[Palliative Care for End-Stage Renal Disease:A Case Report and Literature Review].

Since end-stage renal disease leads to a variety of problems such as disability,reduced quality of life,and mental and psychological disorders,it has become a serious public health problem around the globe.Renal palliative care integrates palliative care philosophy in the care for patients with end-stage renal disease.As a planned,comprehensive,patient-centered care,renal palliative care focuses on the patient's symptoms and needs,aiming to reduce the suffering throughout the course of the disease,including but not limited to end-of-life care.This study reports the palliative care practice for a patient on maintenance dialysis in the Blood Purification Center of Peking Union Medical College Hospital and reviews the present situation of palliative care in end-stage renal disease.

[Nephrotic Syndrome Caused by AA Amyloidosis Secondary to Unicentric Castleman's Disease:Report of One Case].

AA amyloidosis is a rare systemic complication caused commonly by chronic inflammatory arthritis,periodic fever disease,vasculitis,tumors,etc.Castleman's disease is an uncommon cause of AA amyloidosis.Here,we reported a case of unicentric Castleman's disease-induced AA amyloidosis with nephrotic syndrome as the main manifestation.The laboratory examination showed elevated levels of inflammatory indicators.We summarized the clinical manifestations,diagnosis,and therapy of this case,aiming to facilitate the management of patients with unknown reasons of renal amyloidosis.

[Effect of Hope on Self-efficacy and Self-management in Patients with Chronic Kidney Disease(Stages 1-3)].

Objective To describe the status of hope,self-efficacy,and self-management in patients with chronic kidney disease(CKD)(stages 1-3)and to explore the interactions between these three variables.Methods Herth Hope Index,self-efficacy scale,and CKD self-management instrument were used to evaluate the patients with CKD(stages 1-3)in PUMC Hospital(n=153). Structural equation modeling was used to establish the structural equation model of hope,self-efficacy,and self-management.Results The median score of hope was 40.0(36.0,44.5),and 85.0% of patients were in higher level of hope. The median score of self-efficacy was 8.3(7.1,9.4)and the overall score of self-management was 89.0±13.4. There were no significant differences in level of hope and self-management among patients with different age,gender,marital status,educational level,course of disease,and CKD stages(all P>0.05). Age and marriage status were significantly associated with self-efficacy. Self-efficacy was significantly higher in >65 years group than in other age groups(P<0.05)and was significantly higher in married group than in single group(P<0.05).The level of hope had direct effect on self-efficacy(ß=0.67,P<0.05)and self-management(ß=0.46,P<0.05).Conclusions The levels of hope,self-efficacy,and self-management are high in patients with CKD(stages 1-3). Hope directly affects the self-efficacy and self-management of these patients.

[Effect of Glucocorticoid Pulse Therapy on Short-term Prognosis of Anti-neutrophil Cytoplasmic Antibodies-associated Glomerulonephritis].

Objective To investigate the prognosis predictors of anti-neutrophil cytoplasmic antibody(ANCA)-associated glomerulonephritis treated with glucocorticoid(GC).Methods The clinicopathological data of patients with biopsy-confirmed ANCA-associated glomerulonephritis were retrospective analyzed by retrieving the medical database in Peking Union Medical College Hospital from January 2000 to May 2015. Pathological categories were re-classified. Renal remission rates,infection rates,and death events were compared between intravenous glucocorticoid(GC)pulse therapy group and non-pulse group. Logistic regression analysis was performed to analyze factors influencing the short-term prognosis.Results Among the 81 patients with ANCA-associated glomerulonephritis,49(60.5%)received GC pulse therapy and 32(39.5%)did not. The GC pulse group had significantly lower estimated glomerular filtration rate at baseline(eGFR0)than the non-pulse group(t=3.003,P=0.015)but significantly higher 24-hour urinary protein(24 hUP)(t=2.394,P=0.002)and Birmingham Systemic Vasculitis Activity Score(BVAS)(t=0.049,P=0.013). There was no significant difference in the cumulative amount of cyclophosphamide(CTX)(t=1.336,P=0.245)between these two groups. The overall renal remission rate of GC pulse group in the 6 th month was significantly lower(48.7% vs. 79.3%;χ 2=6.591,P=0.024). Univariate analysis showed that baseline 24 hUP(t=6.222,P=0.017),eGFR0(t=3.727,P=0.046),and pathological category(χ 2=7.654,P=0.045)were associated with the overall renal remission rate in the 6 th month. Multivariate analysis showed the crescent category was an independent factor(OR=20.63,95%CI:2.217-191.973,P=0.008;compared with sclerotic category)for overall renal remission rate in the 6 th month,while GC pulse therapy was not an predictor(OR=0.271,95%CI:0.062-1.179,P=0.082). A total of 37 patients experienced infections within 6 months. The infection rate in GC pulse group(55.1%,27/49)was significantly higher than that of non-pulse group(31.3%,10/32)(P=0.042). Univariate regression analysis showed that eGFR0(t=1.912,P=0.049),baseline BVAS(t=-3.360,P=0.001)and GC pulse(χ 2=6.249,P=0.014)were associated with infection events within 6 months. Multivariate analysis showed that the baseline BVAS was the only predictor with 1.089 times for every 1 point increase in BVAS(OR=1.089,95%CI:1.006-1.179,P=0.034). Conclusions Crescentic category favors renal remission independently compared with sclerotic category. Patients with crescentic category may benefit more from intensive treatment. BVAS acts as an independent risk factor of infection.

A Cohort Study of Incidences and Risk Factors for Thromboembolic Events in Patients with Idiopathic Membranous Nephropathy.

Objective The aims of this study were to assess incidences and characteristics of arterial thromboembolic events (ATEs) and venous thromboembolic events (VTEs) in Chinese patients with idiopathic membranous nephropathy (IMN), and to identify the predisposing risk factors of them.Methods A total of 766 consecutive Chinese patients with IMN were enrolled in this retrospective cohort study. The cumulative incidences of newly diagnosed ATEs and VTEs were calculated using Kaplan-Meier methods. Univariable risk prediction model analysis followed by multivariable survival analysis was used to evaluate the potential risk factors of ATE and VTE.Results At 0.5, 1, 2, 3, and 5 years after biopsy diagnosis of IMN, the cumulative incidence of newly diagnosed ATEs were 4.3%, 5.7%, 6.3%, 7.1%, and 8.0%, and of newly diagnosed VTEs were 5.9%, 6.8%, 6.9%, 7.0%, and 7.2%, respectively. In 78 ATEs events (71 patients), cardiovascular diseases, thrombotic ischemic stroke (IS) and peripheral artery disease accounted for 50%, 45% and 5% respectively; in 60 VTEs events(53 patients), the deep vein thrombosis, renal vein thrombosis and pulmonary embolism accounted for 60%, 13% and 27% respectively. At the time of event, 42.1% patients with ATEs and 81.5% patients with VTEs were at nephrotic syndrome(NS) status (χ 2=18.1, P<0.001). Severe proteinuria, aging, smoking, hypertension and prior ATE history were associated with ATEs. Aging was demonstrated as the independent risk factor for ATEs (P=0.001), and hypoalbuminemia was the dominant independent risk factor for VTEs (P=0.03). Conclusions Patients with IMN have increased incidences of ATEs and VTEs, and most of events occurred within the first 6 months of the disease. IS was very common in ATEs in our cohort. Severe proteinuria and classic risk factors for atherosclerosis were associated with onset of ATEs. Hypoalbuminemia independently predicted VTEs. Risks of both ATEs and VTEs were particularly high in the status of NS, particularly VTEs.

[Clinicopathological Features and Treatment of Renal Impair in Primary Sjögren Syndrome].

Primary Sjögren syndrome,characterized by autoimmune epithelitis,is a prevalent systemic autoimmune disease involving multiple organs,among which kidney is a major target organ.Tubulointerstitial lesion is the most frequent form,involving proximal tubule,distal tubule,or collecting duct.The disease has an occult onset and may progressively develop into renal function impairment and end-stage renal disease,which can be accompanied with low-molecular-weight proteinuria,renal tubule acidosis and electrolyte disturbance.Pathologically,it is featured by lymphocyte infiltration,renal tubule atrophy,and interstitial fibrosis.Glomerular lesion is less common and usually takes the form of membranoproliferative glomerulitis.Glucocorticoid combined with immunosuppresant is the main treatment option,and B cell-targeted therapy has been reported.Most patients respond well to these treatments.In this article we review the prevalence,clinicopathological features,and treatment of renal disease in primary Sjögren syndrome.

Predictors of Short-term Outcomes of Patients with Peritoneal Dialysis-associated Peritonitis.

Objective To investigate the risk factors predicting the short-term outcomes of patients with peritoneal dialysis(PD)-associated peritonitis (PDAP). Methods In this retrospective cohort study,the clinical data at baseline and 0-3 months before peritonitis onset (peritonitis-free period) were collected from end-stage renal disease patients who started PD and suffered from PDAP between January 1,2004 and March 31,2017 in Peking Union Medical College Hospital. After 4 weeks of follow-up,these patients were divided into two groups according to the clinical outcomes,namely poor outcome group and good outcome group. Characteristics at baseline and before peritonitis were compared. Risk factors associated with short-term outcomes were also analyzed. Results Totally 162 PDAP patients were enrolled,among whom 55 (34.0%) experienced adverse outcomes and 107 (66.0%) had good outcome. At baseline,the proportion of clinical atherosclerotic vascular disease was significantly higher in poor outcome group than in good outcome group (49.1% vs. 31.8%;χ2=4.639,P=0.031),whereas indicators were comparable (all P>0.05). During the peritonitis-free period,significantly higher level of high-sensitivity C-reactive protein (hsCRP) [9.3(2.2,16.3)mg/dl vs. 3.6(1.4,9.5)mg/dl,Z=-2.879,P=0.004],higher proportion of low transport type of peritoneum function (8.7% vs. 1.0%;Z=4.879,P=0.027),and lower creatinine clearance rate [56.7 (45.7,71.1) ml/(min·w·1.73 m2)vs. 61.4 (54.5,76.4) ml/(min·w·1.73 m2);Z=-2.084,P=0.037] were observed in poor outcome group. Univariate Logistic regression analysis showed the combination of clinical atherosclerotic vascular disease (OR=2.070,95%CI:1.062-4.034,P=0.033) and higher hsCRP before peritonitis (OR=1.032,95%CI:1.001-1.059,P=0.015) were the risk factors of short-term poor outcome in PDAP patients. Multivariate Logistic regression analysis showed that,after the gender,age at peritonitis,PD duration,diabetes,and serum albumin before peritonitis were adjusted,higher hsCRP before peritonitis (OR=1.026,95%CI:1.000-1.052,P=0.046) and comorbidity of clinical atherosclerotic vascular disease (OR=2.105,95% CI:1.014-4.367,P=0.046) were the independent risk factors for the poor outcomes in PDAP patients. Conclusion Higher pre-peritonitis hsCRP and comorbidity of clinical atherosclerotic vascular disease at baseline may predict poor short-term outcomes in PDAP patients.

The Neglected Significance of Glomerular Density as a 5-year Progression Indicator for IgA Nephropathy△.

Objective To investigate whether glomerular density (GD) could be an independent prognostic factor for patients of IgA nephropathy with estimated glomerular filtration rate (eGFR) of 30 to 60 ml/min per 1.73 m2, or for patients with time-average proteinuria < 0.5 g/d. Methods A total of 173 patients with biopsy-confirmed IgA nephropathy diagnosed from January 2000 to December 2010 were included. All of these patients were followed up for more than 5 years. The endpoint was a > 30% of decline in eGFR from baseline after 5-year follow-up. The optimal cut-off value of GD was calculated by ROC curve. Kaplan-Meier method and Cox regression analysis was used for survival analysis. Results A 30% of decline in eGFR occurred in 14.5% of all patients. The optimal diagnostic cut-off value of GD was 1.99/mm2 (AUC = 0.90, sensitivity = 84.0%, specificity = 81.8%) determined by ROC curve. The low GD group (GD < 1.99 per mm2) experienced a significant increase in renal endpoint for patients with eGFR of 30 to 60 ml/min per 1.73 m2 (six patients in lower GD group, while one patient in the other group). For patients with time-average proteinuria < 0.5 g/d, the lower GD group showed a higher eGFR decline from baseline (4.5±16.7 ml/min per 1.73 m2 vs. -8.1±21.4 ml/min per 1.73 m2, P = 0.038); two patients in this group reached the endpoint, while no patients in the higher GD group did. Conclusion GD could be an independent prognostic factor for patients of IgA nephropathy with eGFR at 30 to 60 ml/min per 1.73 m2 of body surface, particularly for those with time-averaged amount of urine protein less than 0.5 g per day.

Effectiveness of Peritoneal Dialysis in Treating Adult End Stage Renal Disease Patients with Polycystic Kidney Disease.

Objective To observe the clinical characteristics,dialysis modalities,and outcomes of end stage renal disease(ESRD)patients with polycystic kidney disease(PKD)and to evaluate the feasibility of peritoneal dialysis in these population. Methods The clinical data of ESRD patient whose primary diagnosis was PKD in Peking Union Medical College Hospital were retrospectively collected from January 1993 to December 2015.PKD patients were divided into two groups according to dialysis modality,namely peritoneal dialysis group(PKD-PD)group and hemodialysis(PKD-HD)group.In addition,we randomly chose non-PKD patients from 622 peritoneal dialysis patients who were matched with PKD-PD patients in age,gender and dialysis time.The primary end point was death.The survival rate was calculated by Kaplan-Meier analysis and the risk factors for suivival were analyzed by Cox regression model. Results Totally 47 PKD patients were enrolled,including 33 patients in PKD-PD group and 14 patients in PKD-HD group,and 42 non-PKD patients as the control group.The average age of PKD patients was(53±11)years,of which 38.3% were women.When compared with PKD-HD group,no significant difference in age,gender,comorbidities,kidney size,and residual glomerular filtration rate were observed in PKD-PD patients at baseline(all P>0.05).The average time on dialysis of PKD-PD patients was(36.2±33.1)months.The weekly urea clearance index(Kt/V)and weekly creatinine clearance were similar to non-PKD-PD group at 3 months,1 year,3 years,and 5 years(all P>0.05).The peritonitis rate was 1 episode/84.5 months.The survival rates at 1 year,3 years,and 5 years of PKD-PD group were 85.7%,78.6%,and 78.6%,which were similar to non-PKD-PD group and PKD-HD group respectively(all P>0.05).Multivariate Cox regression analysis showed that neither PKD nor PD independently predicted the mortality. Conclusion PD can be an option for ESRD patients with PKD.

Value of Chloride Clearance Test in Differential Diagnosis of Gitelman Syndrome.

Objective To investigate the value of chloride clearance test in differential diagnosis of Gitelman syndrome (GS). Methods For patients with hypokalemic metabolic alkalosis and highly suspected GS,clinical data were documented and SLC12A3 gene screening was performed as gold standard to diagnose GS. Hydrochlorothiazide (HCT) test and furosemide (FUR) test were performed according to the standard process. Baseline and maximal increasement of chloride excretion fraction (FECl,the net and relative increase measured as εFECl) were compared between patients and controls to evaluated the reaction to the corresponding diuretics. Receiver operating characteristic (ROC) curve was used to evaluate the sensitivity and specificity of HCT test in GS diagnosis. Results Totally 27 patients and 20 health controls received HCT test. Among those patients,23 were diagnosed with GS genetically. When using the net and relative εFECl to diagnose GS,the areas under the ROC curve were 0.987 (95% CI:0.963～1.000,P<0.001) and 0.984 (95%CI:0.950～1.000,P<0.001),respectively. When a reasonable cutoff value for εFECl was selected,the sensitivity and specificity were both higher than 95%. Eight patients received both HCT test and FUR test. Five of them showed decreased reaction to HCT(net εFECl≤2.86% or relative εFECl≤223%),while normal reaction to FUR.SLC12A3 mutations confirmed their GS. Three patients with blunt reaction to FUR showed normal reaction to HCT,finally they were diagnosed as BS clinically because no SLC12A3 gene mutation was detected. Conclusion Comprehensive application of HCT test and FUR test to evaluate the diuretic reaction can effectively differentiate GS and BS.

Assessment of renal tissue elasticity by acoustic radiation force impulse quantification with histopathological correlation: preliminary experience in chronic kidney disease.

OBJECTIVES: Chronic kidney disease (CKD), a progressive and irreversible pathological syndrome, is the major cause of renal failure. Renal fibrosis is the principal process underlying the progression of CKD. Acoustic radiation force impulse (ARFI) quantification is a promising noninvasive method for assessing tissue stiffness. We evaluated whether the technique could reveal renal tissue fibrosis in CKD patients. METHODS: ARFI assessments were performed in 45 patients with CKD referred for renal biopsies to measure cortical shear wave velocity (SWV). During measurement, a standardized method was employed, which aimed to minimize the potential impact of variation of transducer force, sampling error of non-cortical tissue and structural anisotropy of the kidney. Then SWV was compared to patients' CKD stage and pathological fibrosis indicators. RESULTS: ARFI could not predict the different stages of CKD. Spearman correlation analysis showed that SWV did not correlate with any pathological indicators of fibrosis. CONCLUSION: ARFI assesses tissue stiffness of CKD kidneys by measuring cortical SWV. However, SWV did not show significant correlations with CKD stage and fibrosis indicators despite using standardized measurement methods. We therefore suggest that it would be necessary to evaluate the effect of pathological complexity and tissue perfusion of the kidney on stiffness assessment in future studies. KEY POINTS: • Acoustic radiation force impulse (ARFI) can quantify tissue elasticity of CKD kidney. • Despite standardized measurement, ARFI-estimated elasticity did not correlate with renal fibrosis. • Effects of pathological complexity and tissue perfusion on renal stiffness warrant further study.

[Association of lipid metabolism disorder with peritoneum transport ability and mortality in peritoneal dialysis patients].

OBJECTIVE: To observe the features of lipid metabolism disorders of peritoneal dialysis(PD)patients and hemodialysis(HD)patients and explore the association of lipid metabolism disorder with peritoneum transport ability and mortality. METHODS: The clinical data of 127 PD patients and 95 HD patients who had received regular dialysis for more than 3 months in Peking Union Medical College Hospital since March 2009 were retrospectively analyzed.Serum lipid profiles were tested.Serum hypersensitive C reactive protein(hsCRP)was examined by immune turbidimetric method.Serum carbohydrate antigen 125(CA125)and iPTH were detected by electrochemical luminescence method.Peritoneum transport ability was evaluated through peritoneal equilibration test(PET).After a 2-year follow-up,the levels of CA125 and the peritoneum transport abilities were compared between the baseline data and the end point,and the relationship between lipid disorder and the mortality was analyzed. RESULTS: After the 2-year follow-up,25(19.7%)PD patients died.The leading cause of death was congestive heart failure(56.0%),followed by myocardial infarction(12.0%),septic shock(12.0%),respiratory failure(8.0%),asphyxiation(8.0%),and gastrointestinal bleeding(4.0%).Compared with the survivors,the death patients were older(P=0.005),with significant lower albumin level(P=0.000)and pre-albumin level(P=0.001).However,there was no significant difference in other clinical features including body mass index(BMI),blood pressure,dialysis time,nPCR,iPTH,hemoglobin,hsCRP,and serum lipid level(all P>0.05).COX regression analysis showed that diabetes mellitus(P=0.030)and mean SBP(P=0.048)were significantly associated with the mortality of PD patients.At the baseline,the CA125 level in patients with high,high average,and low average transport status of peritoneum was(38.02±64.37),(21.21±19.41),and(17.55±23.2)U/ml,respectively(P=0.09).There was no association between the transport status and lipid(TC,TG and LDL). CONCLUSIONS: Congestive heart failure is the leading cause of death among PD patients.Diabetes and blood pressure are the dependent risk factors of mortality.Lipid disorder is associated with CA125,while its association with peritoneum transport ability or mortality was not found.

Association between vitamin D insufficiency and the risk for gestational diabetes mellitus in pregnant Chinese women.

OBJECTIVE: To investigate the association between vitamin D deficiency and risk of gestational diabetes mellitus (GDM) in pregnant Chinese women. METHODS: A nested case-control study was conducted. Clinical and biochemical data were analyzed for 200 subjects with GDM and 200 subjects with normal glucose tolerance (NGT). RESULTS: The median (interquartile range) serum 25-hydroxyvitamin D (25OHD) levels were 22.39 (17.67, 29.38) and 25.86 (19.09, 34.88) nmol/L in the GDM and NGT groups, respectively. Rates of 25OHD deficiency or insufficiency were significantly higher in the GDM group than in the NGT group. Subjects with 25OHD levels <25 nmol/L had a 1.8-fold higher risk of GDM compared with subjects with higher vitamin D levels. In the GDM group, serum 25OHD was independently associated with HbA1c and insulin resistance after adjusting for confounding factors. In the NGT group, serum 25OHD was independently associated with fasting plasma glucose and systolic blood pressure after adjusting for maternal age and other confounding factors. CONCLUSION: 25OHD insufficiency is very common in Chinese women. Low 25OHD status may be associated with insulin resistance and act as a risk factor for GDM.

Th17/Treg imbalance in adult patients with minimal change nephrotic syndrome.

To determine whether Th17/Treg balance was abnormal in adult patients with minimal change nephrotic syndrome (MCNS), we studied 25 patients with new-onset MCNS and 20 normal persons. The results showed that MCNS patients exhibited a significant increase in Th17 number, Th17-related cytokines (IL-17 and IL-23), and transcription factor (RORγt) levels, as well as an obvious decrease in Treg number, Treg-related cytokines (TGF-ß1 and IL-10), and transcription factor (Foxp3) levels. The Th17/Treg ratios increased along with increased proteinuria and decreased albumin levels in patients with MCNS. IL-17 protein expression was also detected in the renal biopsy tissue of MCNS patients, particularly in patients with acute renal failure. Further, Th17/Treg balance returned to normal after effective corticosteroids therapy in 16 MCNS patients. These results indicated that Th17/Treg imbalance existed in MCNS patients, suggesting a potential role of Th17/Treg imbalance in the pathogenesis of MCNS.

[Clinical analysis of anti glomerular basement membrane disease with normal renal function].

OBJECTIVE: To investigate the clinical and pathologic characteristics of anti-glomerular basement membrane(GBM) disease with normal renal function. METHODS: The clinical and pathologic data of 6 patients with anti-GBM disease and normal renal function in Peking Union Medical College Hospital were reviewed retrospectively. Furthermore, 29 patients with anti-GBM disease and impaired renal function in the same period in the same hospital were enrolled as the control group. Factors that may influence the prognosis were analyzed. RESULTS: Six (17.1%) of all 35 patients maintained normal renal function for 12-133 months during follow-up. Five patients had microhematuria and proteinuria, one had pulmonary hemorrhage only, and three manifested as Goodpasture syndrome. Renal biopsies from 4 patients revealed linear deposition of IgG 2+-3+ along the glomerular capillary walls by immunofluorescence. As shown by normal light microscopy, mild mesangial proliferation and crescentic glomerulonephritis with a large amount of fibrinoid necrosis of glomerular capillary walls were observed in different patients; however, most pathological changes were mild. Five of these six patients were treated with immunosuppressive drugs and/or plasma exchange. Compared with the control group, the 6 patients with normal renal function had significantly higher hemoglobin[(77.97±20.62 vs.(99.67±19.80 g/L P=0.024], lower titers of anti-GBM antibody[(224.34 ± 145.79 vs.(80.23 ± 85.73 EU/ml P=0.027], and lower ratio of glomeruli with crescents[(0.58±0.29 vs.(0.17±0.27 ,P=0.005]. These 6 patients with normal renal function were followed up for 12-133 months, among whom 4 patients achieved complete remission and 2 had mild proteinuria and microhematuria. CONCLUSION: Anti-GBM disease with normal renal function is not uncommon. Most patients have mild pathologic changes and good prognosis.

Analysis of Glomerular IgG Subclasses Switch in Idiopathic Membranous Nephropathy Classified by Glomerular Phospholipase A2 Receptor Antigen and Serum Antibody.

BACKGROUND: The role of IgG subclass in idiopathic membranous nephropathy (IMN) was unclarified. Recent study found IgG subtype switches from IgG1 to IgG4 in the early pathological stage in IMN. The profile of IgG subclass in phospholipase A2 receptor- (PLA2R-) related and PLA2R-unrelated IMN was unrevealed. Our study is aimed at testifying whether IgG subclass switch existed in PLA2R-related and PLA2R-unrelated IMN, respectively. METHODS: Our study retrospectively enrolled 157 Chinese patients with biopsy-confirmed IMN between September 2017 and November 2019. We measured glomerular PLA2R antigen and serum anti-PLA2R antibody to classify the patients into PLA2R-related (n = 132) and PLA2R-unrelated (n = 25) subgroup. We evaluated glomerular IgG subclass by immunofluorescence (IF) predominance. Our study defined IgG subclass deposition as predominant if the IF score was higher than the other three and ≥1 +, or as codominant if the IF intensity was equal to any other and ≥1 +. We explored the relationship between IF predominance of glomerular IgG subtype and electron microscopic (EM) stages of IMN. RESULTS: We did not find statistical difference of predominant or codominant rate (pre/co-rate) among EM stages in any subclass (P > 0.05). Pre/co-rate of IgG3 linearly associated with EM stage in total and PLA2R-related subgroup (P = 0.044, P = 0.013). PLA2R-related subgroup showed higher IgG4 intensity (2.1 ± 0.6 vs. 1.6 ± 0.7, P = 0.001) and pre/co-rate of IgG4 in stage 1 (97% vs. 57%, P = 0.015) than PLA2R-unrelated group. We found no difference of IgG subclass pre/co-rate in different EM stages or linear association between pre/co-rate of IgG1, IgG2, IgG4, and EM stages (P > 0.05). CONCLUSIONS: Pre/co-rate of IgG3 declined with EM stage in total and PLA2R-related subgroup. We did not find IgG subclass switches from IgG1 to IgG4 in either IMN patients or subgroups.

Circulating tumor cell detection and single-cell analysis using an integrated workflow based on ChimeraX® -i120 Platform: A prospective study.

Circulating tumor cell (CTC) analysis holds great potential to be a noninvasive solution for clinical cancer management. A complete workflow that combined CTC detection and single-cell molecular analysis is required. We developed the ChimeraX® -i120 platform to facilitate negative enrichment, immunofluorescent labeling, and machine learning-based identification of CTCs. Analytical performances were evaluated, and a total of 477 participants were enrolled to validate the clinical feasibility of ChimeraX® -i120 CTC detection. We analyzed copy number alteration profiles of isolated single cells. The ChimeraX® -i120 platform had high sensitivity, accuracy, and reproducibility for CTC detection. In clinical samples, an average value of > 60% CTC-positive rate was found for five cancer types (i.e., liver, biliary duct, breast, colorectal, and lung), while CTCs were rarely identified in blood from healthy donors. In hepatocellular carcinoma patients treated with curative resection, CTC status was significantly associated with tumor characteristics, prognosis, and treatment response (all P < 0.05). Single-cell sequencing analysis revealed that heterogeneous genomic alteration patterns resided in different cells, patients, and cancers. Our results suggest that the use of this ChimeraX® -i120 platform and the integrated workflow has validity as a tool for CTC detection and downstream genomic profiling in the clinical setting.

Diagnosis and treatment of infective endocarditis in chronic hemodialysis patients.

OBJECTIVE: To analyze the clinical features of hemodialysis patients complicated by infective endo carditis. METHODS: The clinical features of six such patients admitted to Peking Union Medical College Hospital during the year 1990 to 2009 were analyzed. All of them were diagnosed based on Chinese Children Diagnostic Criteria for Infective Endocarditis. RESULTS: The average age of the six patients was 52.3 +/- 19.3 years old. Four were males. Vascular accesses at the onset of infective endocarditis were as follows: permanent catheters in three, temporary catheters in two, and arteriovenous fistula in one. Three were found with mitral valve involvement, two with aortic valve involvement, and one with both. Five vegetations were found by transthoracic echocardiography, and one by transesophageal echocardiography. Four had positive blood culture results. The catheters were all removed. Four of the patients were improved by antibiotics treatment, in which two were still on hemodialysis in the following 14-24 months and the other two were lost to follow-up. One patient received surgery, but died of heart failure after further hemodialysis for three months. One was well on maintenance hemodialysis for three months after surgery. CONCLUSIONS: Infective endocarditis should be suspected when hemodialysis patients suffer from long-term fever, for which prompt blood culture and transthoracic echocardiography confirmation could be performed. Transesophageal echocardiography could be considered even when transthoracic echocardiography produces negative findings. With catheters removed, full course of appropriate sensitive antibiotics and surgery if indicated could improve the outcome of chronic hemodialysis patients complicated by infective endocarditis.

Executive Summary: Clinical Practice Guideline for Autosomal Dominant Polycystic Kidney Disease in China.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, with a prevalence of 1/2,500-1/1,000, and it affects 1.25 million people in China. ADPKD is responsible for nearly 5% of end-stage renal disease cases, which leads to a major burden on public health. In 2016, the Chinese working group developed guidelines for the diagnosis and treatment of ADPKD, which promoted the clinical management of ADPKD in China. In the last 3 years, Chinese clinicians have deepened their understanding and standardized the management of ADPKD, and several basic and clinical studies on ADPKD have been conducted. In combination with international guidelines and research results, the working group updated the ADPKD guidelines in China. This guideline includes 5 chapters: introduction, diagnosis, kidney disease progression monitoring, treatment, and family planning. We highlight the main recommendations and suggestions of the ADPKD guidelines in this summary.

Glomerular disease associated with Takayasu arteritis: 6 cases analysis and review of the literature.

OBJECTIVE: To evaluate the clinical features, renal histopathology and therapeutic response to glucocorticoid and immunosuppressive agents in patients with glomerular disease associated with Takayasu arteritis (TA). METHODS: Patients with TA and renal biopsy-confirmed glomerular disease were investigated retrospectively. None of them had renal artery stenosis or occlusive changes. RESULTS: Six patients with glomerulopathy, accounting for 3.75% of the 160 TA patients admitted to our hospital at the same period, were analyzed. All of them were females with a mean age of 35.5 +/- 10.0 years. Four cases presented with lower extremity edema. Laboratory tests showed that one was nephrotic syndrome, three were nephrotic range proteinuria, and two of them had mild renal dysfunction. The other two patients were asymptomatic microscopic hematuria and proteinuria. Renal pathology revealed mild immunoglobulin A nephropathy in two cases, mild mesangial proliferative glomerulonephritis (GN), membranoproliferative GN, minimal change disease, and fibrillary GN in one case respectively. Five cases received glucocorticoids and cyclophosphamide therapy. Proteinuria and microscopic hematuria disappeared in 2 to 4 weeks after the initiation of therapy in three cases. The patient with membranoproliferative GN also reached complete remission of proteinuria and recovered renal function 6 months after the treatment. CONCLUSIONS: TA may induce glomerular disease as a part of its histological spectrum. Apart from ischemic glomerular disease, glomerular disease should be suspected when TA patients have microscopic hematuria or proteinuria, that may be therapeutically responsive to glucocorticoids and immunosuppressive agent in relative early phase.