Objective:To analyze regional differences in MMACHC gene variations among patients with cblC-type methylmalonic acidemia (MMA) in China and to explore the relationship between these variations and neonatal screening, biochemical markers and prognosis.Methods:Retrospective case summary. Clinical and laboratory data, including general condition, biochemical markers and genetic analysis, were collected from 1 859 cblC MMA patients from 2005 to 2023. Patients were divided into 7 groups according to their regions: north China, northeast China, east China, central China, south China, southwest China and northwest China. They were also classified into neonatal screening and non-neonatal screening groups. Mann-Whitney U and Kruskal-Wallis tests were used to compare biochemical marker levels. In contrast, the Chi-square test was applied to compare MMACHC gene variant frequencies, neonatal screening proportion, onset age and prognosis between groups. Results:Among 1 859 cases of cblC MMA, 1 019 were male and 840 were female, with a consultation age of 1.0 (0.1, 5.0) month. A total of 1 787 cases carried compound heterozygous or homozygous variants and only 1 variant site was identified in 72 cases. The 10 most frequent variants were c.609G>A (1 238 cases), c.658_660delAAG (343 cases), c.80A>G (284 cases), c.482G>A (239 cases), c.567dupT (191 cases), c.656_658delAGA (131 cases), c.217C>T (109 cases), c.394C>T (105 cases), c.445_446delTG (51 cases) and c.1A>G (50 cases). The frequency of the c.609G>A was the lowest in northwest China (28.8% (44/154), χ2=-18.42, P<0.05). The frequency of the c.567dupT was the most common in southwest China (25.0% (20/80), χ2=71.70, P<0.001) and c.656_658delAGA had the highest frequency in northeast China (9.3% (19/205), χ2=32.08, P<0.001). Non-missense variants (91.2% (62/68), 88.5% (46/52)) and early-onset patients (90.0% (36/40), 94.4% (34/36)) were both more prevalent in southwest and south China ( χ2=14.95, 31.69, both P<0.05). The proportion of neonatal screening was the lowest in south China (22.2% (8/36), χ2=98.48, P<0.05), where the mortality rate was the highest (19.1% (4/21), χ2=38.98, P<0.001). East China exhibited the highest frequency of missense variants (21.5% (339/1 579)), the highest proportion of patients identified through neonatal screening (54.5% (465/853)), and a more significant proportion of patients with good prognosis (36.6% (227/621), χ2=14.57, 93.49, 38.98, all P<0.05). In addition, the c.482G>A variant was more frequent in patients diagnosed by neonatal screening compared to those diagnosed by other methods (8.3% (132/1 586) vs. 5.9% (122/2 060), χ2=7.97, P<0.05). Conclusions:The frequency of MMACHC gene variation varies across different regions. The c.609G>A was least frequent in northwest China, c.567dupT was most common in southwest China, and c.656_658delAGA was most prevalent in northeast China. South China had the lowest neonatal screening rate and the highest mortality. At the same time, east China exhibited the highest frequency of missense variants, the highest proportion of patients identified through neonatal screening and the best prognosis. The c.482G>A variant was more frequent in patients diagnosed by neonatal screening compared to those diagnosed by other methods.

Objective To assess the value of vascular endothelial growth factor (VEGF) expression in the prognosis of glioma patients.Methods PubMcd,EMbase,Cochrane Library,CNKI and Wanfang data were searched for collecting retrospective cohort studies on the correlation between VEGF expression and prognosis of glioma patients,and relevant articles were also retrieved from inception to June,2016.Two reviewers independently screened the literature,extracted the data,and evaluated the quality.Then Meta analysis was performed with RevMan5.3 software,and the publication bias of literature was evaluated by means of Begg's funnel plot and Egger's method.Results A total of 19 studies with 1657 cases were included.No significant publication bias was found in all groups.The analysis demonstrated positive associations between VEGF expression and poor prognosis (hazard ratio [HR]=1.39,95％CI:1.12-1.73),and the difference was statistically significant (P=0.003).Subgroup analysis showed that the high expression of VEGF in the European and American patients was more valuable than that in the Asian patients in the prognosis of gliomas;large samples of clinical studies of high VEGF expression were more valuable in the prognosis of gliomas than small samples.The detection of VEGF expression by different methods suggested that the patients with high expression of VEGF had poor prognosis;HR provided by the literature suggested that VEGF over-expression was associated with poor prognosis.Conclusion The expression of VEGF has predictive value for the survival time of glioma patients and VEGF can be recommended as a useful survival biomarker in clinical practice.

Objective To explore the application effect of dynamic case scenario simulation in emergency ability training of new nurses in Department of Neurosurgery.Methods A total of 28 nurses newly recruited in 2015 and 2016 to Department of Neurosurgery, Huai'an First People's Hospital, Nanjing Medical University were selected. According to time of joining the department, the 15 nurses recruited in 2015 were selected as control group, and the 13 nurses enrolled in 2016 as observation group. Nurses in the control group were trained by stages, i.e. technique training after theory training. On the basis of above mentioned training stages, nurses in the observation group were trained in simulated scenario, with 1 dynamic case added to each training stage. Emergency management ability, self evaluation of the nurses and patients' satisfaction after the training were compared between the two groups.Results After the training, scores of ability in disease observation, emergency cooperation and first-aid operation of new nurses in observation group were (27.41±1.32), (29.59±1.06) and (38.55±1.58), all higher than those in the control group (t=7.24, 12.26, 10.07;P<0.01). It was shown in results of self-evaluation that, ability to link theory with reality, to arouse subjective initiative and participation of nurses in the observation group were higher than those in the control group (P<0.05). Satisfaction of patients towards nurses in the observation group was 96.15%, higher than 86.00% in the control group (χ2=8.524,P<0.01).Conclusions Trained with method of dynamic case scenario simulation, emergency response ability of new nurses can be improved rapidly in a short time. Also, patients are more satisfied with the nursing. This training management method is worth promoting.

ObjectiveTo discuss the effects of continuing nursing on the living condition of patients with severe craniocerebral injury in neurosurgery department.Methods A total of 303 patients with severe craniocerebral injury from January 2008 to December 2009 in neurosurgery department of Huai'an First People's Hospital,Nanjing Medical University were randomly divided into the control group (140 cases) and the intervention group (163 cases). Patients in the control group were followed up once by the phone at the first week after discharge. There was no longer follow-up after the first week and patients could call or visit hospital in case of problems. Patients in the intervention group received continuing nursing intervention by telephone follow-up visit,the establishment of continuing nursing WeChat group,family visit and the establishment of brain club. Patients were followed up from the first week after discharge until the death of patients or December 2015. Survival rate of two groups was evaluated by survival analysis.Results The survival rate of patients in the intervention group and the control group were 78.8% and 62.3%,which were calculated by the Kaplan-Meier method. The survival rate of patients in the intervention group was significantly higher than that in the control group (χ2=33.91,P<0.05).Conclusions Continuing nursing helps to improve the survival situation of patients with severe craniocerebral injury in the process of time.

Objective To explore the application effects of QCC to the neurosurgery nursing scientific research. Methods We established the QCC activity team in 2013, identified the theme of "improving the ability of neurosurgery nursing scientific research" and analyzed the reasons of problems regarding the neurosurgery nursing research before the establishment of QCC; then we set targets, and formulated and implemented the corresponding measures. We compared the implementation of neurosurgery nursing research before (2012) and after the establishment of QCC. Results The total number of published article was 24 after the QCC, and it was 10 before the QCC (χ2 =11. 53, P <0. 05 ); all the team members had gained an improvement in understanding QCC and in the ability of operating QCC; the power of execution had been improved as well. Additionally, the ability of teamwork, group cohesiveness, and nursing professional diathesis, confidence, and initiative had also been improved and increased. Conclusions The implementation of QCC can improve research awareness of nursing staff members, nursing research ability and writing level of thesis, so as to improve the quality of care in our department.

Objective To explore the effects of individualized nursing intervention on quality of life in patients with hypertensive cerebral hemorrhage. Methods A total of 100 patients with hypertensive cerebral hemorrhage treated in in our hospital from March to August 2014 were selected and divided into control group and observation group, with 50 patients in each group. The patients of control group were given the routine specialist care measures, while the patients of observation group were additionally received personalized care interventions on the basis of routine care measures. We created a profile for each patient and carried out the related lectures plan, rehabilitation guidance, psychological support, extended care interventions and other interventions. The quality of life and satisfaction degree of nursing interventions in two groups were compared at six months after discharging. Results Both groups increased in quality of life after the interventions with observation group were higher than those of the control group (P<0. 05). The satisfaction degree of patients to nurses and doctors before and after interventions were 84. 62% and 82. 05% in control group, they were 97. 87% and 95. 74% in the observation group, which were higher than those of the control group (P<0. 05). Conclusions Personalized nursing intervention can improve the quality of life in patients with hypertensive cerebral hemorrhage, and improve patients'satisfaction to nursing care, so the measure is worthy of promotion.

Objective To study application effect of setting up full-time supervision nurse and shift in neurosurgery patients discharge guidance. Methods Between March and August in 2014, a total of 408 patients were discharged from Neurosurgery Department, Huai′an First People′s Hospital, Nanjing Medical University, who were divided into control group ( 204 cases ) and observation group ( 204 cases ) according to the first treatment ward. Patients in control group received conventional discharge guidance, whereas observation group had an extra full-time supervision on discharge guidance. Full-time supervision personnel should visit the patients discharged that day in the ward, inform the patient or family invoicing process,attention about related disease, medication and dietafter discharge. Meanwhile, full-time supervision personnel should get to know the service attitude of nurses, nursing technology andwhether relevant health education had been grasped during the patients′ hospitalization, based which the full-time supervision personnel provided a targeted discharge guidance. One month later, we compared the two groups for mastering discharge health education and patient or family members′nursing satisfaction. Results Patients in observation grasped discharge health education better than those in control group (93. 30% vs 73. 89%;χ2 =26. 1168,P<0. 05). Patients or their family members in observation group were more satisfied with nursing than that in control group(96. 91% vs 86. 78%;χ2 =11. 236,P<0. 05). Conclusions Full-time supervision nurse and shift used in neurosurgery patients discharge guidance,which build a bridge between patients and health care members, can not only improve the quality of nursing service, but also elevate hospital′social efficiency with a good reputation.

Objective To explore the application effects of 3M circular opening self-adhesive wound dressing on changing tracheostoma dressing.Methods A total of 56 tracheotomized patients from January to December 2014 were randomly admited to either observation group or control group.Different tracheostoma dressings were chosen by the two groups,the patients of observation group adopted 3M circular opening selfadhesive wound dressing and the patients of control group received sterile gauze.Skin situation after tracheotomy,incision infection,dressing frequency,cost and patient's satisfaction were evaluated.Results Three cases suffered from adverse skin reaction in observation group and 13 cases in the control group,with a statistically significant difference(x2 =8.750,P ＜ 0.05);the frequency of changing dressing was no significant difference between two groups (P ＞ 0.05),the observation group was once a day,while the control group was three times a day;the hospitalization costs were (59 242.16 ± 3 276) (￥) in the observation group,while it was (50 369.22 ±2 892) (￥) in the control group (t =6.420,P ＜ 0.05);patient's satisfaction in observation group was 96.43％,but 71.43％ in the control group (x2 =4.766,P ＜ 0.05).Conclusions 3M circular opening self-adhesive wound dressing for neurosurgery patients after tracheotomy can reduce complications,the rate of incisional infection,the hospitalization costs and improve patients' satisfaction and comfort,which also reduce the working load of clinical nurse.

Objective To explore the effect of physical restraint clinical pathway in the management of patients with restlessness in Neurosurgery Department.Methods A total of 84 restless patients with brain injury in neurosurgery of our hospital between October 201 4 and March 201 5 were included and randomly divided into observation group (n =42)and control group (n =42).The routine physical restraint was used in both groups, additionally,the physical restraint clinical pathway was performed in observation group.The nursing quality of physical restraint and satisfaction of patients or their families on the implementation of physical restraint were compared between two groups.Results The duration of first physical restraint in observation group was shorter than that in control group [(32.86 ±8.32)h vs.(35.48 ±8.1 3)h],but the difference between two groups has no statistical significance (t =1 .460,P >0.05).The score of nursing document quality of physical restraint in observation group was higher than that in control group [(98.1 2 ±1 4.1 1 )vs.(90.42 ±1 0.21 )],the difference between two groups was statistically significant (t =-2.865,P <0.05).The incidence of unplanned pulling out of ECG wires,transfusion lines and nasogastric tubes,and the complications of physical restraint such as peripheral circulation block were decreased.The differences between two groups were statistically significant (P <0.05).The satisfaction of patients or their families on the implementation of physical restraint in observation group was higher than that in control group (95.24% vs.78.57%),the difference between two groups was statistically significant (χ2 =5.126,P <0.05).Conclusions The application of physical restraint clinical pathway in the management of patients with restlessness in neurosurgery can increase the safety and effectiveness of physical restraint,reduce the occurrence of accidents,and improve the satisfaction of patients or their families.

Objective To explore the application effect of constrained patrol record form in safety management of agitated patients in neurosurgery. Methods A total of 84 cases of agitated patients with brain injury in the neurosurgery of our hospital from October 2014 to March 2015 were selected and divided into the control group and the observation group by random number table method, with 42 cases in each group. Patients in the control group were given routine constraint care management, while patients in the observation group were given safety management with the use of constrained patrol record form. The quality of constraint care was compared between two groups. Satisfaction of the nurses to the record form and the satisfaction of patients and their families to nursing were also compared between two groups. Results Before the application of constrained patrol record form, the number of complaints in the control group was 16, and the writing quality of nurses′constrained patrol record form was (91.42±10.21); after the application of constrained patrol record form, the number of complaints was 2 and the writing quality of record form was (97.12±14.11) (P<0.05). During the constrained period, the incidence rate and the number of cases of complications in the observation group were lower than that in the control group; and the nurses′s satisfaction to record form and patients′ satisfaction to nursing in the observation group were higher than that in the contro group ( P<0. 05 ) . Conclusions The application of constrained patrol record form in the safety management of agitated patients in neurosurgery, can effectively improve the nursing quality of agitated patients, reduce the occurrence of accidents, protect the safety of patients and improve the satisfaction of patients and their families.