Objective To study the characteristics of the serum free light chain ( sFLC ) and cytokines levels and the effects of cyclophosphamide (CTX) on patients with primary Sj(0)gren's syndrome (pSS) complicated with amyloidosis.Methods Nine patients of pSS with amyloidosis who were admitted to Department of Rheumatology of the hospital from Jan 2005 to Mar 2011 were selected.The serum levels of sFLC and cytokines TNF-α,TGF-β1,IL-4 were measured before and 3,6,12 months after the treatment respectively.Meanwhile,Twelve patients with pSS without complications were selected as controls.Repeated measures analysis of variance was used for data analysis.Results The sFLC in serum of pSS patients with amyloidosis was λ type,the κ type was not found.The level of serum sFLC after 12 months treatment [(22±42) mg/L]was significantly lower than before treatment [(180±15) mg/L].The levels of TNF-α,TGF-β1,IL-4 after 12months treatment [(24.0±1A),(58±5),(38.8±3.2) ng/L,respectively] were significantly lower than before treatment [(31.1±L0),(65±5),(61.2±3.8) ng/L,respectively].Conclusion The levels of serum sFLC and eytokines are abnormally increased in patients of pSS with amyloidosis.After treatment,the levels of cytokines are decreased initially which indicates that these cytokines may play a key role in the pathogenesis of pSS.CTX therapy may lead to better efficacy and more favorable prognosis.

The present paper consults the concept of clinical competence, cultivation actuality, examination and assessment system of master of nursing specialist (MNS) at home and aboard based on literature analysis.Currently there is neither unified concept of master nursing specialist clinical competence, nor a unique clinical ability training mode of MNS.This paper mainly expounds the differences of the nursing professional master degree graduate students clinical ability evaluation system at home and abroad in the assessment methods, assessment tools, assessment agencies and assessment index, pointing out that our country should make a unified concept of the clinical competence.We should develop a flexible, diverse clinical competency assessment method, establish specialized evaluation agencies which are independent of the government, and implement 3 level quality monitoring system-graduate school of the university, clinical college graduate school and nursing department of, to ensure the training quality.

Objective To investigate the development and the risk factor of hip dislocation about children with cerebral palsy. Methods 90 children with cerebral palsy were divided with Chinese version of Gross Motor Function Classification System （GMFCS） and type of cerebral palsy. Their femoral head migration percentage was measured. Results The quadriplegia children were in the biggest risk of hip dislocation, hemiplegia children were the minimum; GMFCS Ⅰ children were in the lowest risk of hip dislocation, GMFCS Ⅴ children were the highest. Conclusion The abnormal development of the hip is correlative with the type of cerebral palsy and motor function in spastic cerebral palsy children.

Objective To investigate the effects of bone marrow mesenchymal stromal cells (BMSCs) transplantation after mannitol pretreatment on behavioral performance and synaptophysin expression in the CA3 region in hippocampus of vascular dementia (VD) rats.Methods The BMSCs of rats were isolated and purified by the whole bone marrow adherence method.The rats were subjected for permanent ligation of bilateral common carotid arteries at an interval of 3 days for each carotid artery.At the same time,Sham group was set in parallel.Four weeks after modeling,the VD rats were divided randomly into five groups:(1) VD control group; (2) culture media group; (3) mannitol group; (4) BMSCs group;(5) mannitol with BMSCs group.Morris water maze performance and synaptophysin expression in the CA3 region in hippocampus were observed at 4 weeks after transplantation.Results The morris water maze performance significantly improved in mannitol with BMSCs group when compared with BMSCs group,VD control group,culture media group,mannitol group.Moreover,the escape latency of fifth day decreased significantly ((9.3 ±2.9),(14.1 ±3.5),(23.5 ±4.4),(22.8 ±4.4),(23.2 ±2.8) s,F =43.900,P =0.000)),and the platform quadrant residence time increased significantly ((40.8 ± 6.3),(34.9 ±5.8),(26.4±4.8),(27.4 ±7.0),(28.5 ±6.2) s,F=13.000,P=0.000)).The synaptophysin expressions of the hippocampal CA3 region were significantly increased in the mannitol with BMSCs group (39 624 ± 7798) when compared with BMSCs group,VD control group,culture media group,mannitol group (27060 ±4668,18 294 ±6446,19 956 ±4244,18 946 ±4953,F =39.206,P =0.000).Conclusions Intravenous BMSCs transplantation after mannitol pretreatment improves the behavioral performance of VD rats and facilitates the synaptophysin expression of hippocampal CA3 region in VD rats than BMSCs transplantation alone.Mannitol pretreatment can amplify the therapeutic effect of intravenous BMSCs transplantation in VD rats.

ObjectiveTo assess the efficacy of low-dose intravenous cyclophosphmide (IV CTX) in treatmerit of old patients with systemic lupus erythematosus(SLE).MethodsTwenty-three old patients with newly diagnosed, untreated SLE were included.Patients received 3 times fortnightly IV CTX pulses at a fixed dose of 400mg followed by 3 monthly pulses.All patients were orally administered 0.8mg/kg of prednisone.The changes of the indexes were observed before and after 12 weeks treatment including the scores of SLE disease activity index(SLEDAI) and the levels of C3, C4 ,24-hour urinary protein and anti-dsDNA antibody.ResultsAmong 23 patients, 19 were followed up to 24 weeks, SLEDAI 4 weeks after treatment were all significantly lower than those before the treatment (P ＜0.01).At week 8, SLEDAI continued to decrease(P ＜0.05).At week 4,the level of urine protein, the levels of complements C3 and C4 and ds-DNA decreased significantly compared with that before the treatment (P ＜0.05).ConclusionIntensive low-dose CTX plus prednisone was effective in newly diagnosed, untreated old patients with SLE, and could reduce adverse effect.

Objective To determine the reliability of hand-held electronic dynamometer (HHD) tests for lower limb muscle strength measurement in children with spastic cerebral palsy (CP). Methods Twenty-eight children ( 15 boys and 13 girls; mean age 5 years 8 months) with different types of CP (2 with spastic quadriplegia,8 spastic diplegia, 6 hemiplegia, 1 triplegia and 1 monoplegia) , and at different functional levels ( 19 graded at gross motor function classification system level Ⅰ, 6 level Ⅱ, 2 level Ⅲ and 1 level Ⅳ ) were recruited from the Rehabilitation Center of the Children's Hospital of Fudan University. Standardized HHD protocols were used to measure the strength of their hip, knee and ankle muscles. The HHD test was performed by the same examiner twice with an interval of 10 min in between. The HDD test was also performed with 15 randomly selected CP children by 2 examiners with an interval of 10 min in between. The test-retest and inter-rater reliabilities of the HDD readings were determined by calculating the intra- and inter-class correlation coefficients. Results The HHD measurements showed fine testretest reliability ( ICC = 0.74-0.97 ) and inter-rater reliability ( ICC = 0.63-0.97 ) in measuring lower-limb muscle strength of children with spastic CP, with the highest test-retest reliability for the hip flexion, foot plantar flexion and knee extension muscle groups. The highest inter-rater reliability was achieved with the hip flexion and foot dorsiflexion muscle groups. Conclusions Standardized HHD testing of lower-limb muscle strength in children with spastic CP shows fine test-retest and inter-rater reliability. The HHD can reliably assess the lower-limb muscle strength of children with spastic CP.

Objective To analyze the content validity of Gross Motor Function Measure (GMFM) for cerebral palsy. Methods Concept contained in each item of GMFM was determined by 3 rehabilitation physicians or therapists who evaluated with GMFM for more than 2 years. All the concepts extracted were discussed in a meeting. The terms finalized were linked to the categories of International Classification of Functioning, Disability and Health Child and Youth version (ICF-CY) by 2 rehabilitation physicians who are familiar with the terms of ICF-CY according to the linking rules after discussion. Third party would intervene informally if there was a disagreement. The distribution of the ICF-CY categories involved were analyzed. Results 88 items from the GMFM linked to 27 categorise of 3rd level within 12 categories of 2nd level, belong to b7 Neuromusculoskeletal and Movement-related Functions and d4 Activities. 86 items linked to d4x, and 47 of then linked to b7x too. 2 items linked to b7x only. 16 items linked to the categories added to ICF-CY 16 times, involved 3 3rd level categories in a 2nd level category. Conclusion GMFM mainly focused on the activities in the framework of ICF-CY, especially the activities of changing and maintaining functional as well as the body posture and walking or moving. For the body function, the GMFM mainly focus on the support from arms.

Objective To investigate the health-related quality of life and its relative factors in children with leukemia during maintenance treatment period. Therefore, the basis for effective individualization intervention can be provided. Methods A total of 224 leukemia children in maintenance treatment were collected in Affiliated Children′s Hospital of Chongqing Medical University from October 2015 to January 2016 by convenience sampling methods, questionnaire was used to assess the physical status, emotional status, social status, school status of the children. Results Single factor and generalized linear regression analysis were used to conclude that patient′s age (F=5.841-36.343, all P<0.01), parenting pattern (F=4.006-4.288, all P<0.05), family economic status (F=3.277-15.865, P<0.05 or 0.01) and the caregivers′information mastery level household location (F=2.044-2.661, P<0.05 or 0.01) had significant influence on the quality of life of children with leukemia in maintenance treatment. Conclusions The health-related quality of life and its relative factors in children with leukemia during maintenance treatment period should be analysis comprehensively. At the same time, take intervention should be taken from physiology, psychology, society, and school, to improve the social adaptability of children with leukemia and help them return to society as soon as possible.

Objective To explore the effect of the gene silencing of phosphatidic acid-preferring phospholipase A1 (PA-PLA1) on insulin secretion in mouse insulin-secreting cell line MIN6. Methods The siRNA expression vector of mouse PA-PLA1 gene targeting was constructed using mouse PA-PLA1 mRNA sequence available in GenBank, and MIN6 cells were transfected with the vector. Fluorescence quantitative PCR and Western-blotwere applied to screen efficient RNAi-vector. After transfection with obtained efficient RNAi-vectors for 48 hours, glucose-stimulated insulin secretion experiments were conducted, and the changes of insulin secretion were examined. Results Four siRNA expression vectors of mouse PA-PLA1 gene targeting were confirmed to be successfully constructed by the analyses of enzyme cleavage and sequencing. The results of fluorescence quantitative PCR and Western blot analyses indicated that the siRNA expression vectorpGPU6-PA-PLA1-1885was the most effective RNAi-vector in the four vectors. The expression levels of the PA-PLA1 mRNA and protein of the MIN6 cells transfectedwith pGPU6-PA-PLA1-1885 decreased to 46.3% and 33.9% of that of the control, respectively, and meanwhile the insulin secretion levels of the cells decreased to 65.0% of that of the control (P < 0.05). Conclusion The gene silencing of phosphatidic acid-preferring phospholipase A1 might decrease insulin secretion in MIN6 cells.

Objective To explore the characteristics of gross motor development in 1～6-year-old children with cerebral palsy at different levels. Methods 708 children (487 males and 221 females, age range: 1～6 years, from 6 rehabilitation centers in Shanghai) with cerebral palsy (CP) were assessed with Chinese version of Gross Motor Function Classification System （GMFCS） and Gross Motor Function Measure (GMFM). Distributions of GMFM scores at different GMFCS levels in children with cerebral palsy were analyzed. Results The GMFM-66 scores increased most in children with GMFCS Level Ⅰ, and more than 75% of them would be greater than 67 points in GMFM-66 score after the age of 48～50 months. The children with GMFCS Level Ⅱ～Ⅳ appeared similar increasing range of GMFM-66 scores in 1～6 years old. Less than 25% of the children at GMFCS Level Ⅱ would be greater than 67 points in GMFM-66 score before the age of 6 years, more than 50% of those at GMFCS Level Ⅲ would be less than 56 points, more than 75% of those at GMFCS Level Ⅳ couldn't exceed 46 points （except groups of 54～56 months and 66～68 months）. Compared with the children with other GMFCS levels, the GMFM-66 scores were always at very low level in children with GMFCS Level V, and trended to decrease with time after 5 years old. Conclusion The characteristics of gross motor development are different in children with cerebral palsy at different GMFCS levels.