Objective To evaluate the feasibility and value of multi-detector computed tomography venography (MDCTV) and three dimensional reconstruction image in the assessment of central venous occlusive disease in hemodialysis patients, and in the value of guiding interventional treatments. Methods Sixty hemodialysis patients with swelling of upper limbs were scanned by Toshiba 128-multislice spiral computed tomography (128-MSCT) and totally 80-100 ml non-ionic contrast media was injected into each of the patients via the peripheral veins of the contralateral limb with the rate of 4 ml/s. MSCT scanning was taken by the technique of intelligent triggering after setting scanning triggering threshold, with the monitoring?point?set in the development of the lumen of inferior vena cava, to detect the position and degree of vascular stenosis. The images were reformed as maximum intensity projection (MIP), volume rendering (VR), curved planar reformation and three-dimensional image reconstruction technique. Results MDCTV clearly demonstrated the lesion location in all cases enrolled. Seventy-five occlusive lesions were detected in the total of 60 hemodialysis patients with swelling of upper limbs by MDCTV, of which the lesions of brachiocephalic vein was 47, superior vena cava 15 and subclavian vein 13. Among the 75 stenosis lesions, the number of complete occlusive, severe, moderate and mild stenosis was 31, 24, 19 and only 1, respectively. MDCTV provided information coincident with that of digital?subtraction?angiography (DSA), which the correlation index was 0.401, while DSA showed that number of complete occlusive, severe, moderate and mild stenosis was 49, 7, 14 and 5, respectively. Percutaneous transluminal angioplasty was performed in 53 patients, and stent placement was done in 40 patients. After interventional treatments, swelling of upper limbs were obviously relieved and vascular accesses got functional recovery to?the?extent?that?they could meet the requirement of hemodialysis. Conclusions MDCTV is the first choice to evaluate the condition of central?venous?occlusive diseases of hemodialysis patients with advantages of non-invasion, high definition and three-dimensional?reconstruction. It can provide accurate evaluations of the conditions of occlusive lesions, which can be of great clinical significance to subsequent interventional therapy.

Objective To explore the risk factors and characteristics in patients with peritoneal dialysis who died in different periods. Methods The clinical data of new peritoneal dialysis patients in the Department of Nephrology and Peritoneal Dialysis Center of the First Affiliated Hospital of Nanchang University from November 1, 2005 to February 28, 2017 was retrospectively analyzed. The patients were divided into two groups according to the time of death: those who died within one year and died after one year. The risk factors of mortality between the two groups were analyzed by Cox regression model. Results A total of 997 patients were enrolled and 244 patients died. There were 69 patients (28.3%) died within one year and 175 patients (71.7%) died after one year. Cardiovascular and cerebrovascular disease was the dominating reason of death in both groups, accounting for 59.4% (died within one year group) and 51.4% (died after one year group) respectively. Cox regression analysis showed that for died within one year group, old age (HR=1.035, 95%CI: 1.016-1.055, P＜0.001), low blood total calcium (HR=0.167, 95%CI: 0.053-0.529, P=0.002), low albumin (HR=0.899, 95%CI: 0.856-0.943, P＜0.001) and low apolipoprotein A1 (HR=0.274, 95%CI: 0.095-0.789, P=0.016) were risk factors associated with mortality. However, for died after one year group, old age (HR=1.053, 95%CI: 1.038-1.069, P＜0.001), combined with diabetes (HR=2.181, 95%CI: 1.445-3.291, P＜0.001) and hypertriglyceride (HR=1.204, 95%CI: 1.065-1.362, P=0.003) were risk factors associated with mortality. Conclusions The risk factors of mortality for peritoneal dialysis patients of different periods were not exactly the same. For died within one year patients, old age, low blood total calcium, low albumin and low apolipoprotein A1 were independent risk factors for mortality.However, for died after one year patients, old age, combined with diabetes, and high triglycerides were independent risk factors for mortality.

Objective To study the effects of cisapride on symptoms of digestive and gastrointestinal hormones in chronic renal failure patients. Methods There were 46 cases of chronic renal failure patients in this paper, all patients were given routine treatment of the underlying disease and were randomly divided into two groups. 23 patients were additionally treated with cisapride as research group, and the others as control group. The gastrointestinal symptoms, serum somatostatin (SS), motilin (MOT) and vasoactive intestinal peptide (VIP) and other gastrointestinal hormones and renal function in two groups of patients were compared before and after treatment. Results The score of acid reflux, nausea, vomiting, abdominal distension, belching and other symptoms of hard feces and other gastrointestinal after treatment were significantly lower than that before treatment in research group and after treatment in control group (all P＜0.05). There was no significant change between before and after treatment in SS and VIP of two group (all P＞0.05), and MOT in control group (P＞0.05), but the MOT in research group was decreased significantly(P＜0.05). There was no significant difference in Scr, Ccr and BUN between the control and research group after treatment (all P＞0.05). Conclusion Conventional treatment combined with cisapride can improve the effect of gastrointestinal symptoms in patients with chronic renal failure, while reducing serum motilin level.

Objective To evaluate the prevalence of chronic kidney disease (CKD) in Chinese adult health check-up population, and to compare with the prevalence of CKD in the study of the general population as well as the large CKD cross-sectional study in China. Methods Epidemiological studies about CKD in Chinese adults health check-up population from January 2007 to December 2017 were searched in PubMed, SinoMed, CNKI, VIP and Wanfang Data. Meta-analysis of the prevalence of CKD was performed with software of Stata 12.0. Subgroup analyses of CKD staging, urban and rural, as well as geographical areas of the general population were executed. Results Twenty-two studies from adult health check-up population were included (238 349 persons). Egger's regression showed no publication bias (P＞0.05). The unstandardized prevalence rate of CKD was 12.49% (male 12.8%, female 12.5%). The respective unstandardized prevalences of proteinuria, hematuria and eGFR decline were 5.90%, 5.83% and 2.75%. The unstandardized prevalences of CKD in urban and rural population were 13.21% and 11.90%. The stages of CKD were mainly concentrated in the early stages. There was no significant difference in the non-standard detection rate of total eGFR decline among the adult medical examination population, the general population and the population studied cross-sectionally (P＞0.05). Furthermore, no significant difference in the non-standard detection rate of total hematuria and male hematuria was found between the adult health check-up population and the general population. In addition, the total proteinuric non-standard detection rate of the adult general population was similar with that of population studied cross-sectionally (P＞0.05). Conclusions The prevalence of CKD in Chinese adults is higher, the overall prevalence is however underestimated. The results of epidemiological investigation in adult health check-up population are similar to those of the general population, especially in men.

Objective To elucidate the clinical and pathological characteristics of the patients with thymoma-associated glomerulonephropathy. Methods In this retrospective study, the clinicopathologic characteristics of patients diagnosed as thymoma-associated glomerulonephropathy in Peking University First Hospital during the period between Oct 2008 and Jun 2017 were analyzed, including the histological classfication of thymoma, the clinicopathological features and the short-term prognosis. Results Altogether twelve patients were included with an average age of (55±16) years; male/female ratio was 3∶1. The B2 type thymoma was the most common type. Nine cases also suffered from myasthenia gravis, and eight cases of glomerulopathy accompanied by thymoma activity. The clinical presentation of glomerulopathy included nephrotic syndrome (11/12), acute kidney injury (10/12). Eleven patients received renal biopsy, among which five cases were minimal change nephropathy, three cases were membranous nephropathy, and the other three cases were focal segmental glomerulosclerosis, thrombotic microangiopathy and endocapillary proliferative glomerulonephritis, respectively. Eleven patients received immunosuppression therapy. After a median 12 months follow up, the proteinuria decreased in 7 cases, and renal function completely or partially recovered in 6 cases. Conclusions Minimal change disease is the most frequent pathological type of thymoma-associated glomerulonephropathy. Immunotherapy with glucocorticoid as first-line drug may be considered for thymoma-associated glomerulonephropathy with surgery, chemoradiation contraindications or non-remission of kidney disease after anti-tumor therapy.

Objective To Summarize and review the clinical data of two Bardet-Biedl syndrome (BBS) children so as to improve our understanding of the disease. Methods Clinical data of two BBS pedigree were collected. Gene analysis was performed by exon capture and next-generation sequencing, validated using Sanger sequencing. Results Both cases were male, Han nationality, born with polydactyly and had rapid weight gain after birth. They went to see the pediatric endocrinologist due to obesity, and found increased serum creatinine level, so were referral to pediatric nephrologists. Case one was further diagnosed rod-cone dystrophy, bilateral renal multiple cysts (chronic kidney disease, stage 4), atrial septal defect, mental retardation, hypertension and abnormal hearing. Two novel heterozygous compound mutation of BBS12 gene [c.1604T＞G (p.V535G) paternal, c.173delA (p.E58Efs*5) maternal] and one known BBS4 missense mutation (paternal) were detected. Case two was detected multiple cysts in kidneys by ultrasound in fetal phase. He was suspected to have autism. He had small penis, hypertension and renal injury (chronic kidney disease, stage 3). Two novel heterozygous compound mutation of BBS12 gene [c.1783T＞C (p.W595R) paternal, c.1749_1750delA (p.R584Dfs*54) maternal] were detected. All mutations were predicted to be harmful. Conclusions BBS is a rare disease. It is difficult to be diagnosed at early age. Polydactyly and obesity can be the early two symptoms for seeing doctors. Few cases have been diagnosed upon gene analysis. In this study, the mutation of BBS12 in Chinese and 4 novel mutations in BBS12 with severe renal injury are reported for the first time. It will extend the spectrum of BBS gene mutations.

Objective To analyze the mutations of SLC12A1 gene in nine Chinese families with Bartter syndrome type I (BS1), and analyze the relationship between genotype and phenotype. Methods The next generation sequencing was used to detect mutations in nine BS1 patients including eight with antenatal BS (aBS) and one with classical BS (cBS). Clinical characteristics and biochemical findings at the first admission as well as follow-up were reviewed. Results 15 different mutations of SLC12A1 gene were identified, including 11 novel ones. Among nine probands, seven were compound heterozygotes, two were homozygotes. All patients presented with polydipsia and polyuria, and eight with growth retardation. All patients had lower than-normal serum chloride concentration, metabolic alkalosis, and elevated basal renin activity and aldosterone, and seven had hypokalemia. Through treatment of indomethacin and/or potassium chloride, biochemical indicators could roughly restored normal. Conclusion These findings will enrich the human gene mutation database (HGMD) and provide valuable references to the genetic counseling and diagnosis for Chinese population.

Objective To investigate the role of BMP-2/Smad signaling pathway in the osteogenic differentiation of human aortic smooth muscle cells (HASMCs) caused by hyperphosphatemia-induced calcium phosphate (CaP) crystals. Methods High-phosphate medium was incubated at 37℃ for 3 days. CaP crystals and supernatant were isolated by ultracentrifugation. Scanning electron microscope and energy dispersive X-ray spectroscopy were performed for analysis of physicochemical characteristics of CaP crystals. HASMCs were cultured in vitro, and divided into high-phosphate, control, crystals and supernatant groups. Calcification was visualized by Alizarin red staining. Calcium loads in cells were quantified by o-cresolphthalein complexone method. Protein expression of bone morphogenetic protein-2 (BMP-2), Runt-related transcription factor 2 (RUNX2), osteopontin (OPN), phospho-Smad1/5/9 (p-Smad1/5/9) were quantified by Western blotting. After knockdowns of BMP-2 and Smad1 with small hairpin RNA (shRNA) interfering respectively in HASMCs, protein expressions were measured by Western blotting. Results High-phosphate medium induced the formation of CaP crystals. Compared with the cells in control group, CaP crystals significantly induced HASMCs calcification, increased calcium loads and up-regulated the levels of BMP-2, RUNX2 and OPN proteins (all P＜0.05). After the addition of CaP crystals into HASMCs, the level of p-Smad 1/5/9 protein peaked at 30 min (P＜0.05). After BMP-2 was knocked down in HASMCs, the expression of p-Smad1 caused by CaP crystals was blocked completely, and the expressions of RUNX2 and OPN caused by CaP crystals were reduced significantly (all P＜0.05). After Smad1 was knocked down in HASMCs, the expressions of RUNX2 and OPN caused by CaP crystals were decreased significantly (all P＜0.05). Conclusions Hyperphosphatemia-induced CaP crystals promoted osteogenic differentiation of HASMCs through the BMP-2/Smad signaling pathway.

Objective To investigate the effects of WNK3 kinase on the regulation of large-conductance calcium-activated potassium channels (Maxi K channels) on African green monkey kidney fibroblast-like cells (Cos-7 cells) and its mechanisms. Methods (1) Cos-7 cells were transfected with 0, 0.6, 1.2, 1.8 μg WNK3 plasmid+0.5 μg Maxi K plasmid. The total protein expression of Maxi K channel and the phosphorylation of mitogen-activated protein kinase (MAPK) extracellular regulated kinase-1 and-2 (ERK1/2) were detected by Western blotting. (2) Cos-7 cells were divided into the control group (2.5 μg Maxi K plasmid) and the experimental group (2.5 μg WNK3 plasmid+2.5 μg Maxi K plasmid). Cell surface biotinylation was used to investigate the cell surface protein expression of Maxi K channel in Cos-7 cells. Immunoprecipitation and Western blotting were used to detect the ubiquitination of Maxi K channel protein. (3) WNK3 kinase was knocked down by WNK3 siRNA. The lysosomal degradation pathway was blocked by the proton pump inhibitor (Baf-A1). Cos-7 cells were divided into Maxi K+negative control siRNA group, Maxi K+WNK3 siRNA group and Maxi K+WNK3 siRNA+Baf-A1 group. The protein expression of Maxi K channel protein was detected by Western blotting. Results (1) Compared with those in 0 μg WNK3 plasmid groups, in 0.6, 1.2, 1.8 μg WNK3 plasmid groups the total protein expression of the Maxi K channel increased and the phosphorylation level of MAPK ERK1/2 reduced on a dose-dependent manner (all P＜0.01). (2) Compared with those in the control group, the total protein expression and cell surface membrane protein expression of the Maxi K channel increased in the experimental group (P＜0.01), while the ubiquitination of the Maxi K channel protein reduced (P＜0.01). (3) Compared with the Maxi K+negative control siRNA group, the expression of Maxi K protein reduced in the Maxi K+WNK3 siRNA group (P＜0.01), but did not change in the Maxi K+WNK3 siRNA+Baf-A1 group (P＞0.05). The expression of Maxi K protein in Maxi K+WNK3 siRNA+Baf-A1 group was higher than that in Maxi K+WNK3 siRNA group (P＜0.01). Conclusions WNK3 kinase inhibits the lysosomal degradation pathway of Maxi K channel protein by reducing the ubiquitination of Maxi K channel, and promotes the expression of Maxi K channel protein in cells and on cell membrane. These effects may be achieved by suppressing MAPK ERK1/2 signal transduction pathway.