Objective To report four patients with secondary α-dystroglycanopathy caused by guanosine diphosphate-mannose pyrophosphorylase-B ( GMPPB ) gene mutations and review the literature aiming to analyze the clinical manifestations , muscle image , molecular pathology and genetic characteristics of the disease.Methods The medical history , physical examination , electromyography and other clinical data of four patients with secondary α-dystroglycanopathy from two families were collected and retrospectively reviewed from 2009 to 2017.Case 1 ( proband of pedigree 1) and case 2 ( proband of pedigree 2) were then further analyzed with muscle imaging , muscle pathology and targeted next generation gene sequencing (NGS).Results Four patients came from two families (three from the same pedigree), two males and two females, with an onset age of 17 -18 years.All four cases presented as limb-girdle muscular dystrophy (LGMD) overlapping with congenital myasthenic syndrome (CMS) characterized by evident proximal limb weakness in early adulthood and fluctuating muscle weakness .They all had delayed motor milestone and did not perform well in physical education since childhood . Serum creatine kinase was elevated markedly (1877-5275 U/L).Myogenic changes on electromyography and marked attenuation on three Hz repetitive nerve stimulation were observed in all patients .Muscle MRI showed prominent involvement of bilateral hamstrings in case 1 and case 2.Muscular dystrophic patterns were demonstrated on muscle biopsies . Targeted NGS revealed two compound heterozygous missense mutations in GMPPB for each case .Case 1 carried c.860G>T (p.R287L)/c851T>C (p.V284L).Case 2 and his two affected sisters (case 3 and case 4) carried c.1097A >G ( p.N366S)/c.589G >T ( p.V197F) .All of these mutations were novel variants and pedigree analysis suggested that the two mutations were from parents .Compared with normal controls, immunohistochemistry and Western blotting showed significantly decreased expression of α-dystroglycan in the muscle tissue from case 1 and case 2.The myasthenic symptoms of all four patients were improved to varying degrees after treatment with pyridostigmine bromide . Conclusions Mutations in GMPPB can lead to dysfunction both in muscle and in neuromuscular transmission causing overlapping between LGMD and CMS phenotypes . Cholinesterase inhibitors can partly improve the symptoms of myasthenia in such patients .

Objective To analyze the patients′ psychological resilience level of postpartum pelvic floor dysfunction and to explore the influencing factors, in order to provide the scientific basis for patients′psychological intervention. Methods A cross-sectional survey was conducted on 208 patients of postpartum pelvic floor dysfunction by using the Connor-Davidson Resilience scale(CD-RISC), Positive and Negative Affective scales(PANAS) and Medical Coping Modes questionnaire(MCMQ, which includes "confrontation","acceptance-resignation"and"avoidance"). Results The score of psychological resilience of 208 patients was (53.64 ± 12.29)points, differences in age, family monthly income, living locations, delivery methods and delivery times reached a statistically significant level(F=15.287,5.731,t=-2.571,-15.911,-2.922,all P<0.05). Patients′psychological resilience level had significant positive correlation with positive emotions(P<0.01)and"confrontation",and it had negative correlation with"acceptance-resignation"(P<0.01).Age,living locations, delivery methods, positive emotions and "acceptance- resignation" are the forecast factors of psychological resilience, which can explain 69.6% of total variation. Conclusions Patients of postpartum pelvic floor dysfunction have low levels of psychological resilience, which associated with positive emotions and medical coping modes. Therefore, nursing staffs should pay attentions to the patients′ psychological status, develop individualized psychological guidelines,implement psychological intervention and health education,in order to improve the level of patients′resilience and improve their living quality.

Objective To systematically evaluate the effectiveness of different nursing interventions for female overactive bladder, and to provide evidence- based evidence for nursing intervention for overactive bladder in women. Methods PubMed, Cochrane Library, Embase, ScienceDirect and CNKI,Wanfang database and CBM comprehensively by computer and included in the literature of nursing intervention for female overactive bladder patients. Results Four randomized controlled trials were included, a total of 426 participants were in, the intervention group was 214 participants, control group was 212 participants, the results of the study showed that pelvic floor muscle training,health education,psychological nursing intervention can improve patients with overactive bladder symptoms and enhance the quality of life. Conclusion Nursing intervention for women with overactive bladder is an effective way to control symptoms such as frequent micturition and urgency,improve bladder overactivity symptoms and enhance quality of life.

Fatigue is commonly experienced in patients with advanced kidney disease and associated with poor outcomes. The purpose of this review was to discuss the prevalence, assessment methods and contributing factors of fatigue in patients with end-stage renal disease (ESRD), and also examined possible interventions to improve fatigue and concludes by defining some future research directions.

Cardiovascular disease has become a major health problem facing human beings, and it is also the leading cause of death world wide.Now Cardiovascular disease risk factors have been found and many new complex risk factors (such as sleep, atmospheric pollution, social psychological factors, cortisol, C-reactiveprotein, homocysteine, age at menarche etc.) are put forward, which let people have a deeper understanding. In this paper, the recent advances in cardiovascular disease risk factors and influence mechanism are reviewed, so as to provide basis and support for cardiovascular disease in prevention, treatment and nursing.

Subjective well-being,a comprehensive psychological index for evaluating the quality of life,is closely related to the physical and mental health of the elderly.At present,the mainly Methods for measuring subjective well-being in the elderly is scale. Subjective well-being assessment scales include generic scales and specific scales.Domestic elderly subjective well-being scales commonly imported from abroad.Specific scales for elderly are less in mainland China.This article will review the current research status for subjective well-being assessment scales in the elderly, in order to strengthen the localization research of specific scales and further provides reference for the clinical research of this topic.

Objective To investigate the levels of cancer related fatigue (CRF) and the correlations between CRF and serum inflammatory factors and hypothalamus-pituitary-adrenal (HPA) axis in patients with gastrointestinal cancer.Methods The CRF level was assessed by brief fatigue inventory (BFI).The level of C-reactive protein (CRP) was measured by immunoturbidimetry, and the level of cortisol was measured by electrochemiluminesence.The levels of interleukin (IL)-6, IL-1β, tumor necrosis factor-α (TNF-α), adrenocorticotropic hormone (ACTH) and norepinephrine (NE) were measured by enzyme-linked immunosorbent assay (ELISA).Results The average total score of CRF was 3.15±1.93, and the degree was mild to moderate, which was positively correlated with the CRP (r=0.321, P=0.000), TNF-α (r=0.265, P=0.000), NE (r=0.174, P=0.015) and ACTH (r=0.257, P=0.000), but was not correlated with the cortisol (r=0.033, P=0.652).Eastern Cooperative Oncology Group (ECOG) score (t=8.081, P=0.000), education (t=-4.244, P=0.000), treatment (t=4.563, P=0.000), time from diagnosis to sampling (t=3.453, P=0.001) and CRP (t=2.837, P=0.006) were important factors of CRF.Conclusion The CRF status is common in gastrointestinal cancer patients.The CRF is correlated with the NE and ACTH of HPA axis.Medical staff should pay attention to the inflammatory factors and hormone levels to improve the fatigue status and the quality of patients.

The cardiovascular disease has become one of the important chronic health problems for humans. At present, the research on the traditional risk factors of cardiovascular disease has been studied from the relevance to the mechanism. In this paper, the recent progress of the traditional risk factors of CVD and the impact mechanism are reviewed in order to provide a basis for the prevention, treatment and nursing of cardiovascular diseases.

Objective: To investigate the feasibility of contrast-enhanced computer tomography (CT) texture analysis in predicting early recurrence after transarterial chemoembolization (TACE) in patients with liver cancer. Methods: A retrospective analysis was performed for 47 patients with liver cancer confirmed by liver biopsy and digital subtraction angiography who underwent upper abdominal contrast-enhanced CT scan before TACE, and according to the presence or absence of focal recurrence within half a year, these patients were divided into early recurrence (ER) group and non-early recurrence (NER) group. The texture analysis was used to delineate tumor boundary layer by layer on the axial contrast-enhanced CT image before liver cancer surgery, and related parameters of tumor heterogeneity, including entropy, mean, non-uniformity, skewness, and kurtosis, were obtained. The independent samples t-test was used for comparison of texture parameters between the two groups. The receiver operating characteristic (ROC) curve was used for the analysis of entropy, mean, and non-uniformity, and the area under the ROC curve (ROC), optical cut-off value, sensitivity, and specificity were calculated to evaluate the efficiency of texture analysis in predicting early focal recurrence after TACE. Results: There were 20 patients in the ER group and 27 in the NER group. The ER group had a maximum major axis length of 88.2±36.3 mm and a maximum minor axis length of 41.4±21.4 mm, and the NER group had a maximum major axis length of 66.9±30.2 mm and a maximum minor axis length of 29.3±19.8 mm; the ER group had significantly higher maximum major and minor axis lengths than the NER group (t = 4.89 and 4.62, P < 0.001). The ER group had significantly higher entropy and non-uniformity values than the NER group, and there were no significant differences in skewness and kurtosis between the two groups. Entropy, non-uniformity, and mean had high efficiency in predicting early recurrence after TACE, and the optimal cut-off value of entropy was 4.135. Conclusion Volumetric texture analysis of contrast-enhanced CT images before liver cancer surgery has a high value in predicting early recurrence after TACE.

Aiming at status of training the rural order directed free medical educational talents, we should improve the understanding of training the free medical students, perfect the relevant management system, strengthen medical ethics education of students, pay attention to humanistic care of students,strengthen the continuing education of students, standardize the access system of students, reform the medical distribution system, to ensure the quality of training free medical students.