Articles: function.
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Pompe disease is caused by a deficiency of the enzyme acid alpha-glucosidase (GAA). People with infantile-onset disease have either a complete or a near-complete enzyme deficiency; people with late-onset Pompe disease (LOPD) retain some residual enzyme activity. GAA deficiency is treated with an intravenous infusion of recombinant human acid alglucosidase alfa, an enzyme replacement therapy (ERT). Alglucosidase alfa and avalglucosidase alfa are approved treatments, but cipaglucosidase alfa with miglustat is not yet approved. ⋯ One trial compared the effect of ERT to placebo in LOPD, showing that alglucosidase alfa probably improves 6MWT and respiratory function (both moderate-certainty evidence). Avalglucosidase alfa probably improves 6MWT compared with alglucosidase alfa (moderate-certainty evidence). Cipaglucosidase plus miglustat probably improves FVC compared to alglucosidase alfa plus placebo (moderate-certainty evidence). Other trials studied the adjunct effect of clenbuterol and albuterol along with alglucosidase alfa, with little to no evidence of benefit. No significant rise in adverse events was noted with all ERTs. The impact of ERT on some outcomes remains unclear, and longer RCTs are needed to generate relevant information due to the progressive nature of LOPD. Alternative resources, such as post-marketing registries, could capture some of this information.
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Review Meta Analysis
Re-evaluation for systematic reviews of traditional Chinese medicine in the treatment of chronic bronchitis.
Chronic bronchitis (CB) is a common clinical chronic respiratory disease, which has a high incidence in the middle aged and elderly population. With the development of the disease, the number of acute attacks becomes more and more frequent, which leads to the continuous decrease of lung function. If not treated in time, it will lead to a variety of complications and seriously affect the quality of life of patients. Traditional Chinese medicine (TCM) or TCM combined with western medicine is highly effective in the treatment of CB disease. In recent years, there are many systematic reviews on the use of TCM therapy in the treatment of CB, and the efficacy and safety of TCM in the treatment of CB diseases are evaluated. The aim of this study was to re-evaluate the Meta analysis/Systematic reviews (MAs/SRs) of TCM for the treatment of CB, aiming to provide a clinical basis for the treatment of CB by TCM. ⋯ The MAs/SRs methodological quality of using TCM for treatment CB is generally poor, the quality of reports as well as evidence are generally low, and the risk of bias is high, therefore we should treat these results with caution.
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At present, acute myelitis (AM) is a great challenge to diagnosis and treatment because of its complicated etiology, critical condition, and poor prognosis, and it is easy to leave different degrees of limb motor dysfunction. The report of this case is helpful to improve the understanding of AM after lumbar surgery, reduce misdiagnosis and provide reference for clinical treatment. ⋯ Due to the rarity of AM in clinical work, it is easy for doctors to ignore the disease and miss the best treatment stage, which will lead to serious sequelae.
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To discuss the early changes of Glu and Cho in the affected areas of different types of subjective cognitive decline, including amnestic MCI (aMCl), non-amnestic MCI (naMCI) and vascular cognitive impairment no dementia (VCIND), using Proton Magnetic Resonance Spectroscopy (1H-MRS) technology. Routine head MRI and lH-MRS examinations were performed on 50 clearly diagnosed aMCI patients, 44 naMCI patients, 44 VCIND patients, and 44 elderly individuals with normal cognitive function. Measure the volume of the patient bilateral hippocampus. ⋯ In the left frontal lobe, relative to the aMCI and NC, the mI/Cr values in the naMCI were higher (P < .05). The changes in the concentration of 1H-MRS metabolites in the hippocampus can indicate the presence of hippocampal neuronal damage before morphological changes occur in the hippocampus. 1H-MRS NAA/Cr can reflect the cognitive function changes of patients to a certain extent. There are regional differences in mI and Glu metabolism in the brain between aMCI and naMCI groups. 1H-MRS provides an effective basis for clinical differentiation between aMCI and naMCI.
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Review Meta Analysis
Lack of associations between thyroid dysfunction and obstructive sleep apnea-hypopnea syndrome: A meta-analysis.
Obstructive sleep apnea-hypopnea syndrome (OSAHS) is a comprehensive syndrome with endocrine and metabolic complications. This review aims to explore the correlation between thyroid hormone levels and the severity of OSAHS in patients. ⋯ The prevalence of thyroid dysfunction is relatively low in OSAHS individuals. Thyroid hormone levels show no significant difference between OSAHS patients and healthy subjects. Furthermore, there is no significant correlation between AHI and serum TSH, FT3, and FT4 levels. Based on existing data, the relationship between OSAHS and thyroid function remains controversial, and further in-depth research is warranted to validate the connection and elucidate the underlying mechanisms.