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    Clinical Study

  • Shi Kehui, Dang Xilong, Yan Senhui, He Quan, Liu Hua, Gao Julin, Wang Meng, Xue Jinhong, Wei Meng, Chen Lei, Sun Lingshuang, Liu Wenyan, Liu Xiaomin, Jiang Hongli
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    Objective To investigate the clinical effect on ultrasound-guided vascular access-interventional therapy of hemodialysis in day surgery mode. Methods Hemodialysis patients with vascular access dysfunction who underwent ultrasound-guided interventional therapy in the First Affiliated Hospital of Xi'an Jiaotong University from September 1, 2018 to October 31, 2020 were retrospectively analyzed. Demographic and clinical data were collected by electronic medical record system and telephone follow-up. Kaplan-Meier method was used to analyze the patency rate of vascular access. Results A total of 421 cases of ultrasound-guided vascular access intervention were performed in 269 patients. The technical success rates of stenosis, chronic occlusion and acute occlusion lesion were 98.8%, 90.6% and 86.4%, respectively, and 406 cases (96.4%) of 246 patients were clinically successful. The postoperative brachial artery blood flow was 821(627, 1 029) ml/min, which was significantly higher than 309(202, 453) ml/min before the operation (Z=-13.547, P<0.001). No serious complications occurred during and after the operation. At 6, 12, 18 and 24 months after operation, the primary patency rate was 74%, 59%, 48% and 45%, respectively, the assisted primary patency rate was 94%, 91%, 88% and 82%, and the secondary patency rate was 96%, 93%, 91% and 86%. Compared with the conventional inpatient surgery mode, the total cost of the day surgery mode was significantly reduced [12 067(10 051, 13 198) yuan vs 14 986(12 411, 20 643) yuan, Z=-13.185, P<0.001], and the hospital stay was significantly shortened [5.1(3.5, 6.9) h vs 73.4(31.6, 146.6) h, Z=-13.348, P<0.001]. Conclusion It is safe and effective to perform interventional therapy for vascular access malfunction under ultrasound in day surgery mode, which can save cost and time of hospitalization, and can be carried out in hospitals with relevant conditions.

  • Lin Zhi, Lin Xiaoyuan, Zhu Hongzhang, Chen Bin, Dai Haitao, Huang Qiuping, Yang Jianyong, Huang Yonghui
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    Objective To evaluate the efficacy of angioplasty on percutaneous superior vena cava occlusion in hemodialysis patients with tunnel-cuffed catheter (TCC) under digital subtraction angiography (DSA) guidance. Methods A total of 62 hemodialysis patients with TCC in the First Affiliated Hospital of Sun Yat-sen University from December 2017 to June 2020 were enrolled retrospectively. According to the patency of the superior vena cava, the patients were divided into experiment group (n=20) and control group (n=42) in this study. Hemodialysis patients with superior vena cava occlusion in the experiment group received angioplasty, including balloon angioplasty, stenting and sharp recanalization, and catheterization with TCC under DSA guidance, while hemodialysis patients without superior vena cava occlusion in the control group only underwent catheterization with TCC under DSA guidance. The 1-year TCC patency rate, postoperative TCC blood flow and treatment-related complications between the two groups were compared. Results In the experiment group, a total of 11 patients were treated only by percutaneous transluminal angioplasty, while 9 patients were treated combined percutaneous transluminal angioplasty with stent placement. In addition, 3 patients underwent sharp recanalization of superior vena cava occlusion. A total of 9 stents and 29 balloons were used. The course of dialysis in experiment group was longer than that in control group (P<0.05). There were no significant differences in the 1-year TCC patency rate (85.0% vs 95.2%, P>0.05), postoperative TCC blood flow [(257.83±16.55) ml/min vs (251.90±18.79) ml/min, P>0.05] and incidence of treatment-related complications (grade 1-2, 30.0% vs 35.7%, P>0.05) between the two groups, respectively. Patients in the two groups had none of serious operation-related complications, and only some patients had mild clinical manifestations, such as postoperative pain and bleeding at the puncture point. Conclusions For patients with longer duration of hemodialysis and superior vena cava stenosis and occlusion treated with angioplasty, the clinical effect of TCC within one year is equivalent to that of hemodialysis patients without angioplasty.

  • Li Aihua, Deng Lizhen, Lai Aijun, Zhuo Wanling, Deng Xiushan, Deng Yinghui, Liang Mengjun, Jiang Zongpei
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    Objective To explore the difference of blood pressure compliance rate in patients with continuous ambulatory peritoneal dialysis (CAPD) in the internet of things (IoT) follow-up and conventional care. Methods CAPD patients from 3 peritoneal dialysis centers from May 2019 to October 2019 were included in this retrospective cohort study. They were divided into IoT group and conventional care group according to the way of follow-up. The difference in blood pressure compliance rate during 1 year of follow-up between the two groups was observed. The primary outcome was defined as the proportion of patients with blood pressure compliance rate≥85%. Results A total of 75 patients were included in this study, in during 32 patients in IoT group and 43 patients in conventional care group. The comparison of baseline data between the two groups showed that the dialysis age of patients in IoT group was shorter (P<0.01). After a median of 9(9, 12) months follow-up, the median blood pressure compliance rate was 85.2% (65.2%, 95.1%), and 25 patients (65.6%) in IoT group had met the target of blood pressure compliance rate≥85%, which was significantly higher than that in the conventional care group (17 cases, 39.5%) ( χ2=4.996, P=0.025). The cumulative probability of the target of blood pressure compliance rate≥85% was 97%, 90%, 90% and 52%, respectively in IoT group, while 95%, 86%, 55% and 34%, respectively in conventional care group after 3, 6, 9 and 12 months of follow-up, and the different between the two groups was significant (Log-rank χ2=4.774, P=0.029). Adjusted for age, sex and dialysis age, the multivariate Cox proportional risk regression model showed that serum creatinine level(for every 1 μmol/L increase, HR=1.002, 95%CI 1.000-1.003, P=0.033), follow-up mode (IoT follow-up vs conventional care, HR=0.023, 95%CI 0.003-0.210, P=0.001), follow-up times (for each additional time, HR=0.879, 95%CI 0.823-0.939, P<0.001) and the rate of weight compliance (for each increase of 1%, HR=0.964, 95%CI 0.939-0.991, P=0.008) was the independent influencing factors for the blood pressure compliance rate<85%. The results of subgroup analysis showed that patients with shorter dialysis age (<10 months) and in the centers where the nurses finished the PD follow-up work as part-time job had better blood pressure control in IoT follow-up. Conclusions IoT follow-up is helpful to improve CAPD patients' blood pressure compliance rate. Elevated serum creatinine level at baseline is the independent risk factor associated with poor blood pressure compliance. However, IoT follow-up, more follow-up times and the elevated rate of weight compliance are the protective factors for blood pressure compliance. IoT follow-up mode is more recommended for patients with short dialysis age and for dialysis centers where most of the nurses are part-time.

  • Fei Danfeng, Lan Lan, Ren Pingping, Liu Guangjun, Wang Yaomin, Zhu Yilin, Chen Jianghua, Han Fei
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    Objective To investigate the efficacy and safety of individualized rituximab rescue therapy for active lupus nephritis with acute kidney injury (AKI). Methods The clinical data of lupus nephritis patients with AKI treated with rituximab at the Kidney Disease Center of the First Affiliated Hospital of Zhejiang University School of Medicine from April 2017 to June 2020 were collected, and the renal remission rate and adverse events after rituximab treatment were analyzed retrospectively. The Kaplan-Meier method was used to calculate the cumulative incidence of patients' remission. Results There were 13 patients enrolled, including 8 females, and aged (35.23±15.92) years old. The urinary protein/creatinine ratio was (5.22±1.57) g/g before rituximab treatment. Four patients were on dialysis at admission, and 9 patients without dialysis had serum creatinine of (223.22±85.73) μmol/L. Eight patients were confirmed as proliferative lupus nephritis by renal biopsies, including 7 cases with crescent formation and 1 case with thrombotic microangiopathy (TMA), and the other 5 cases without renal biopsies were clinically diagnosed as TMA. The dose of rituximab was (815±516) mg (200-2 100 mg), and all the patients reached the state of peripheral blood B cells clearance (CD19+ B cell count was<5/μl). After the first treatment of rituximab, the median time to B-cell clearance was 21(15, 35) days, and 8 patients reached B-cell depletion (CD19+ B cell count was 0). The remission rate was 12/13 (two cases reached complete remission, and 10 cases reached partial remission). Three cases stopped dialysis, and 1 case (with glomerulosclerosis of 52.94%) entered maintaining dialysis. The relapse times in the maintenance remission period of 7 patients with refractory lupus nephritis declined significantly from (1.57±0.53) times in a median history of 60(20, 109) months to (0.43±0.79) times in a median history of 18(10, 23) months after the use of rituximab (P=0.015). After using rituximab, the incidence of infection was 7/13. The median time from the use of rituximab to infection was 26(4, 44) days. Pulmonary infection (5/13) was the most common type and all infected patients recovered after anti-infection treatment. Conclusions Rituximab can be used in the treatment of active lupus nephritis with AKI, especially in patients with crescent formation and TMA, but the infection should be paid close attention to and prevented.

  • Cao Tao, Xu Ricong, Xu Yi, Wan Qijun
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    Objective To investigate the relationship between serum C3 and progression of renal function in IgA nephropathy. Methods A single-center retrospective cohort study was conducted in patients with IgA nephropathy confirmed by renal biopsy who were admitted to the Second People's Hospital of Shenzhen from January 2011 to June 2020 and the patients were followed up until January 2021. Patients with secondary IgA nephropathy, baseline estimated glomerular filtration rate (eGFR)<30 ml·min-1·(1.73 m2)-1, lack of baseline serum C3 or creatinine, and follow-up time<6 months were excluded. The clinical data, laboratory examination and renal pathology were collected. The threshold effect analysis was used to obtain the cut-off point, and inflection point and 95% confidence interval were obtained using bootstrapping resampling technique. According to the cut-off point, the patients were divided into serum C3<0.97 g/L group and C3≥0.97 g/L group. The baseline data between the two groups were compared. Cox regression model was used to analyze the correlation between serum C3 level and renal function progression. Results A total of 414 patients were enrolled in this study, with 145 males (35.0%), and age of (35.15±9.18) years old. The baseline eGFR was 77.80(46.67, 106.10) ml·min-1·(1.73 m2)-1, and the serum C3 was (1.04 ± 0.19) g/L. There were 153 patients with serum C3<0.97 g/L and 261 patients with serum C3≥0.97 g/L. Compared to patients with serum C3≥0.97 g/L, those patients with serum C3<0.97 g/L were younger and had higher proportion of females, higher levels of hemoglobin and eGFR, and lower levels of mean arterial pressure, total cholesterol, triglyceride, serum uric acid, serum creatinine, 24 h urinary protein, IgA and C4 (all P<0.05). The relationship between serum C3 and progression of renal function was found to be U-shaped by smooth curve fitting. After adjustment for confounding factors such as age, sex, mean arterial pressure, serum uric acid, 24 h urinary protein, and renal pathology (MESTC), the results of the threshold effect and multivariate Cox regression showed, for patients with C3<0.97 g/L, the risk of renal function progression decreased by 40% for every 0.1 g/L increase of C3 (HR=0.60, 95%CI 0.39-0.94, P=0.024), but for patients with C3≥0.97 g/L, every 0.1 g/L increase in serum C3 increased the risk of renal function progression by 27%(HR=1.27, 95%CI 1.03-1.57, P=0.027). The inflection point was 0.97(95%CI 0.92-1.01) g/L. Conclusions Serum C3 is nonlinear correlated with the progression of renal function in patients with IgA nephropathy. Serum C3 level maintaining at 0.92-1.01 g/L is associated with better renal prognosis.

  • Zhao Hongjuan, Jiang Hong, Xu Shiru, Yang Shufen, Song Xue, Wang Xiaoqin, Lu Chen
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    Objective To investigate the pathological spectrum and variation of adult renal biopsies in People's Hospital of Xinjiang Uygur Autonomous Region from 1986 to 2020. Methods The pathological data of 5 652 adult renal biopsies from August 1986 to December 2020 were retrospectively collected, and characteristics of pathological spectrum were analyzed. Regarding every 5 years as a research stage, the whole period was divided into 7 stages to analyze the pathological features and variation of renal biopsies. The first stage (P1) started from August 1986 to December 1990. The second stage (P2) started from January 1991 to December 1995. The third stage (P3) started from January 1996 to December 2000. The fourth stage (P4) started from January 2001 to December 2005. The fifth stage (P5) started from January 2006 to December 2010. The sixth stage (P6) started from January 2011 to December 2015. The seventh stage (P7) started from January 2016 to December 2020. Results The age was (36.47±14.86) years old (18-83 years old) in 5 652 renal biopsies. There were 2 961 males (52.39%). There were 5 636 cases of autologous kidney biopsy and 16 cases of transplanted kidney biopsy. The descending order of incidence classified by disease types were primary glomerular disease (PGD, 4 470 cases, 79.31%), secondary glomerular disease (SGD, 994 cases, 17.64%), tubular-interstitial disease (160 cases, 2.84%), and hereditary nephropathy (12 cases, 0.21%). IgA nephropathy (IgAN, 1 573 cases, 35.19%) was the most frequent pathologic type of PGD, followed by membranous nephropathy (MN, 1 028 cases, 23.00%), mesangial proliferative glomerulonephritis (MsPGN, 878 cases, 19.64%), minimal change disease (MCD, 427 cases, 9.55%), and focal segmental glomerulosclerosis (345 cases, 7.72%). Lupus nephritis (LN, 251 cases, 25.25%) was the most common type of SGD, followed by hypertension nephropathy (193 cases, 19.42%), diabetic kidney disease (168 cases, 16.90%), purpura nephritis (138 cases, 13.88%), and ischemic nephropathy (90 cases, 9.05%). IgAN was the most common type of primary glomerulonephritis and mainly occurred in the age group of 18-59 years old. PGD was the most common glomerular disease in Han nationality (78.33%), Uygur nationality (81.72%) and other ethnic (77.15%) groups. Using Bonferroni correction method, the incidence of PGD in Uygur nationality was higher than that in Han nationality (P<0.017). From P1 to P7, the detection rates of MN and MCD were increased in common renal pathological types, meanwhile, the ratio of MsPGN was decreased (all P<0.05). From P5 to P7, the detection rates of MN in Han nationality and Uygur nationality increased in the common pathological types of PGD, meanwhile, the ratio of MsPGN decreased (all P<0.05). LN was the most common SGD. The incidence of LN in females was higher than that in males (P<0.001). Using Bonferroni correction method, the incidence of SGD in Uygur nationality was lower than that in Han nationality (P<0.017). There was no significant variation in the common pathological type of SGD in Han and Uygur nationalities. Chronic rejection was the main pathological type of transplanted kidney biopsies. Conclusions PGD is the main type of kidney disease spectrum in People's Hospital of Xinjiang Uygur Autonomous Region. IgAN is the most common PGD and mainly occurrs in the age group of 18-59 years old. As time goes by, the proportion of MN and MCD is increased, meanwhile the proportion of MsPGN is decreased significantly. LN is the most common SGD.

  • Xu Rongrong, Zhu Lin, Guan Yang, Zhou Jianhua, Yuan Huiqing, Qiu Liru
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    Objective To report two cases of steroid-resistant nephrotic syndrome (SRNS) caused by LAMB2 gene mutation, and summarize the characteristics of genotype, clinical and pathological phenotypes of children with LAMB2 gene mutation. Methods Two cases with SRNS caused by LAMB2 gene mutation were from Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology in December 2013 and September 2019. The demographic, family history and clinical data of two cases were collected, and the peripheral blood genomic DNA was captured and sequenced by whole exome sequencing. PubMed, Medline, CNKI and Wanfang databases were searched to summarize the clinicopathological phenotypes and genotypes of patients with LAMB2 mutation. Results Among the two cases with SRNS caused by LAMB2 gene mutation, the clinical phenotypes were all manifested as nephrotic level of proteinuria and hypoalbuminemia, and there was no extrarenal clinical manifestation. One case presented with basement membrane delamination and the other with focal segmental glomerulosclerosis (FSGS). LAMB2 mutations of two cases were Exon32 c.5390G>T(p.Cys1797Phe), Exon19 c.2557C>T(p.Arg853*) and Exon27 c.4370G>A(p.R1457Q), Exon23 c.3325G>A(p.E1109K), respectively. In literature retrieval, there were 37 cases with LAMB2 gene mutation, including 24 cases with renal biopsy data, 13 cases of focal segmental glomerulosclerosis (FSGS), 4 cases of minimal change disease, one case of diffuse mesangial sclerosis, one case of IgM nephropathy, two cases of thin basement membrane nephropathy, and three cases of mesangial hyperplasia. Among them, eight cases had basement membrane delamination tear. Among the 37 cases, 11 cases were homozygous, 22 cases were complex heterozygosity, and 4 cases were heterozygous mutation. Conclusions LAMB2 mutation may cause delamination tear of glomerular basement membrane. The clinical phenotype is congenital nephrotic syndrome or SRNS. The literature review shows the extrarenal manifestations caused by LAMB2 mutation are mostly various ocular abnormalities, as well as respiratory, digestive and nervous system abnormalities, and the time of progression to end-stage renal disease is also different.

  • Basic Study

  • Zheng Yan, Ma Yuan, Ren Xuejing, Niu Peiyuan, Yan Lei, Cao Huixia, Shao Fengmin
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    Objective To establish a rat model of neurogenic bladder and analyze the changes in kidney morphology and function and the expression of proteins in AngiotensinⅡ(AngⅡ)/transforming growth factor β1 (TGF-β1)/Smads pathway. Methods Sprague-Dawley rats were randomly divided into experimental group (spinal nerve amputation, n=36) and control group (sham operation, n=12). At 6, 12, and 24 weeks, the bladder compliance was measured by cystometry, the kidney morphology was detected by B-ultrasound, blood urea nitrogen (BUN) and serum creatinine (Scr) in blood samples were examined, the kidney pathological changes were detected by Masson and HE staining, the distribution of AngⅡ/TGF-β1/Smads pathway proteins was analyzed by immunohistochemisty, and the protein expressions in kidney were detected by Western blotting. Results Urodynamics showed that the basic bladder pressure in experimental group was higher than that in control group. B-ultrasound showed that compared with the control group, the diameter of the renal pelvis of the rats with nerve dissection gradually increased (P<0.05), and the hydronephrosis was gradually obvious. Compared with the control group, the BUN and Scr in experimental group gradually increased (both P<0.01). Masson and HE staining showed that compared with the control group, the collagen expression and renal tubulointerstitial scores in experimental group were gradually increased (both P<0.01). Immunohistochemisty showed that compared with the control group, in experimental group the expression of angiotensinⅡ receptor type 1 (AT1), TGF-β receptor 1(TGF-βR1), phosphorylated Smad2 gradually increased (all P<0.01), the pathway inhibitor Smad6 gradually decreased (P<0.01), and the distribution of each protein in kidney was consistent. Western blotting showed a corresponding expression trend with immunohistochemisty. Conclusions In neurogenic bladder caused by bilateral spinal nerve amputation, due to bladder dysfunction, increased bladder pressure induces hydronephrosis, destruction of the nephron structure, activation of AngⅡ/TGF-β1/Smads pathway, and renal fibrosis. This method is effective and has clinical similarities, laying a foundation for exploring neurogenic bladder treatment.

  • Zhan Xiaojiang, Fu Mengru, Liao Lu, Mei Wenjuan, Zhu Hengmei, Wei Xin, Xiao Jun
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    Objective To investigate the effect of pirfenidone (PFD) on the proliferation of human glomerular mesangial cells (HMC) stimulated by serum IgA1 in patients with IgA nephropathy (IgAN) and its possible mechanism. Methods Serum IgA1 of IgAN patients was purified by Jacalin affinity chromatography combined with Sephacryl S-200 gel filtration, and then heated to aggregated form (aIgA1). CCK8 method was used to confirm the concentration and time of PFD. The cells were divided into blank control group, IgA1 (0.5 mg/ml) group and IgA1 (0.5 mg/ml)+PFD (2 mmol/L) group. The CCK8 method was used to detect proliferation of mesangial cells. The cell cycle was detected by flow cytometry, and the proliferation index of mesangial cells was calculated. The expression levels of transforming growth factor β1 (TGF-β1), Smad4, Smad7, fibronectin (FN) and collagen Ⅳ protein and mRNA were detected through Western blotting and real-time PCR. Results Compared with blank control group, the proliferation of HMC was promoted significantly by aIgA1 (P<0.05). After PFD treatment, the proliferation of HMC was significantly inhibited (P<0.01). Compared with the blank control group, the number of G1 phase cells decreased, the number of S phase cells and cell proliferation index increased in IgA1 group (all P<0.05). Compared with IgA1 group, the number of cells in G1 phase increased significantly, the number of cells in S phase and G2/M phase decreased significantly, and the cell proliferation index decreased in IgA1+PFD group (all P<0.05). Western blotting and real-time PCR results showed that compared with the blank control group, the protein and mRNA expressions of collagen Ⅳ, FN and Smad4 in HMC stimulated by aIgA1 were significantly increased, while TGF-β1 protein expression was increased and Smad7 protein expression was decreased (all P<0.05). After PFD treatment, the protein and mRNA expression of collagen Ⅳ, FN and Smad4 in HMC was significantly decreased, while TGF-β1 protein expression was obviously decreased, and Smad7 protein was up-regulated (all P<0.05). There was no significant difference in the mRNA expression of TGF-β1 and Smad7 in each group before and after PFD treatment (all P>0.05). Conclusions PFD can increase the arrest of HMC in G1 phase, inhibit the proliferation of HMC induced by aIgA1 of IgAN patients, and reduce the production of extracellular matrix. The mechanism may be related to up-regulation of Smad7 expression and down-regulation of TGF-β1/Smad4 pathway.

  • Case Report

  • Xing Zhihua, Chu Xiaoxin, Wang Kun, Hu Shaoxian, Zeng Rui
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  • Liu Lingling, Qiu Beifen, Jiang Linsen, Feng Sheng, Sun Ci, Shen Huaying, Song Kai
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  • Review

  • Wang Zongyu, Fan Qiqiang, Zhou Yun
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