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Objective:To evaluate the efficacy and safety of rituximab in the treatment of adult primary focal segmental glomerulosclerosis (FSGS).Methods:Adult FSGS patients treated with rituximab in the First Affiliated Hospital of Zhejiang University College of Medicine were retrospectively enrolled. One or two doses of rituximab (375 mg/m 2) were used aiming to achieve B cell depletion (defined as<5 B cells per microliter in peripheral blood) and the interval between the two doses was 2 weeks. The evaluated major outcomes were remission and relapse of nephropathy, and the secondary outcome measures were adverse events and renal outcomes. Results:A total of 14 patients (9 males) were enrolled, among whom 7 cases were steroid-dependent nephrotic syndrome (SDNS) or frequently relapsing nephrotic syndrome (FRNS), 6 cases were steroid-resistant nephrotic syndrome (SRNS) and one patient was new onset FSGS with contraindication to steroid. After treatment with rituximab, 7 patients with SDNS/FRNS achieved complete remission. At 6 months, the daily oral steroid dose reduced significantly compared with the baseline [(33.3±5.2) mg/d vs (6.7±6.6) mg/d, P<0.01]; while one patient still received tacrolimus 1.0 mg/d, the other 6 patients stopped using immunosuppressants; and the total number of relapse/total follow-up months decreased from 0.257 times/month to 0.058 times/month after the use of rituximab. For the other 6 SRNS patients and one patient with contraindication to steroid, three SRNS patients achieved partial remission and one patient with contraindication to steroid achieved complete remission at 34.50(20.25, 95.25) days after use of rituximab, and the other 3 SRNS patients failed to achieve remission, of whom one patient developed end stage renal disease at 23 months. Conclusions:Rituximab may reduce the risk of relapse and help steroid or immunosuppressant-tapering in adult steroid-dependent/frequently relapsing idiopathic FSGS. However, it is not effective in SRNS patients.
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Objective@#To analyze the distribution of glomerular immunofluorescence IgG4 subtypes in primary membranous nephropathy, and to explore the relationship between IgG4 deposit intensity and renal pathology, clinical manifestations and prognosis.@*Methods@#All the patients of biopsy-proven primary membranous nephropathy with IgG staining and at least one IgG subtype staining 1+ or higher on capillary loops from September 2015 to April 2017 were retrospectively enrolled. The distribution of IgG4 deposits were analyzed, and the relationship between IgG4 positive intensity and clinical manifestations, pathological indexes and clinical remission was investigated.@*Results@#A total of 250 cases were enrolled, including 157 males (62.8%) and 93 females (37.2%), and age was (54.4 ± 14.6) years. There were 40 patients in IgG4-negative group, and 210 patients in IgG4-positive group. The IgG4-positive group was divided into subgroups as 114 cases of the mild positive subgroup (1+) and 62 cases of the moderate positive subgroup (2+), and 34 cases of the strong positive subgroup (3+, 4+). The IgG4-positive group had higher 24-hour urine protein and higher positive rate of phospholipase A2 receptor staining than those in the negative group (both P<0.05), while the strong positive subgroup had lower serum albumin and higher IgG1 staining than those in the mild positive subgroup (both P<0.05). There was no difference in the ratio of glomerular sclerosis, tubular atrophy, IgG2, IgG3 or other immunofluorescence between the groups. After a median follow-up of 180(122, 209) days, 32 individuals were lost to follow-up. Among the rest 218 patients, 45 patients (20.6%) got complete remission, 104 patients (47.7%) got partial remission, and 69 patients (31.7%) showed no response. For no response as the outcome event, multivariate Cox regression analysis showed that higher IgG4 staining intensity (HR=1.371, 95%CI 1.068-1.759, P=0.013), male (HR=1.818, 95%CI 1.028-3.214, P=0.040), higher 24-hour urine protein level (HR=1.108, 95%CI 1.003-1.225, P=0.043) were independent risk factors for disease remission.@*Conclusions@#The glomerular IgG4 positivity and intensity are related to the severity of primary membranous nephropathy. The glomerular IgG4 deposit degree may be an effective prognostic marker for the treatment response of primary membranous nephropathy.
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Objective:To analyze the distribution of glomerular immunofluorescence IgG4 subtypes in primary membranous nephropathy, and to explore the relationship between IgG4 deposit intensity and renal pathology, clinical manifestations and prognosis.Methods:All the patients of biopsy-proven primary membranous nephropathy with IgG staining and at least one IgG subtype staining 1+ or higher on capillary loops from September 2015 to April 2017 were retrospectively enrolled. The distribution of IgG4 deposits were analyzed, and the relationship between IgG4 positive intensity and clinical manifestations, pathological indexes and clinical remission was investigated.Results:A total of 250 cases were enrolled, including 157 males (62.8%) and 93 females (37.2%), and age was (54.4 ± 14.6) years. There were 40 patients in IgG4-negative group, and 210 patients in IgG4-positive group. The IgG4-positive group was divided into subgroups as 114 cases of the mild positive subgroup (1+) and 62 cases of the moderate positive subgroup (2+), and 34 cases of the strong positive subgroup (3+, 4+). The IgG4-positive group had higher 24-hour urine protein and higher positive rate of phospholipase A2 receptor staining than those in the negative group (both P<0.05), while the strong positive subgroup had lower serum albumin and higher IgG1 staining than those in the mild positive subgroup (both P<0.05). There was no difference in the ratio of glomerular sclerosis, tubular atrophy, IgG2, IgG3 or other immunofluorescence between the groups. After a median follow-up of 180(122, 209) days, 32 individuals were lost to follow-up. Among the rest 218 patients, 45 patients (20.6%) got complete remission, 104 patients (47.7%) got partial remission, and 69 patients (31.7%) showed no response. For no response as the outcome event, multivariate Cox regression analysis showed that higher IgG4 staining intensity ( HR=1.371, 95% CI 1.068-1.759, P=0.013), male ( HR=1.818, 95% CI 1.028-3.214, P=0.040), higher 24-hour urine protein level ( HR=1.108, 95% CI 1.003-1.225, P=0.043) were independent risk factors for disease remission. Conclusions:The glomerular IgG4 positivity and intensity are related to the severity of primary membranous nephropathy. The glomerular IgG4 deposit degree may be an effective prognostic marker for the treatment response of primary membranous nephropathy.
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Objective To analyze the pathological characteristics and prognostic factors of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).Methods A retrospective analysis of AAV patients with renal biopsy results admitted to Kidney Disease Center of the First Affiliated Hospital from January 2004 to February 2017 was performed.The patients were divided into 4 types according to Berden classification,and their clinical,pathological characteristics and prognosis were compared.The survival curves of each type of patients were plotted by Kaplan-Meier method,and the difference of survival curves was compared using Log-rank test.With entering the maintenance dialysis as the endpoint,Cox regression was used to analyze the prognostic factors.Results A total of 175 patients with AAV,including 59 cases (33.7％) of focal type,39 cases (22.3％) of crescent type,32 cases (18.3％) of sclerosis type,45 cases (25.7％) of mixed type.The basal serum creatinine levels in crescent type group and sclerosis type group were significantly higher than those in the focal type group or mixed type group (all P ＜ 0.05),and loop necrosis rate in sclerosis type group was significantly lower than chat in the focal type group or crescent type group (both P ＜ 0.05).The median follow-up period was 11.8 (0.5-86.7) months.The event-free survival rates were 83.1％,77.8％,64.1％ and 50.0％ in the focal type,mixed type,crescent type and sclerotic type groups (Log-rank x2=11.537,P=0.009).Cox regression analysis showed higher parathyroid hormone (HR=1.013,95％ CI 1.007-1.019,P ＜ 0.001),glomerular sclerosis ≥50％ (HR=10.532,95％CI 2.903-38.203,P ＜ 0.001) were independent risk factors for AAV patients entering maintenance dialysis,and higher estimated glomerular filtration rate (HR=0.943,95％ CI 0.896-0.993,P=0.025) was protective factor.Conclusion The prognosis of AAV renal damage is worsened according to focal,mixed,crescent and sclerosis types.Lower estimated glomerular filtration rate,higher parathyroid hormone and glomerular sclerosis ≥ 50％ are independent risk factors for AAV patients entering maintenance dialysis.
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Objective To investigate the clinical manifestations,renal pathology and prognosis of antineutrophil cytoplasmic antibody-associated small-vessel vasculitis (AAV) accompanied with renal glomerular IgA deposition.Methods A retrospective analysis was performed at the First Affiliated Hospital of Zhejiang University College of Medicine.Patients diagnosed with AAV associated renal injury by renal biopsy from February 2004 to February 2017 were enrolled.Patients with antiglomerular basement membrane antibody-mediated nephritis,systemic lupus erythematosus nephritis,Henoch Schonlein purpura nephritis,hepatitis B virus associated nephritis and other known etiology were excluded.According to immunofluorescence examination,the patients were divided into IgA deposition group and pauci-immune complex deposition group.The differences in clinical manifestation,pathological features and prognosis were compared between groups.Results A total of 150 AAV cases were included,among which 25 cases were with IgA deposition and 125 cases with pauci-immune complex deposition.The level of serum albumin in IgA deposition group was higher than that in pauci-immune complex deposition group [(35.0±6.2) g/L vs (32.6±5.3) g/L,P=0.049],but the titer of MPO-ANCA was lower [24.8(10.4,71.8) U/ml vs 63.0(21.9,100.0) U/ml,P=0.044] in IgA deposition group.There was no significant difference between two groups in other laboratory indexes and renal pathological findings.The median follow-up time was 15.2 months in IgA deposition group and 8.9 months in pauci immune complex deposition group.During the follow-up there were 8 patients (32.0％) in IgA deposition group and 29 patients (23.2％) in pauci immune complex deposition group on maintaining dialysis;2 patients (8.0％) in IgA deposition group and 7 patients (5.6％) in pauci immune complex deposition group died.There was no significant difference between two groups in patients' outcomes.Conclusions AAV patients with glomerular IgA deposition and AAV patients with typical glomerular immunoglobulin complex deposition are similar as regards clinical appearance and prognosis.
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Objective To study the optimum combination of orientation parameter of total hip prosthesis and acetabular safe zone on condition that the range of motion for activities of daily living (ADL) is fulfilled.Methods A three-dimensional generic parametric and visually kinematic simulation module of THA was developed.Range of motion ( ROM ) of hip flexion ≥ 110°,internal-rotation ≥30° at 90° flexion,extension ≥30° and external rotation ≥40° were defined as the normal criteria for ADL.ROM of hip flexion ≥ 120°,internal-rotation ≥45° at 90° flexion,extension ≥30° and external rotation ≥40° were as the severe criteria.The ranges of changes in general ratios (GRs) of head-neck,femoral neck antevemion ( FNA ),operative inclination (OI) and anteversion (OA) of acetabulur components were 2.0-2.92,0°-30°,10°-60°,and 0°-70° respectively.Within the limits of the upper two activity criteria,the synchronous OA of acetabulur components was calculated with every 5°change in OI of the cup,and the collodiaphyseal angle ( CDD ) was set as 135°.The safe-zone of combination of acetabulur operative anteversion (OA) and inclination (OI) was defined as the area that fulfilled the two mentioned criteria of ROM without cup-neck impingement.All parameters were analyzed by using SAS 6.12 software.Results The safe zone of acetabdar angle rose with the increase of GRs of head-neck and the safe zone of severe criteria was smaller than that of normal criteria.When the CDD angle was 135°,the sum of average aeetabular OA and acetahular OI plus 0.816 times of the FNA equaled to 84.76° innormal criteria; and the sum of average acetabular OA and acetabular OI plus 0.873 times of the FNA equaled to 92.04° in severe criteria.Conclusions A high GR of head-neck greatly increases the size of safe-zone of acetabular angle.The higher demand of ROM of hip joint requires the smaller safe zone of acetabular angle,as can be corrected by increasing the GR of head-neck.The optimum combination between the sum of average acetabular OA and acetabular OI ( Y) plus FA (X)in the normal criteria and severe criteria can be estimated by using the following formulae:Y1 =-0.816X1 + 84.76 (R2 =0.993 ),Y2 =-0.873X2 + 92.04( R2 =0.999) respectively.
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Objective To investigate the feasibility and clinical effects of suture anchors technique for acute distal avulsion fracture of patella. Methods From January 2007 to July 2009, 33 patients(18males and 15 females, ranging from 28 to 72 years, with an average of 49.3 years) were treated with the suture anchors technique. The average time from injury to surgery was 3.5 days(2 to 7 d). Injury reason: traffic accident in 12 cases, injured in daily life in 11 cases, sports injury 9 cases, no clear trauma history in one case. The Bostman evaluation system, follow-up X-ray, and knee mobility were used to analyze the clinical effects of suture anchors technique. Results All patients obtained the follow-up and the average time was 16.5 months(12 to 23 months ). One patient suffered foreign body reaction, and his wound healing was good after operation. The remaining patient wound healing was good. No complications were found in all patients,such as popliteal fossa artery, tibial nerves or peroneal nerve complication. Bostman patella fracture functional score 27.3±2.3 points at the sixth months follow up, 28 cases were rated as excellent, 5 cases were good, and the excellent and good rate was 100%. Six months later, X-ray showed bone healing, knee activity recovered well. The Range of Motion was extension for -2.5°±2.1° and flexion for 122°±5.3°. Conclusion Suture anchors fixation in treatment of acute distal avulsion fracture of patella has a good efficacy, safety and reliability advantages, and without the second operation. This technique provides an alternative in lower pole patella fracture.