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Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) is an autosomal recessive hereditary disorder characterized by cerebellar ataxia, sensory neuropathy and bilateral vestibulopathy. Recently a biallelic intronic AAGGG repeat expansion in the replication factor C1 (RFC1) gene was identified as the cause of this disorder. Clinical studies of genetically-confirmed CANVAS in the past 2 years have significantly expanded the clinical phenotype of the disease and the concept of RFC1-related disease was proposed. The clinical manifestations, characteristic auxiliary examination, genetic changes of CANVAS were reviewed and the new diagnostic criteria to improve clinicians′ awareness of the disease was discussed in this paper.
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Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a rare hereditary cerebrovascular disease. The clinical presentations are characterized by early-onset visual impairment, liver and kidney dysfunction and neurological symptoms. Imaging examinations are often misdiagnosed as neoplasms or tumefactive multiple sclerosis for invasive examination. Early and correct identification is essential for optimizing treatment strategies and comprehensive management. This review is to improve the understanding of RVCL-S, which may help to reach more accurate diagnosis and better treatment, and improve further research.
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Objective@#To explore the effect of Wenrun-Xinjin-Peiben paste on the nutrition status, quality of life and pulmonary function in the stable COPD patients.@*Methods@#A total of 94 malnutrition patients with stable COPD were randomly divided into treatment group (47 cases) and control group (47 cases). The patients in the control group took Symbicort 1 suction/time, bid, and the patients were given oxygen therapy, health and diet nutrition education. The patients in the treatment group were given Wenrun-Xinjin-Peiben paste 6 months as well as the control group treatment. The nutritional status (body mass index, triceps skinfold thickness, upper arm circumference, hemoglobin, albumin, prealbumin), quality of life and pulmonary function in the two groups were compared.@*Results@#The curative effect of TCM syndromes was 90.48% in the treatment group, which is significantly higher than 61.90 % in the control group (Z=3.173, P=0.002). The hemoglobin in the treatment group was superior to the control group (135.69 ± 16.42 g/L vs. 122.75 ± 14.99 g/L; t=12.346, P<0.001). The Albumin in the treatment group was superior to the control group (35.89 ± 6.14 g/L vs. 33.01 ± 6.20 g/L; t=2.139, P=0.035). The disease impacts domain of ST George's respiratory questionnaire in the treatment group was superior to the control group (24.9 ± 16.1 vs. 36.6 ± 16.9; t=3.252, P=0.002). Total score of ST George's respiratory questionnaire in the treatment group was superior to the control group (41.4 ± 14.9 vs. 48.0 ± 14.9; t=2.006, P=0.048). The FEV1 predicted value in the treatment group was superior to the control group (65.22% ± 4.01% vs. 60.17% ± 4.34%; t=5.538, P=0.000).@*Conclusions@#The Wenrun-Xinjin-Peiben paste combined with western medicine could help to improve the nutritional status and quality of life of patients in stable COPD.
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Objective To explore the effect of Wenrun-Xinjin-Peiben paste on the nutrition status, quality of life and pulmonary function in the stable COPD patients. Methods A total of 94 malnutrition patients with stable COPD were randomly divided into treatment group (47 cases) and control group (47 cases). The patients in the control group took Symbicort 1 suction/time, bid, and the patients were given oxygen therapy, health and diet nutrition education. The patients in the treatment group were given Wenrun-Xinjin-Peiben paste 6 months as well as the control group treatment. The nutritional status (body mass index, triceps skinfold thickness, upper arm circumference, hemoglobin, albumin, prealbumin), quality of life and pulmonary function in the two groups were compared. Results The curative effect of TCM syndromes was 90.48% in the treatment group, which is significantly higher than 61.90 % in the control group (Z=3.173, P=0.002). The hemoglobin in the treatment group was superior to the control group (135.69 ± 16.42 g/L vs. 122.75 ± 14.99 g/L; t=12.346, P<0.001). The Albumin in the treatment group was superior to the control group (35.89 ± 6.14 g/L vs. 33.01 ± 6.20 g/L; t=2.139, P=0.035). The disease impacts domain of ST George's respiratory questionnaire in the treatment group was superior to the control group (24.9 ± 16.1 vs. 36.6 ± 16.9;t=3.252, P=0.002). Total score of ST George's respiratory questionnaire in the treatment group was superior to the control group (41.4 ± 14.9 vs. 48.0 ± 14.9; t=2.006, P=0.048). The FEV1 predicted value in the treatment group was superior to the control group (65.22% ± 4.01% vs. 60.17% ± 4.34%; t=5.538, P=0.000). Conclusions The Wenrun-Xinjin-Peiben paste combined with western medicine could help to improve the nutritional status and quality of life of patients in stable COPD.
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To develop a patient-reported outcome (PRO) scale for patients with stable chronic obstructive pulmonary disease (sCOPD) and to study its clinical applicability in traditional Chinese medicine (TCM).
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<p><b>OBJECTIVE</b>To test and vertify the clinical efficacy of Chinese syndrome-differentiation therapy in treating stable chronic obstructive pulmonary disease.</p><p><b>METHOD</b>Two hundred and sixteen stable-COPD patients were randomly divided into 2 groups: the treated group and control group, they were treated with respiratory exercises increases Chinese medicine and respiratory exercises increases placebo for 4 weeks respectively. Then evaluate the health-related quality of life of patients with stable COPD by using SF-36 before and after treatment.</p><p><b>RESULT</b>Comparison of the health-related quality of life before and after treatment: the health-related quality of life in treated group was significantly improved after treatment and total score of SF-36 was better than control group (P < 0.05).</p><p><b>CONCLUSION</b>Respiratory exercises increases Chinese medicine could improve the health-related quality of life significantly in patients with stable COPD, moreover its efficacy was superior to respiratory exercises increases placebo.</p>