Background and Objectives: This study aims to clarify the systems underlying regulation and regulatory roles of hydrogen combined with 5-Aza in the myogenic differentiation of adipose mesenchymal stem cells (ADSCs). Methods: and Results: In this study, ADSCs acted as an in vitro myogenic differentiating mode. First, the Alamar blue Staining and mitochondrial tracer technique were used to verify whether hydrogen combined with 5-Aza could promote cell proliferation. In addition, this study assessed myogenic differentiating markers (e.g., Myogenin, Mhc and Myod protein expressions) based on the Western blotting assay, analysis on cellular morphological characteristics (e.g., Myotube number, length, diameter and maturation index), RT-PCR (Myod, Myogenin and Mhc mRNA expression) and Immunofluorescence analysis (Desmin, Myosin and β-actin protein expression). Finally, to verify the mechanism of myogenic differentiation of hydrogen-bound 5-Aza, we performed bioinformatics analysis and Western blot to detect the expression of p-P38 protein. Hydrogen combined with 5-Aza significantly enhanced the proliferation and myogenic differentiation of ADSCs in vitro by increasing the number of single-cell mitochondria and upregulating the expression of myogenic biomarkers such as Myod, Mhc and myotube formation. The expressions of p-P38 was up-regulated by hydrogen combined with 5-Aza. The differentiating ability was suppressed when the cells were cultivated in combination with SB203580 (p38 MAPK signal pathway inhibitor). Conclusions Hydrogen alleviates the cytotoxicity of 5-Aza and synergistically promotes the myogenic differentiation capacity of adipose stem cells via the p38 MAPK pathway. Thus, the mentioned results present insights into myogenic differentiation and are likely to generate one potential alternative strategy for skeletal muscle related diseases.

Blau syndrome is a rare genetic disorder characterized by the a mix of granulomatous arthritis, uveitis, and dermatitis. Patients typically manifest multisystem involvement, including ocular, skin, and skeletal abnormalities. Blau syndrome is extremely rare, with a global incidence of less than one in a million among children. In this multidisciplinary consultation, we present a case of a 21-year-old young female patient having multisystemic involvement since early childhood. She was presented with multiple joint swelling, skin lesions, increased eye discharge, and accompanied by hypertension and arterial abnormalities, and received a diagnosis of uveitis. The patient had been receiving steroid treatment since the age of 6 and has tried various medications, with some improvement in joint swelling and ocular symptoms. Through this rare disease multidisciplinary consultation, we aim to provide guidance in the molecular diagnosis of the patient, multisystem assessment, and the selection and formulation of treatment plans. Additionally, we hope that by reporting this case, clinical physicians can gain a better understanding of the diagnosis and comprehensive treatment strategies for Blau syndrome, thereby improving the management and treatment of rare diseases.

Objective:To investigate the clinical and electrophysiological characteristics of facial onset sensory motor neuronopathy (FOSMN) syndrome.Methods:Ten patients diagnosed with FOSMN syndrome in Peking Union Medical College Hospital from January 2012 to December 2022 were included. The clinical and electrophysiological characteristics of patients were analyzed and summarized, and the genetic testing was also performed in these patients.Results:The age of onset was (56.6±6.5) years, and the longest survival duration of disease was 10 years. All patients had numbness around the face and mouth as the first symptom and abnormal blink reflex. A total of 52 sensory nerve conduction nerves were detected, among which 2 median nerves and 2 μlnar nerves showed decreased amplitude of sensory nerve action potential. Needle electromyography showed neurogenic lesions, with both progressive and chronic denervation. Whole exome sequencing identified the heterozygous variant c.272A>C in the exon 4 of the SOD1 gene resulting in the amino acid change p.Asp90Ala in 1 patient. In all patients, the disease progressed relentlessly and eventually led to involvement of respiratory muscle. Conclusion:FOSMN syndrome is characterized by abnormal blink reflex and sometimes abnormal sensory nerve conduction may be shown on electrophysiologic testing.

We presented an adolescent with recurrent intracranial hemorrhage and skin lesion. The diagnosis was unclear and the treatment was difficult. Through a multidisciplinary effort type Ⅰ interferon disease was suspected and later, an interferon-stimulated gene was further detected. Considering the high morbidity and fatality rate of recurrent intracranial hemorrhage, tofacitinib and hydroxychloroquine were administered. After treatment, the livedo reticularis was significantly regressed. Unfortunately, the intracranial hemorrhage recurred due to a pre-existing cerebral aneurysm, leading to death of the patient. The diagnosis and treatment of this case highlight the importance of multidisciplinary collaboration in the diagnosis and treatment of difficult and rare diseases.

Objective:The investigation and research on the application status of Hepatic Venous Pressure Gradient (HVPG) is very important to understand the real situation and future development of this technology in China.Methods:This study comprehensively investigated the basic situation of HVPG technology in China, including hospital distribution, hospital level, annual number of cases, catheters used, average cost, indications and existing problems.Results:According to the survey, there were 70 hospitals in China carrying out HVPG technology in 2021, distributed in 28 provinces (autonomous regions and municipalities directly under the central Government). A total of 4 398 cases of HVPG were performed in all the surveyed hospitals in 2021, of which 2 291 cases (52.1%) were tested by HVPG alone. The average cost of HVPG detection was (5 617.2±2 079.4) yuan. 96.3% of the teams completed HVPG detection with balloon method, and most of the teams used thrombectomy balloon catheter (80.3%).Conclusion:Through this investigation, the status of domestic clinical application of HVPG has been clarified, and it has been confirmed that many domestic medical institutions have mastered this technology, but it still needs to continue to promote and popularize HVPG technology in the future.

Objective:To investigate the clinical efficacy of Dog-Bone double button in the treatment of acute acromioclavicular dislocation under shoulder arthroscopy.Methods:A retrospective analysis was conducted of the 20 patients with acute acromioclavicular dislocation who had been treated at Department of Sports Medicine, Northern Jiangsu People's Hospital of Jiangsu Province from November 2018 to December 2020 by Dog-Bone double button under shoulder arthroscopy. They were 11 males and 9 females, aged from 31 to 63 years. Recorded were their visual analogue scale (VAS), Constant-Murley shoulder function score and range of shoulder anteflexion at preoperation and the last follow-up, as well as complications and the X-ray parameters at one month postoperation and the last follow-up [including coracoclavicular distance (CCD), distance between the upper and lower Dog-Bone titanium plates (DDD), angle between the coracoid process tunnel and the tangent line of the superior clavicle (CTCA), and widths of the clavicle tunnel and the coracoid process tunnel].Results:The 20 patients were followed up for 6 to 12 months (average, 10.5 months). Their preoperative VAS score, Constant score, and range of shoulder anteflexion were 3.0 (3.0, 4.0), 57.0 (54.3, 61.5) and 130° (110°, 140°), which were significantly improved to 0 (0, 0.8), 90.0 (86.5, 91.0) and 170° (170°, 180°) at the last follow-up ( P<0.05). Their CCD, DDD, CTCA, and widths of the clavicle tunnel and the coracoid process at one month postoperation were (5.0±1.0) mm, (32.4±3.5) mm, 91.7° (88.5°, 104.9°), 3.0 (3.0, 3.0) mm and 3.0 (3.0, 3.0) mm, which were significantly improved to (6.3±1.3) mm, (32.8±3.7) mm, 84.8° (81.8°, 92.0°), 3.5 (3.4, 3.6) mm and 3.2 (3.1, 3.3) mm with the exception of DDD at one month postoperation ( P<0.05). The last follow-up observed postoperative reduction loss in only one patient. Conclusion:For acute acromioclavicular joint dislocation, the Dog-Bone fixation technique under shoulder arthroscopy can lead to fine surgical efficacy and patient satisfaction, because it has advantages of limited trauma, good functional recovery of the shoulder, and effective prevention of reduction loss.

The economic evaluation compares the costs and effectiveness of different strategies for osteoporosis prevention and treatment to facilitate decision-making. In this paper, international and Chinese economic evaluation studies on osteoporosis prevention strategies were reviewed and results were summarized. We have found that the research design and quality of reporting need to be improved. Furthermore, both general and osteoporosis-specific health economic evaluation guidelines were introduced to improve the quality of economic evaluation in osteoporosis in China. Accuracy and integrity of data used in economic evaluation also should be improved.

Objective:To evaluate the prognostic value of extravascular lung water index (EVLWI) , soluble intercellular adhesion molecule-1(sICAM-1) and Krebs yon den lungen-6 (KL-6) in severe pneumonia patients with Severe Acute Respiratory Syndrome (ARDS).Methods:A prospective study was conducted in Respiratory Intensive Care Unit of the Affiliated Zhengzhou Central Hospital of Zhengzhou University from October 2017 to February 2020. The study included 65 severe pneumonia patients with ARDS, who was performed by measurement of pulse index continuous cardiac output and survived more than 3days after admission. The Extravascular Lung Water Index (EVLWI) , sICAM-1, KL-6 and Oxygenation Index(OI) on 1st, 3rd and 5th day were detected. APACHEⅡ score, patient survival events (days) and survival outcome were recorded. Correlation analysis between EVLWI, sICAM-1, KL-6 and OI was performed on the 1st, 3rd and 5th day after admission. Independent risk factors of mortality in severe pneumonia patients with ARDS were analyzed by multiple logistic regression. Receiver operating characteristic curve was drawn, and the prognostic value of each parameter was assessed finally.Results:The PCT, EVLWI, sICAM-1, KL-6 and APACHEⅡ score in the death group were significantly higher than those in the survival group ( P<0.05) at RICU admission, and the length of RICU stay was significantly shorter than that in the survival group ( P<0.05), while differences in other clinical characteristics between two groups were not statistically significant ( P>0.05) . These parameters including levels of EVLWI, sICAM-1, KL-6, Procalcitonin and APACHE Ⅱscore in the death group were significantly higher than those in the survival group on the 1st, 3rd and 5th day ( P<0.05), whereas the OI was significantly lower than that of the survival group on the 3rd and 5th day ( P<0.05). Logistic regression analysis showed that EVLWI, sICAM-1, KL-6 level were significantly related with the mortality of these patients. The levels of sICAM-1, kl-6 and EVLWI on 1st, 3rd and 5th day after RICU admission showed a significant negative correlation with OI ( P<0.001). Whereas, The levels of sICAM-1, kL-6 on 1st, 3rd and 5th day showed a significant positive correlation with EVLWI ( P<0.001). The sensitivity and specificity of sICAM-1, KL-6 combined with EVLWI in prognosis evaluation on 1st, 3rd and 5th day were 75.0%, 84.4%, 85.0%, 66.7%, 80.0%, 86.7%, respectively. The AUC was 0.864, 0.881, 0.892 on 1st, 3rd and 5th day, respectively ( P<0.001), which had a better prognostic value than each of them. Conclusions:EVLWI, sICAM-1 and KL-6 were independent risk factors for the prognosis of severe pneumonia patients with ARDS. The combination of EVLWI, sICAM-1 and KL-6 might be important in early predicting the prognosis of the 28d mortality.

Objective: To investigate the correlations of indexes of isokinetic muscle testing with grip, the circumference of muscle, the time up and go test(TUGT), and the distance of 6-minutes walk test(6MWT). And to identify the availability of isokinetic muscle testing in diagnosing sarcopenia in the elderly. Methods: A total of 27 elderly patients with sarcopenia admitted in rehabilitation department of our hospital from Jan.2017 to Dec.2017 were enrolled in this cross-sectional study.Patients were evaluated in many aspects, including comprehensive evaluation by the comprehensive assessment, the skeletal muscle mass detection by dual energy X-Ray absorptiometry, the power of gripping by dynamometer, the circumference of muscle of upper and lower extremities, the muscle strength by isokinetic muscle testing, the sports ability by TUGT and 6MWT. Results: Isokinetic muscle testing including the knee flexion, knee extension, elbow extension, wrist extension, TUGT, 6MWT, the power of left gripping, the circumference of left forearm muscle showed a negative correlations to the age(r=-0.54, -0.44, -0.52, -0.61, -0.53, -0.41 and-0.39, P<0.05). A positive correlation were observed between TUGT and the age(r=0.44, P<0.05). The positive correlations between every index in isokinetic muscles testing and the power of both left and right gripping were observed(r=0.66, 0.67, 0.67, 0.67, 0.56, 0.62, 0.54, 0.54, 0.64, 0.59, 0.59 and 0.56, P<0.05). The knee flexion, knee extension, elbow flexion, elbow extension and wrist extension of Isokinetic muscle testing showed a positive correlations to 6MWT(r=0.71, 0.65, 0.62, 0.60 and 0.59, P<0.05). The knee flexion, knee extension, elbow extension and wrist extension of isokinetic muscle testing showed a negative correlations to TUGT(r=-0.57, -0.52, -0.42 and-0.48, P<0.05). Every index of isokinetic muscle testing showed a positive correlations to the circumference of both forearms(r=0.45, 0.47, 0.52, 0.42, 0.41, 0.44, 0.49, 0.52、0.60, 0.54, 0.44 and 049, P<0.05). Conclusions Isokinetic muscle testing can accurately evaluate the changes of muscle strength in the elderly with sarcopenia.It can be considered as the reference index of motor function in diagnosing sarcopenia in the elderly.

Objective To investigate the protective effects of antioxidant taurine plus metoprolol on the blood pressure(BP)and blood vessel function of elderly spontaneously hypertensive rats(SHR).Methods SHRs were divided into three groups (6 rats,each):metoprolol group (100 mg · kg-1 · d-1,intragastric administration);taurine group (200 mg · kg-1 · d-1,intraperitoneal injection);taurine plus metoprolol group(metoprolol 100 mg· kg-1 · d-1,intragastric administration ± taurine 200 mg· kg-1 · d-1,intraperitoneal injection).The control group[6 Wistar-Kyoto(WKY) rats]was treated with the same volume of sterile normal saline on the same schedule in intragastric administration and intraperitoneal injection.Blood pressure variability(BPV),diurnal variation of BP,the level of glutathion peroxidase (GSH-Px),malonaldehyde (MDA) and the aorta GSTM1 enzyme expression were evaluated before and 14 days after treatment.Results The serum GSH-Px activities were higher in metoprolol group[(2 759.8 ± 117.6) kU/L],taurine group [(2 848.0 ± 280.2) kU/L] and taurine plus metoprolol group[(3 052.8±283.7)kU/L]than in control group[(2 368.0± 60.4) kU/L] (all P＜0.05).Fourteen days after treatment,MDA level was significantly lower in taurine group[(9.5±0.7)ng/L,P＜0.01]than in control group[(13.7±1.5)ng/L].However,there was no statistical difference in MDA level between metoprolol group/taurine plus metoprolol group and control group.Forty weeks after treatment with taurine,GSTM1 protein expression was increased,but it had no statistical difference.Conclusions The combined antioxidant taurine and metoprolol can effectively decrease BPV of elderly SHRs,in which the antioxidant effect might be associated with elevated GSTM1 protein expression.