with zinc salts.Conclusion: Our analysis suggests that symptomatic WD patients treated with d-penicillamine have higher incidence of adverse effects and neurological deterioration than that of zinc salts. The therapeutic effectiveness of these two regimens does not seem to be significantly different, and these results must be interpreted with caution.Systematic Review Registration: PROSPERO registration, identifier CRD 42021287126.
IntroductionAlthough the vast majority of patients with a myocardial bridge (MB) are asymptomatic, the anomaly was found to be associated with stable or unstable angina, vasospastic angina, acute coronary syndrome, and even malignant arrhythmias and sudden cardiac death in some cases.MethodsBy retrieving the relevant literature on MB from 1 January 1980 to 31 July 2022 from the Web of Science Core Collection (WoSCC) database, we used the bibliometric tools, including CiteSpace, VOS viewer, and alluvial generator, to visualize the scientific achievements on MB.ResultsA total of 630 articles were included. The number of published articles was in a fluctuating growth trend. These publications came from 37 contries, led by the USA and China. The leading country on MB was the United States, the leading position among institutions was Stanford University, and the most productive researcher on MB was Jennifer A. Tremmel. After analysis, the most common keywords were myocardial bridge, mortality, coronary angiography, descending coronary artery, and sudden death.ConclusionOur findings can aid researchers in understanding the current state of MB research and in choosing fresh lines of inquiry for forthcoming investigations. Prevalence and prognosis, mechanism atherosclerosis, hemodynamic significance, and molecular autops will likely become the focus of future research. In addition, more studies and cooperations are still needed worldwide.
This study aims to investigate functional brain reorganization brought about by the loss of physical movement and sensory feedback in lower limbs in chronic spinal cord injury (SCI). Eleven paraplegia patients with SCI and 13 healthy controls (HCs) were recruited. The experimental task used was a visuomotor imagery task requiring subjects to engage in visualization of repetitive tapping movements of the upper or lower limbs. Blood oxygen level-dependent (BOLD) responses were captured during the experimental task, along with the accuracy rate and the response time. The SCI patients performed worse in the Rey Auditory Verbal Learning Test (RAVLT) and the Trail Making Test. SCI patients had a larger BOLD signal in the left lingual gyrus and right external globus pallidus (GPe) when imagining lower-limb movements. For the upper-limb task, SCI patients showed stronger BOLD responses than the HCs in extensive areas over the brain, including the bilateral precentral gyrus (preCG), bilateral inferior parietal gyrus, right GPe, right thalamus, left postcentral gyrus, and right superior temporal gyrus. In contrast, the HCs displayed stronger BOLD responses in the medial frontal gyrus and anterior cingulate gyrus for both upper- and lower-limb tasks than the SCI patients. In the SCI group, for the upper-limb condition, the amplitudes of BOLD responses in the left preCG were negatively correlated with the time since injury ( r = −0.72, p = 0.012). For the lower-limb condition, the amplitudes of BOLD responses in the left lingual gyrus were negatively correlated with the scores on the Short Delay task of the RAVLT ( r = −0.73, p = 0.011). Our study provided imaging evidence for abnormal changes in brain function and worsened cognitive test performance in SCI patients. These findings suggested possible compensatory strategies adopted by the SCI patients for the loss of sensorimotor function from the lower limbs when performing a limb imagery task.
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