Objective:To determine the motion detection uncertainty of the real-time CybeRay ? imaging system and patient intrafractional motion with thermoplastic mask-based immobilization. Methods:Real-time CybeRay ? imaging system was used for irradiation and treatment for head phantom and patients with brain tumors. All patients were immobilized with thermoplastic masks. Real-time imaging was delivered using kilovoltage projection images during radiotherapy. The detected patient motion data was collected from 5 head phantom measurements and 27 treatment fractions of 9 brain tumor patients admitted to Kaifeng Cancer Hospital. The accuracy and uncertainty of the motion monitoring system were determined. Results:The mean and standard deviation (SD) of the detected motion in the X, Y, and Z directions for phantom were (-0.02±0.41) mm, (-0.05±0.22) mm and (0.01±0.35) mm, respectively. The detected motion in the X, Y and Z directions for patents were (-0.13±0.48) mm, (-0.05±0.48) mm and (0.11±0.36) mm, respectively. After removing the motion detection uncertainty, the actual intrafractional motion of patients were (-0.11±0.25) mm, (0±0.43) mm and (0.10±0.08) mm in three directions, respectively. Conclusions:The uncertainty of real-time imaging-based motion monitoring system of CybeRay ? is less than 0.5 mm. It is feasible to apply thermoplastic masks for brain tumor patients in clinical practice, which can provide steady immobilization and limit the SD of patient intrafractional motion within 0.5 mm. Real-time imaging-based motion monitoring system of CybeRay ? is accurate for patient motion monitoring during frameless stereotactic radiosurgery/radiotherapy.

Objective:To analyze the clinical and pathological characteristics, treatment and prognosis of renal changes in patients with Kimura disease and improve the clinicians′ understanding on renal manifestations of Kimura disease.Methods:The clinical data of Kimura disease patients with definite diagnosis and detailed data in Peking Union Medical College Hospital from January 1980 to August 2020 were retrospectively analyzed. The patients were divided into renal impairment group and non-renal impairment group according to whether the kidney was involved or not and the related clinical data between the two groups were compared. The patients presenting with nephrotic syndrome were followed up.Results:There were 60 patients with Kimura disease confirmed by pathological diagnosis with 48 males. The median age was 33(3, 62) years old, and the median duration was 36(12, 111) months. There were 18 cases complicated with renal injury in 49 patients with complete routine urine and renal function examination and the main manifestations of renal injury were proteinuria and/or microscopic hematuria. There was no significant difference at age, sex and absolute value of eosinophils between the two groups (all P>0.05). Compared with the renal inpairment group, patients in non-renal inpairment group had longer course of disease, higher levels of hypersensitive C-reactive protein and erythrocyte sedimentation rate, and lower median values of total eosinophils and total IgE, but there was no statistically significant difference (all P>0.05). Among the patients with renal involvement, 6 patients met the diagnostic criteria for nephrotic syndrome, and 5 of them completed renal biopsies. The renal pathological diagnosis was membranous nephropathy in 2 cases and minimal change disease in 3 cases, and no interstitial eosinophil infiltration was found in renal biopsy tissues. These patients had a good response to glucocorticoids and/or immunosuppressive therapy, and achieved complete remission of nephrotic syndrome; at the same time, lymphadenopathy caused by Kimura disease could be well controlled. Conclusions:Kimura disease can combine with various renal lesions, and the pathology of nephrotic syndrome can be membranous nephropathy or minimal change nephropathy. After energetic treatment of glucocorticoids and/or immunosuppressive therapy, nephrotic syndrome can be completely relieved, and lymphadenopathy can be well controlled. The relationship between Kimura disease and renal disease needs further study.

Objective: To investigate the effect of gross motor skills on physical activity of children aged 3-6 years, and to provide basis for children’s health behavior intervention. Methods: A total of 332 young children in Shanghai Xiangyin kindergarten were selected as survey objects. The development of gross movements of young children was measured by TGMD-2. Caregivers reported children’s physical activity using the International Physical Activity Questionnaire (IPAQ-SF). Results: The development level of gross movements of children aged 3-6 in Shanghai was relatively low(54.76±13.86). The physical activity of children was mainly light-intensity physical activity, and the daily moderate-to-vigorous intensity physical activity time was (58.62±52.73) minutes every day. Locomotor skills had the greatest effect on children’s VPA (β=0.19) and TPA(β=0.12), with a contribution rate of 3.3% and 1.1%, respectively; Object control skills has the greatest effect on children’s MVPA (β=0.17) and MPA (β=0.12) had the largest effect(P<0.05), with contribution rates of 2.5% and 1.2%, respectively. The relationship between gross motor skills and physical activity had nothing to do with gender and age. Conclusion Children’s gross motor skills in the 3-6 year-old population should be improved and may be an effective way to promote physical activity.

Objective To investigate the clinical and pathological features of patients with a combination of Sjogren's syndrome (SS) and antineutrophil cytoplasmic antibody (ANCA) associated vasculitis with renal involvement.Methods By searching the Peking Union Medical College Hospital medical database and literature between January 1990 and June 2017,patients had a combination of SS and ANCA associated vasculitis with renal involvement were included.Data of clinical information,autoimmune antibodies,renal manifestations and renal pathology were retrieved and analyzed.Results Eighteen patients were enrolled:4 from our hospital and 14 from literature.SS was diagnosed no later than ANCA associated vasculitis in all the patients,among which 83.3％(15/18) of patients had extra-glandular and extra-renal organs involved.All the patients were tested positive for myeloperoxidase (MPO)-ANCA,and only two were protein 3 (PR3)-ANCA positive concurrently.The positivity rates of antinuclear antibody (ANA),rheumatoid factor (RF),anti-SSA antibody,and anti-SSB antibody were 83.3％(15/18),55.6％(10/18),77.8％(14/18),and 38.9％(7/18),respectively.The renal manifestations were characterized by renal insufficiency with a median serum creatinine of 174 μmol/L,hematuria,moderate proteinuria with a median 24 hour urine protein of 1.70 g,and necrotizing vasculitis with oligo-immune complex and varying degrees of interstitial damage in pathology.Conclusions A combination of Sjogren's syndrome and ANCA associated vasculitis with renal involvement is rare in clinical setting,and almost all of the patients are MPO-ANCA positive,with high probability of ANA positivity and extra-glandular involvement.Physicians should beware of ANCA associated glomerulonephritis in SS patients with inexplicable renal dysfunction and renal biopsy should be carried out in time.

Objective To evaluate the predictive factors and renal outcomes of idiopathic membranous nephropathy (IMN) in patients with type 2 diabetes (T2DM).Methods In this retrospective study,clinical data of 101 IMN patients with T2DM and 96 patients with diabetic nephropathy (DN) were consecutively collected.Logistic regression was used to assess potential clinical factors indicating IMN and COX regression was employed to analyze risks of IMN in developing to endstage renal disease (ESRD),as compared with that of DN,in patients with T2DM.Results In a multivariate model,age ≥55 years old,presence of nephrotic syndrome,estimated glomerular filtration rate (eGFR) ＞ 60 ml · min-1 · (1.73 m2)-1,duration of diabetes≤5 years and absence of diabetic retinopathy,were associated with IMN,as compared with DN,in patients with T2DM.In T2DM patients presented with nephrotic syndrome,age≥55 years old,eGFR ＞ 60 ml· min1· (1.73 m2)-1,duration of diabetes≤5 years and absence of diabetic retinopathy,were also associated with IMN,as compared with DN.Receiver operating characteristic curve (ROC) showed eGFR 65.5 ml · min-1 · (1.73 m2) 1 was an optimal cutoff in differentiating DN and IMN.DN was associated with 16.8 times as high risk of incident ESRD as compared with IMN in T2DM patients.Conclusions In patients with T2DM,age≥55 years,presence of nephrotic syndrome,early stage of CKD,duration of diabetes≤5 years and absence of retinopathy,may indicate IMN rather than DN.T2DM patients with IMN have much better renal prognosis as compared with DN.

Objective To analyze the clinic-pathological data and peritubular capillary (PTC) injuries of malignant nephrosclerosis (MN) patients and their correlations with the long term renal survival.Methods This was a retrospective cohort study of 52 MN patients in Peking Union Medical College Hospital from January 2003 to March 2012.Their clinical data and renal biopsy samples were carefully studied.CD34 staining was performed to evaluate the PTC area,using Benign nephrosclerosis (BN,n=17) patients and glomerular minimal lesions (GML,n=19) patients as controls.Multivariate Cox proportional hazard model was used to identify the potential independent risk factors for long term renal survival.Results Fifty-two MN patients were enrolled.The sex ratio of male to female was 12:1 and the average age was (34.0±8.2) years.The maximum blood pressure (SBP/DBP) was (230.4 ± 25.0)/(156.4 ± 20.6) mmHg,companied with significant loss of eGFR and proteinuria.Glomerular sclerosis index,tubular atrophy and interstitial fibrosis correlated with eGFR and proteinuria (P ＜ 0.05).After aggressive treatment,BP control rate improved significantly (76.9％ vs 3.7％,P ＜0.01),Scr [(376.4±263.8) μmol/L vs (486.8±375.7) μmol/L,Wilcoxon test,P＜ 0.01] and proteinuria [(1.10±0.70) g/24 h vs (2.04± 1.26) g/24 h,P ＜ 0.01,n=21] also improved.PTC area in MN patients was significantly lower than those in BN patients and GML patients,and it correlated well with Scr (r=-0.553,P=0.001) and eGFR (r=0.476,P=0.004).The median follow-up time was 74 months,the cumulative renal survival rate at 1 year,5 year and 10 year was 90％,64％ and 23％,respectively.Kaplan-Meier analysis showed that the patients with higher PTC area had longer renal survival time [(114.8± 12.4) months vs (63.0±8.3) months, x2=5.312,P ＜ 0.05].Univariate Cox proportional hazard model found that unsatisfied BP control,eGFR ＜ 30 ml · min-1 · (1.73 m2)-1 upon discharge,lower PTC area,severer tubular-interstitial damage and anemia were associated with poor renal outcome.Multivariate Cox model showed that unsatisfied BP control (RR=3.89,95％ CI 1.75-8.65,P=0.001),eGFR ＜ 30 ml · min-1 · (1.73 m2)-1 upon discharge (RR=4.27,95％ CI 1.40-13.09,P=0.011) were independent risk factors for long-term renal survival.Conclusions The correlation between PTC area and renal functions in MN patients are much better than that of classic vascular changes.Unsatisfied BP control and eGFR ＜ 30 ml · min-1 · (1.73 m2)-1 upon discharge are independent risk factors for long-term renal survival.

Objective To investigate the clinicopathological characteristics of IgAN patients with massive proteinuria,as well as their treatment response to glucocorticoids and long-term prognosis.Methods Clinical and pathological parameters were collected in patients diagnosed with IgA nephropathy in our hospital from Jan 2003 to Oct 2015.Patients were followed up for at least six months under the treatment with full dosage of glucocorticoids.Responses of patients with and without nephrotic syndrome were compared.Results A total of 156 patients were enrolled for the analysis (86 patients in the nephropathic proteinuria group,and 70 patients in the nephrotic syndrome group).Patients presented with nephrotic syndrome showed higher proportion of IgM deposition in renal slides.There exited no difference in treatment response to glucocorticoids between the two groups.Patients with full or partial remission showed a better prognosis by Kaplan-Meier analysis than no remission group (P ＜ 0.001).The ratio of segmental sclerosis was negatively correlated with treatment response to glucocorticoids by multiple linear regression (3 value=-0.330,P ＜ 0.001).Multivariate Cox regression model showed that glomerular density (HR=0.45,P=0.02) and eGFR (HR=0.95,P=0.001)were independent influential factors for renal survival.Conclusions Patients presented with nephrotic syndrome show higher proportion of IgM deposition in renal slides.Patients in remission after treatment with 6-month glucocorticoids present a better prognosis than no remission patients,and glomerular density as well as eGFR are independent influential factors for renal survival.

Objective To analyze serum amyloid protein A (SAA) subtype and amino acid mutation sequence of the renal biopsy specimens from patients with renal amyloidosis secondary to ankylosing spondylitis (AS) by laser microdissection combined with mass spectometry.Methods Kidney biopsy formalin-preserved paraffin-embedded (FFPE) specimen slices were stained by Congo red,the positive areas of Congo red staining were selected by microdissection,after trypsin hydrolysis and filtration,peptide samples were subjected to liquid chromatography tandem mass spectrometry.Analysis softwares were used to evaluate the results,and the patient's amino acid sequence of SAA protein was compared to mutant amino acid sequence reported by literature or deduced from mutant SAA gene to determine whether there was a variation.Results SAA1 and SAA2 proteins with high abundance were identified by mass spectrometry,serum amyloid P and apolipoprotein E were also detected.No variation of SAA1 and SAA2 protein was detected.Conclusions The SAA1 and SAA2 proteins in AA amyloidosis secondary to ASwere identified for the first time,which enriched the pathogenesis of amyloidosis secondary to AS and provided a new method for the accurate classification of AA amyloidosis.

Objective To assess the predictive value of Berden classification in ANCA associated glomerulonephritis.Methods Patients with confirmed ANCA associated glomerulonephritis were included,by retrieving the medical database in Peking Union Medical College Hospital from January 2000 to May 2015.Their detailed information during hospitalization and follow-up was recorded.The patients were divided into four categories based on Berden classification.The differences in clinical characters,renal function and response for treatment were compared.Results Among the 88 patients with ANCA-associated glomerulonephritis,19 (21.6％),21 (23.9％),32 (36.4％)and 16 (18.2％) patients were classified as focal,mixed,crescentic and sclerotic category.22 patients developed ESRD,and 19 patients died during follow up (1 patient developed ESRD before died).The mean estimated glomerular filtration rate (eGFR) at baseline was 68.04,25.45,30.04,15.16 ml·min-1·(1.73 m2)-1 (P ＜ 0.05) in focal,crescentic,mixed and sclerotic category,respectively.During follow-up period,focal category always had the best renal function,while sclerotic category had the worst renal function.Crescentic category and mixed category were similar and in the middle.Remission rate at 6m was 62.5％,73.7％,57.5％,30.8％(P ＞ 0.05).And crescentic category had the greatest improvement in eGFR at 6m.Conclusions Focal category had relatively preserved renal function and favorable renal outcome,while the sclerotic category had the worst renal outcome.Crescentic and mixed category had an intermediate outcome.We support the use of the Berden classification in predicting the renal prognosis of patients with ANCA associated glomerulonephritis.

Objectives To analyze the spectrum of renal diseases associated with monoclonal gammopathy and unrelated renal diseases.Methods Hospitalized patients in Peking Union Medical College Hospital who underwent renal biopsy between January,2013 and December,2015.They had monoclonal gammopathy on serum protein electrophoresis (SPE),serum immunofixation electrophoresis (IFE),urine IFE and/or serum free light chain (FLC).64 patients met the inclusion criteria and were classified as monoclonal gammopathy of renal significance (MGRS) (n=36),monoclonal gammopathy of undetermined significance (MGUS) (n=17) and hematologic malignancy (n=11).Results Renal lesions in MGRS subgroup included light chain amyloidosis (n=28,77.8％),light chain deposition disease (n=7,19.4％),and fibrillary glomerulopathy (n=l,2.8％).eGFR in light chain amyloidosis subgroup differed significantly,compared with light chain deposition disease [eGFR 93 ml· min-1 · (1.73m2)-1 vs 28 ml· min-1 · (1.73 m2) 1,P ＜ 0.01],as well as HTN incidence (35.7％ vs 100.0％,P ＜ 0.01).Renal diseases in MGUS subgroup included membranous nephropathy (n=10,58.8％),focal segmental glomerulosclerosis (n=3,17.6％),diabetic glomerulopathy (n=l,5.9％),Henoch-Schonlein purpura nephritis (n=l,5.9％),anti-glomerular basement membrane disease concurrent with membranous nephropathy (n=l,5.9％) and glomerulomegaly (n=l,5.9％).Various renal lesions related/unrelated to hematologic malignancy were seen in third subgroup,including light chain cast nephropathy (n=3,27.3％),tubulo-interstitial lesions (n=2,18.2％),light chain amyloidosis (n=1,9.1％),light chain deposition disease(n=1,9.1％),IgA nephropathy (n=1,9.1％),mesangial proliferative glomerulonephritis (n=l,9.1％),endocapillary proliferative glomerulonephritis (n=1,9.1％) and acute tubular necrosis (n=1,9.1％).Positive rates of SPE,serun IFE and urine IFE in MGRS subgroup were 40.6％,52.8％ and 69.4％,respectively.Positive rates of SPE,serum IFE and urine IFE in MGUS subgroup were 68.8％,100.0％ and 37.5％,respectively.Positive rates of SPE,serum IFE and urine IFE in hematologic malignancy subgroup were 54.5％,72.7％ and 81.8％ respectively.MGRS and MGUS subgroups differed significantly in positive rate of serum IFE (P ＜ 0.001).Abnormal rates of serum FLC ratio in above three subgroups were 83.3％,17.6％ and 90.9％,respectively,with that in MGUS group being significantly lower than the rates in other two groups (P ＜ 0.001,respectively).Conclusions The significance of monoclonal gammopathy in patients with renal disease should be evaluated by other clinical data,as well as renal pathology.