Objective To investigate the status quo and its influencing factors of nurses'organizational silence in 3 Grade A general hospitals.Methods Convenient sampling method was used to investigate clinical nurses in 3 Grade A general hospitals in Xi'an from April to August 2023 by general data questionnaire,nurses'organizational silence questionnaire and hospital magnetic factor scale.Multiple linear regression was used to analyze the influencing factors of organizational silence.Results A total of 855 nurses completed the study.The total silence score of nurses was(56.33±8.55);The total score of hospital magnetic level was(107.63±12.85).There was a negative correlation between nurse tissue silence and hospital magnetic level(r=-0.318,P<0.01).Hospital magnetic level,age,job title and working time were the influential factors of nurses'organizational silence(all P<0.001),which together explained 62.60%of the variation.Conclusions The silence of nurses'tissue and the level of hospital magnetism are in the low-medium level.Nurses are younger in age,lower in professional title,shorter in nursing age and lower in hospital magnetism level,the higher the tissue age level is,the nursing managers can reduce the tissue silence of nurses by improving the hospital magnetism level.

Objective:To investigate the clinical features and outcomes of adolescence-onset methylmalonic acidemia (MMA) and explore preventive strategies.Methods:This was a retrospective case analysis of the phenotypes, genotypes and prognoses of adolescence-onset MMA patients. There were 55 patients diagnosed in Peking University First Hospital from January 2002 to June 2023, the data of symptoms, signs, laboratory results, gene variations, and outcomes was collected. The follow-ups were done through WeChat, telephone, or clinic visits every 3 to 6 months.Results:Among the 55 patients, 31 were males and 24 were females. The age of onset was 12 years old (range 10-18 years old). They visited clinics at Tanner stages 2 to 5 with typical secondary sexual characteristics. Nine cases (16%) were trigged by infection and 5 cases (9%) were triggered by insidious exercises. The period from onset to diagnosis was between 2 months and 6 years. Forty-five cases (82%) had neuropsychiatric symptoms as the main symptoms, followed by cardiovascular symptoms in 12 cases (22%), kidney damage in 7 cases (13%), and eye disease in 12 cases (22%). Fifty-four cases (98%) had the biochemical characteristics of methylmalonic acidemia combined with homocysteinemia, and 1 case (2%) had the isolated methylmalonic acidemia. Genetic diagnosis was obtained in 54 cases, with 20 variants identified in MMACHC gene and 2 in MMUT gene. In 53 children with MMACHC gene mutation,1 case had dual gene variants of PRDX1 and MMACHC, with 105 alleles. The top 5 frequent variants in MMACHC were c.482G>A in 39 alleles (37%), c.609G>A in 17 alleles (16%), c.658_660delAAG in 11 alleles (10%), c.80A>G in 10 alleles (10%), c.567dupT and c.394C>T both are 4 alleles (4%). All patients recovered using cobalamin, L-carnitine, betaine, and symptomatic therapy, and 54 patients (98%) returned to school or work.Conclusions:Patients with adolescence-onset MMA may triggered by fatigue or infection. The diagnosis is often delayed due to non-specific symptoms. Metabolic and genetic tests are crucial for a definite diagnosis. Treatment with cobalamin, L-carnitine, and betaine can effectively reverse the prognosis of MMA in adolescence-onset patients.

Objective:To explore the clinical characteristics and genetic variants in three children with late-onset Multiple acyl-Coenzyme A dehydrogenase deficiency (MADD type Ⅲ).Methods:Clinical data of three children diagnosed with late-onset MADD at the Children′s Hospital Affiliated to Zhengzhou University between March 2020 and March 2022 were retrospectively analyzed. All children were subjected to whole exome sequencing (WES), and candidate variants were verified by Sanger sequencing. All children had received improved metabolic therapy and followed up for 1 ~ 3 years.Results:The children had included 2 males and 1 female, and aged from 2 months to 11 years and 7 months. Child 1 had intermittent vomiting, child 2 had weakness in lower limbs, while child 3 had no symptom except abnormal neonatal screening. Tandem mass spectrometry of the three children showed elevation of multiple acylcarnitines with short, medium and long chains. Children 1 and 2 showed increased glutaric acid and multiple dicarboxylic acids by urine Gas chromatography-mass spectrometry (GC-MS) analysis. All children were found to harbor compound heterozygous variants of the ETFDH gene, including a paternal c. 1211T>C (p.M404T) and a maternal c. 488-22T>G variant in child 1, a paternal c. 1717C>T (p.Q573X) and a maternal c. 250G>A (p.A84T) variant in child 2, and a paternal c. 1285+ 1G>A and maternal c. 629A>G (p.S210N) variant in child 3. As for the treatment, high-dose vitamin B2, levocarnitine and coenzyme Q 10 were given to improve the metabolism, in addition with a low fat, hypoproteinic and high carbohydrate diet. All children showed a stable condition with normal growth and development during the follow-up. Conclusion:The compound heterozygous variants of the ETFDH gene probably underlay the muscle weakness, remittent vomiting, elevated short, medium, and long chain acylcarnitine, as well as elevated glutaric acid and various dicarboxylic acids in the three children with type Ⅲ MADD.

Objective:To investigate the quality of life in children with McCune-Albright syndrome (MAS) and its influencing factors, so as to provide scientific basis for improving their quality of life.Methods:The clinical data of 31 children with MAS diagnosed (MAS group) and followed up in Henan Children′s Hospital from June 2015 to December 2021 were retrospectively analyzed.During the same period, 37 healthy age and sex-matched children at a ratio of 1∶1 were recruited as healthy control group.The children′s Quality of Life Universal Core Scale (PedsQL?4.0) was used for the investigation and comparative analysis.Statistical analysis was performed using the independent sample t test, Chi- square test and multiple regression analysis. Results:Compared with the healthy control group, the physiological function ( t=2.092, P<0.05), emotional function ( t=2.373, P<0.05) and total score ( t=2.360, P<0.05) of MAS group significantly decreased.Multiple regression analysis showed that physiological function was negatively correlated with the annual number of vaginal bleeding ( t=-2.367, P<0.05) and the age of first fracture ( t=-2.606, P<0.05). Social function was negatively correlated with the number of fractures ( t=-2.481, P<0.05). Conclusions:The overall quality of life of MAS children is low, especially the quality of physiological function and emotional function.The annual number of vaginal bleedings, the age of the first fracture and the number of fractures are influencing factors for the reduction of the quality of life of children with MAS.

Objective:To establish the mouse model with radiation-induced pulmonary fibrosis, and to identify and analyze it from the aspects of function, imaging and pathology.Methods:Thirty C57BL/6 mice were randomly divided into the control group, 16 Gy irradiation group and 20Gy irradiation group. The mice in the irradiation groups received a single 16 Gy or 20 Gy chest X-ray irradiation, and underwent functional examination, imaging examination and pathological examination at 3 and 6 months after irradiation.Results:At 6 months after irradiation, hair on the chest and back of the mice turned white and fell off, and the airway resistance was increased significantly. CT images showed extensive patch shadows and consolidation in the lung. Three dimensional reconstruction suggested that the lung of mice was distorted and deformed, and the volume was decreased significantly. Pathological examination confirmed that there was extensive pulmonary fibrosis.Conclusions:Significant pulmonary fibrosis occurs after 6 months of chest irradiation in mice. The animal model of radiation-induced pulmonary fibrosis in C57BL/6 mice was successfully established.

Objective To investigate the relationship between the level of circulating CD133+/KDR+ endothelial progenitor cells (EPCs) and outcome in patients with acute ischemic stroke.Methods Inpatients with first-ever ischemic stroke within 24 hfrom the onset and age-and sex-matched healthy subjects were enrolled in the study.The demographic and clinical data of the patients were collected.The level of CD133+/KDR+ EPCs was detected by flow cytometry.All patients were followed up at 90 d.The modified Rankin Scale was used to evaluate the clinical outcome,0-2 was defined as good outcome and >2 was defined as poor outcome.Results A total of 126 consecutive patients with first-ever ischemic stroke within 24 hfrom the onset and 60 age-and sex-matched healthy subjects were enrolled.In patients with ischemic stroke,33 (26.19%) were large artery atherosclerosis (LAA),74 (58.73%) were small artery occlusion (SAO),19 (15.08%) were cardioembolism (CE);82 (65.08%) had good outcomes and 44 (34.92%) had poor outcomes.The number of circulating EPCs at baseline in patients of the LAA subtype (0.071%±0.018%),CE subtype (0.068%±0.16%) and SAO subtype (0.118%±0.12%) was significantly lower than that in the control group (0.246%±0.052%;all P<0.05),and the CE subtype (P=0.028) and LAA subtype (P=0.037) were significantly lower than the SAO subtype;the CE subtype was lower than the LAA subtype,but the difference was not statistically significant (P=0.762).The proportions of patients with LAA subtype (40.91% vs.18.29%;χ2=7.577,P=0.006) and CE subtype (29.55% vs.7.32%;χ2=11.049,P=0.001) and atrial fibrillation (29.55% vs.10.98%;χ2=6.582,P=0.009),and age (69.64±9.62 years vs.61.12±7.31 years;t=5.570,P<0.001),and baseline NIHSS score (14.16±4.22 vs.6.96±2.04;t=12.919,P<0.001),baseline systolic blood pressure (176.06±13.42 mmHg vs.164.12±11.69 mmHg,1 mmHg=0.133 kPa;t=5.187,P<0.001),low-density lipoprotein cholesterol (2.92±0.52 mmol/L vs.2.49±0.36 mmol/L;t=5.447,P<0.001),fasting blood glucose (8.76±2.88 mmol/L vs.6.82±2.24 mmol/L;t=4.185,P<0.001),C-reactive protein (7.62±1.82 mg/L vs.4.57±1.58 mg/L;t=9.790,P<0.001),and D-dimer (1.14±0.08 mg/L vs.0.97±0.22 mg/L;t=4.946,P<0.001) levels in the poor outcome group were significantly higher than those in the good outcome group,while the proportion of the SAO subtype patients (29.55% vs.74.39%;χ2=23.759,P<0.001),high-density lipoprotein cholesterol (0.94±0.68 mmol/L vs.1.16±0.14 mmol/L;t=2.829,P=0.005),and baseline EPCs (0.069%±0.018% vs.0.098%±0.021%;t=7.755,P<0.001) were significantly lower than those in the good outcome group.Multivariate logistic regression analysis showed that the higher baseline NIHSS score (odds ratio 1.242,95% confidence interval 1.126-1.372;P<0.001),CE subtype (odds ratio 3.460,95% confidence interval 1.312-5.146;P=0.016),and the lower baseline EPCs (odds ratio 1.632,95% confidence interval 1.006-3.024;P<0.001) were the independent risk factors for poor outcome in patients.Conclusion s The level of circulating EPCs was decreased significantly in patients with acute ischemic stroke,and the lower level of baseline EPCs was an independent predictor of poor outcome in patients with ischemic stroke at 90 d.

Objective To systematically evaluate the efficacy and safety of stereotactic body radiotherapy (SBRT) for resectable stage Ⅰ non-small cell lung cancer (NSCLC).Methods Clinical trials of SBRT or surgery for resectable stage Ⅰ NSCLC were collected by computerized search of Cochrane Library,MEDLINE,EMbase,CBM,CNKI,and VIP.Literature selection,quality evaluation,and data extraction were performed by two inspectors based on the inclusion and exclusion criteria.A meta-analysis was performed on the enrolled studies using RevMan 5.3 software.Results A total of four clinical trials involving 410 patients were included.The results of meta-analysis showed that there was no significant difference in 3-year overall survival rate between SBRT and surgery (RR=1.13,95％ CI=0.66-1.94,P=0.66);there was no significant difference in local control rate between SBRT and surgery (RR=0.71,95％ CI=0.26-1.93,P=0.50);patients treated with SBRT had significantly lower incidence rates of grade 3-4 adverse reactions than those treated with surgery (RR=0.29,95％ CI=0.16-0.53,P=0.000).Conclusions SBRT shows equivalent efficacy to surgery in the treatment of resectable stage Ⅰ NSCLC.However,due to the limitations in this systematic evaluation,the conclusion needs to be further confirmed by large randomized controlled trials.

Objective To analyze the clinical epidemiology of primary hepatocellular carcinoma (PHC) in the Xinjiang region. Methods Clinical data of the patients with PHC were collected at First Affiliated Hospital of Xinjiang Medical University from 5 577 cases from January 2002 to December 2014, their gender, race, age, household distribution, hepatitis virus-positive rate were analyzed retrospectively. Results Among the 5 577 eases, the men/women gender ratio was 3.45∶1;the proportion of Han, Uighur, Kazakh, and other ethnic groups (Hui, Mongolian, Manchu, Xibo) was 79.67%, 9.86%, 4.55%, 3.31%and 2.61%, respectively. The Constituent ratio difference between Uighur and Han was significant (P<0.05);4 232 patients had hepatitis B surface antigen (HBsAg) detection, and 3 833 patients had HCV antibody (HCV-Ab) detection. HBsAg was positive in 2 560 cases (60.49%), HCV-Ab was positive in 490 cases (12.78%). Hepatitis B virus detection positive rate in Uygur was 35.52%, in Kazak was 40.00%, which was lower than the Han's (65.68%, P<0.05). Urban and rural population had 3589 cases (64.35%) and 1988 cases (35.65%). Conclusion An increased risk for PHC was found in hepatitis virus-positive patients, the Xinjiang Uygur and Kazak people had significantly lower prevalence of HBV infection than the Han's. Appropriate measures should be taken for clinical diagnosis, treatment and prevention of PHC.

Angioplasty, Balloon, Coronary ; Heart Injuries ; drug therapy ; etiology ; Humans ; Thrombin ; administration & dosage

BACKGROUND:Femoral fractures easily cause damages to patient’s blood vessels and nerves. If there is no proper treatment, it is easy to result in physical disabilities in patients. Early neurovascular repair may have a positive impact on the patients. OBJECTIVE: To compare and analyze the clinical effects of early neurovascular repair on femoral fractures combined with knee joint injuries. METHODS: Eighty patients with femoral fractures combined with knee joint injuries were selected and equaly divided into control group (the injury time was > 8 hours but≤ 48 hours) and observation group (the injury time≤ 8 hours) prior to early neurovascular repair. Amputation rate and the time of femur and knee ligament healing in patients with no amputation were analyzed in the two groups, and Lysholm scores were measured to assess the knee function of patients without amputation in the two groups. RESULTS AND CONCLUSION: The amputation rate was 22% in the control group and 5% in the observation group, and there was a significant difference between the two groups (P < 0.05). The time of femoral and ligament healing in the observation group was significantly lower than that in the control group (P < 0.05). Lysholm scores were also better in the observation group than the control group (P < 0.05). These findings indicate that early neurovascular repair is preferred for patients with confirmed femoral fractures combined with knee joint injuries as soon as possible.