Gaucher disease in a patient with membranoproliferative glomerulonephritis: case report.

BACKGROUND: Gaucher disease (GD) is a rare autosomal recessive inherited, lysosomal storage disoder that involves liver, spleen, lung, bone, bone marrow even central nervous. However, GD associated membranoproliferative glomerulonephritis (MPGN) is seldom reported. CASE PRESENTATION: Here we described a case of 35-year-old man suffering from GD with hepatosplenomegaly, ascites, bone destruction, myelofibrosis and MPGN. Renal biopsy revealed MPGN and Gaucher cells presented in the glomeruli capillaries. ß-glucosidase activity was 1.95nmol/1 h/mg and gene detection demonstrated that one homozygous pathogenic variant Leu483Pro in GBA. He received the treatment of oral prednisone and mycophenolate mofetil and his ascites and renal outcomes had been significantly improved. CONCLUSIONS: Therapy of prednisone and mycophenolate mofetil may be an optional choice for patients with Gaucher disease who have no opportunity to use enzyme treatment.

Double-photoelectrode redox desalination of seawater.

High energy consumption and low salt removal rate are key barriers to realizing practical electrochemical seawater desalination processes. Here, we demonstrate a novel solar-driven redox flow desalination device with double photoelectrodes to achieve efficient desalination without electrical energy consumption. The device consists of three parts: one photoanode unit, one photocathode unit, and one redox flow desalination unit sandwiched between the two photoelectrode units. The photoelectrode units include a TiO2 photoanode and a NiO photocathode sensitized with N719 dye, triiodide/iodide redox electrolyte, and graphite paper integrated electrodes decorated with 3,4-ethylene-dioxythiophene. Two salt feeds are located between two ferro/ferricyanide redox flow chambers. Under light illumination, high-quality freshwater is obtained from brackish water containing different concentrations of NaCl from 1000 to 12,000 ppm with a high NaCl removal rate. The device can work in multiple desalination cycles without significant performance declines. Furthermore, natural seawater with an ionic conductivity of 53.45 mS cm-1 is desalinated to freshwater. This new design opens opportunities to realize efficient and practical solar-driven desalination processes.

High-Performance Photoelectrochemical Desalination Based on the Dye-Sensitized Bi2O3 Anode.

In this work, a solar-driven redox flow desalination system is reported, which combines a solar cell based on a Bi2O3 photoanode and a redox flow desalination cell through an integrated electrode. The Bi2O3 film was prepared through a simple one-step water bath deposition method and served as a photoanode after the coating of the N719 dye. The activated carbon (AC)-coated graphite paper served as both the integrated electrode and counter electrode. The I3-/I- redox electrolyte circulates in the solar cell channel between the photoanode and intergrated electrode, while the [Fe(CN)6]4-/[Fe(CN)6]3- electrolyte circulates in the redox flow desalination part between the integrated electrode and counter electrode. This dye-sensitized solar-driven desalination cell is capable of achieving a maximum salt removal rate of 62.89 µg/(cm2·min) without consuming any electrical power. The combination of the solar cell and redox flow desalination is highly efficient with double functions of desalination and energy release using light as a driving force. This current research work is significant for the development of efficient and stable photoanode materials in photoelectrochemical desalination.

Photo-Assisted Rechargeable Battery Desalination.

Herein, we propose a novel design of photo-assisted battery desalination, which provides the tri-function within a single device including the photo-assisted charge (electrical energy saving), energy storage, and desalination (salt removal). The photoelectrode (N719/TiO2) is directly integrated into the zinc-iodide (Zn-I) battery with the desalination stream in the middle portion of the device. This architecture can provide a reduced energy consumption up to 50%, an energy output of 42 W h mol-1NaCl, and a desalination rate of 13 µg/cm2 min-1. This work is significant for the inter-discipline study of the redox flow energy storage and energy-saving desalination.

The effectiveness and safety of corticosteroid therapy for IgA nephropathy with crescents: a prospective, randomized, controlled study.

BACKGROUND: Pozzi protocol (methylprednisolone intravenous infusion in 1st-3rd-5th months and oral steroid for 6 months) has been thought to be the classic therapy for IgA nephropathy (IgAN) patients with proteinuria> 1.0 g/24 h. There is no consensus on the treatments for IgAN with active pathological changes, especially for IgAN patients with crescents proportion < 50%. This study aimed to evaluate the effectiveness and safety of the treatment protocol of methylprednisolone intravenous infusion at the 1st-2nd-3rd months for IgAN patients with crescents. METHODS: In this prospective, randomized, controlled, non-blind study, 68 IgAN patients with crescents proportion < 50% were divided into the 1-2-3 group receiving 0.25 g/d methylprednisolone intravenously for 3 consecutive days in the 1st-2nd-3rd months, and oral prednisone 0.5 mg/kg/d on consecutive days for 6 months and the 1-3-5 group with the same intravenous methylprednisolone treatment in the 1st-3rd-5th months, and the same oral prednisone. The primary outcome measure was remission of proteinuria (complete or partial); while the secondary outcome measures were deterioration of renal function (evidenced by a 50% rise from baseline serum creatinine levels, or a 25% decline from baseline eGFR levels). RESULTS: There was no significant difference in incidence of crescents (median 14.66% vs. 11.45%, p = 0.143) between the 1-2-3 and 1-3-5 groups. From month 1 to month 6, there was a decreasing trend in the levels of urine protein and serum creatinine and an increasing trend in eGFR in both groups. The mean period of remission in the 1-2-3 group seemed shorter. Overall, there were 55 (80.89%) patients meeting remission. The rates of remission in the 1-2-3 group and 1-3-5 group were 85.3 and 76.47%, respectively (P = 0.644). The 1-2-3 group had lower creatinine and higher eGFR than the 1-3-5 group, but the difference was not significant. The complication rate was 11.11 and 15.79% in the two groups, respectively. There was no significant difference in the complications between groups. CONCLUSIONS: Both the 1st-3rd-5th and 1st-2nd-3rd protocols can effectively alleviate proteinuria and protect renal function in IgAN patients with crescents but the 1st-2nd-3rd protocol seemed to have better effectiveness. TRIAL REGISTRATION: ClinicalTrials.gov, Identifier: NCT02160132 , Registered June 10, 2014.

Hypertriglyceridemia is a risk factor for treatment failure in patients with peritoneal dialysis-related peritonitis.

OBJECTIVE: Disorders of triglycerides (TG) are common in patients with peritoneal dialysis (PD). Hypertriglyceridemia has been demonstrated in various infections. The association between triglycerides and the outcomes of peritoneal dialysis-related peritonitis (PDRP) was investigated in this study. METHODS: We retrospectively investigated patients with PDRP from January 1, 2013 to October 31, 2020. Hypertriglyceridemia was defined as triglycerides ≥ 1.7 mmol/L. PDRP episodes were divided into two groups: hypertriglyceridemia and normal levels of triglycerides. The clinical and laboratory baseline data of the two groups were collected and compared. The association between triglycerides and treatment failure was analyzed by logistic regression analysis. RESULTS: Ninety episodes in 66 patients were recorded in our center. Hypertriglyceridemia occurred in 38% (34/90) of episodes. Twenty-five episodes were not cured in 90 episodes (27.8%, 25/90). The levels of thrombocytes, high-sensitivity C-reactive protein (hs-CRP), low-density lipoprotein cholesterol (LDL-C) and glycated hemoglobin, were higher in hypertriglyceridemia episodes of PDRP at baseline. The bacterial classification was different between elevated triglyceride group and normal triglyceride group. Adjusted for age, duration of dialysis, residual renal function, diabetes, thrombocytes, hs-CRP, serum albumin, cholesterol, HDL-C, LDL-C, intact parathyroid hormone (iPTH), glycated hemoglobin and spectrum of bacteria, hypertriglyceridemia were associated significantly with treatment failure of PDRP in our study (OR 3.416, 95% CI 1.223-9.540 p < 0.05). CONCLUSION: Hypertriglyceridemia at baseline was an independent risk factor for treatment failure of PDRP.

Achieving High-Quality Freshwater from a Self-Sustainable Integrated Solar Redox-Flow Desalination Device.

Solar-assisted electrochemical desalination has offered a new energy-water nexus technology for sustainable development in recent studies. However, only a few reports have demonstrated insufficient photocurrent, a low salt removal rate, and poor stability. In this study, a high-quality freshwater level of 5-10 ppm (from an initial feed of 10 000 ppm), an enhanced salt removal rate (217.8 µg cm-2 min-1 of NaCl), and improved cycling and long-term stability are achieved by integrating dye-sensitized solar cells (DSSCs) and redox-flow desalination (RFD) under light irradiation without additional electrical energy consumption. The DSSC redox electrolyte (I- /I3- ) is circulated between the photoanode (N719/TiO2 ) and intermediate electrode (graphite paper). Two DSSCs in parallel or series connections are directly coupled to the RFD device. Overall, this hybrid system can be used to boost photo electrochemical desalination technology. The energy-water nexus technology will open a new route for dual-role devices with photodesalination functions without energy consumption and solar-to-electricity generation.

Single-Nucleus Transcriptomic Analysis Reveals Important Cell Cross-Talk in Diabetic Kidney Disease.

Diabetic kidney disease (DKD) leads to the loss of renal function and cell cross-talk is one of the crucial mechanisms participating in the pathogenesis of DKD. However, the mechanisms of cell communication were not fully elucidated in previous studies. In this study, we performed cell cross-talk analysis using CellPhoneDB based on a single-nucleus transcriptomic dataset (GSE131882) and revealed the associations between cell communication-related genes and renal function, providing overall insight into cell communication in DKD. In addition, this study may facilitate the discovery of novel mechanisms, promising biomarkers, and therapeutic targets that are clinically beneficial to patients.

PD-L1 induces macrophage polarization toward the M2 phenotype via Erk/Akt/mTOR.

PD-L1 (programmed death-ligand 1) is the ligand of PD-1 (programmed cell death protein 1) and regulates inhibitory immune responses. It is well known that PD-L1 suppresses T cell function via binding to PD-1. However, little is known about the role of the PD-1/PD-L1 axis in macrophage polarization. According to previous studies, the function of the PD-1/PD-L1 axis in macrophage polarization is controversial, and the underlying mechanism has not been fully elucidated. Thus, we treated THP-1-derived macrophages with human PD-L1 Fc to determine the role of the PD-1/PD-L1 axis in macrophage polarization. To further explore the mechanism, we performed RNA sequencing and used specific inhibitors to identify the implicated signalling pathways. In this study, we found that PD-L1 induces the upregulation of CD206 expression, which is inhibited by nivolumab, LY294002, U0126, and rapamycin. Evaluation of differentially expressed genes (DEGs) and bioinformatics analysis indicated that PD-L1 also induces the upregulation of the expression of genes that maintain mitochondrial function and mediate metabolic switching. In addition, we did not detect PD-L1-induced CD86 alterations, indicating that PD-L1 treatment has no significant influence on M1 polarization. Taken together, these results suggest that PD-L1 binds to PD-1 and promotes M2 polarization accompanied by mitochondrial function enhancement and metabolic reprogramming via Erk/Akt/mTOR. This study elucidates the role of PD-L1 in macrophage polarization and verifies the underlying mechanisms for the first time. Considering that aberrantly upregulated PD-L1 expression contributes to a wide variety of diseases, targeting PD-L1-mediated macrophage polarization is a prospective therapeutic strategy for both neoplastic and nonneoplastic diseases.

Bi metal/oxygen-deficient BiO2-x with tetrahedral morphology and high photocatalytic activity.

Vacancy-rich materials with high photocatalytic activity are of great interest for pollutants removal and play a significant role in green chemistry. Herein, we successfully synthesized Bi/BiO2-x composite through hydrothermal route. In this case, the surface plasmon resonance effect of Bi and oxygen vacancies of BiO2-x collectively increase the removal rate of pollutants. More importantly, the Bi/BiO2-x composites have enhanced activity in the degradation of RhB, MO, BPA and CIP, and the reduction of Cr(VI) and PNA. Besides, an enhanced photocatalytic activity is due to the main reactive species of ·[Formula: see text] and h+ that is confirmed by trapping experiments and ESR analyses. The electronic structure and visible light harvesting of photocatalysts were measured and also theoretically calculated by using density functional theory and finite difference time domain calculations, DRS, VB x-ray photoelectron spectroscopy and Mott-Schottky plots, which allowed to propose a possible photocatalytic mechanism for the degradation process.

Prevalence, associated factors and cardiocerebral vascular prognosis of anaemia among patients on chronic haemodialysis in South Guangdong, China.

OBJECTIVE: To assess the prevalence, associated factors and cardiocerebral vascular prognosis of anaemia in patients undergoing haemodialysis. METHODS: This multicentre, retrospective, observational cohort study included patients on maintenance haemodialysis in South Guangdong, China. Anaemia in haemodialysis was defined as haemoglobin (Hb) <90 g/l. A proportion of patients were enrolled in a follow-up of the cardiocerebral vascular prognosis. RESULTS: A total of 1161 patients were enrolled and 938 were followed-up for cardiocerebral vascular events. Of 1161 patients, 250 (21.5%) had anaemia and 524 (45.1%) had an Hb level of 100-120 g/l. Adjusted multivariate logistic regression analysis demonstrated that frequency of dialysis ≤ twice weekly, hypoalbuminaemia and use of unfractionated heparin were independent factors associated with anaemia. Kaplan-Meier survival curve analysis for no myocardial infarction was 100%, 100%, 100% and 100% after 3, 6, 9 and 12 months, respectively, in patients with Hb < 90 g/l; compared with 97%, 95%, 93% and 93%, respectively, in patients with Hb ≥ 130 g/l. Adjusted Cox proportional hazards regression demonstrated that Hb ≥ 130 g/l was an independent risk factor for myocardial infarction. CONCLUSION: Anaemia is highly prevalent among patients undergoing haemodialysis in South Guangdong and requires careful management.

Self-Sustained Visible-Light-Driven Electrochemical Redox Desalination.

The freshwater scarcity and increasing energy demand are two challenging global issues. Herein, we propose a new route for desalination, self-sustained visible-light-driven electrochemical redox desalination. We propose a novel device architecture involving internal integration of a quasi-solid-state dye-sensitized solar cell and continuous redox-flow desalination units with a bifunctional platinized-graphite-paper electrode. Both the solar cell and redox-flow desalination units are integrated using the bifunctional electrode with one side facing the solar cell operating as a positive electrode and the other side facing the redox-flow desalination unit operating as a negative electrode. The solar cell contains a gel-based tri-iodide/iodide redox couple sandwiched between an N719 dye-modified photoanode and cathode. In contrast, the redox-flow desalination consists of re-circulating ferro/ferricyanide redox couple sandwiched between the anode and cathode with two salt streams located between these electrodes. The performances of bifunctional electrodes in both redox couples were thoroughly investigated by electrochemical characterization. The brackish feed can be continuously desalted to the freshwater level by utilizing visible light illumination. As a device, this architecture combines energy conversion and water desalination. This concept bypasses the need for electrical energy consumption for desalination, which provides a novel structural design using photodesalination to facilitate the development of self-sustained solar desalination technologies.

Zinc-Air Battery-Based Desalination Device.

Efficiently storing electricity generated from renewable resources and desalinating brackish water are both critical for realizing a sustainable society. Previously reported desalination batteries need to work in alternate desalination/salination modes and also require external energy inputs during desalination. Here, we demonstrate a novel zinc-air battery-based desalination device (ZABD), which can desalinate brackish water and supply energy simultaneously. The ZABD consists of a zinc anode with a flowing ZnCl2 anolyte stream, a brackish water stream, and an air cathode with a flowing NaCl catholyte stream, separated by an anion-exchange membrane and a cation-exchange membrane, respectively. During the discharging, ions in brackish water move to the anolyte and catholyte, and they return to the feed steam during charging. The ZABD can desalt brackish water from 3000 ppm to the drinking water level at 120.1 ppm in one step and concurrently provide an energy output up to 80.1 kJ mol-1 under a discharge current density of 0.25 mA cm-2. Further, the ZABD can be charged/discharged over 20 cycles without significant performance deterioration, demonstrating its reversibility. Moreover, the desalination performances can be adjusted by varying current densities and are also influenced by the initial concentration of salt feeds. Besides, two ZABD devices were connected in series to drive 60 light-emitting diodes during the salt removal process without external power supply over 2000 min. Overall, this ZABD system demonstrates the potential for simultaneous water desalination and energy supply, which is suitable for many urgent situations.

Flower-like Bi2SiO5/Bi4MoO9 heterostructures for enhanced photocatalytic degradation of ciprofloxacin.

Bi2SiO5/Bi4MoO9 photocatalysts with heterostructures were successfully prepared using a one-pot solvothermal route. The effect of the molybdenum source on composite formation is discussed. Under ultraviolet light irradiation, the Bi2SiO5/Bi4MoO9 heterojunction photocatalyst exhibited higher photocatalytic performance than Bi2SiO5 and Bi4MoO9 towards the degradation of ciprofloxacin (CIP). This dramatically enhanced photoactivity can be ascribed to the construction of a heterojunction interface between Bi2SiO5 and Bi4MoO9, which not only suppresses the recombination of photoexcited charge carriers but also enhances light absorption. In addition, from a practical point of view, the the effect of initial CIP concentration and coexisting ions on the photodegradation process using as-prepared Bi2SiO5/Bi4MoO9 heterojunction photocatalysts was explored. Trapping experiments demonstrate that photoexcited holes and superoxide radicals are the main active species in the photodegradation of CIP over Bi2SiO5/Bi4MoO9 heterojunctions. Meanwhile, the conduction band and valence band potentials of Bi2SiO5 and Bi4MoO9 were measured by density functional theory calculation, diffuse reflectance spectroscopy and Mott-Schottky curves. A possible photocatalytic mechanism for CIP degradation over the Bi2SiO5/Bi4MoO9 heterojunction is proposed.

Elevated baseline serum IgA may predict earlier proteinuria remission in IgA nephropathy patients.

The goals of this work were to investigate the correlations of elevated serum IgA with renal pathology and outcome of proteinuria in IgA nephropathy patients. Retrospective cohort analysis enrolled 90 IgA nephropathy patients (proteinuria ≥0.5 g/24 hr, estimated glomerular filtration rate (eGFR) ≥30 ml/min/1.73 m2) who were admitted to The Sixth Affiliated Hospital of Sun Yat-sen University from 2013.01 to 2017.04. The elevated serum IgA level was found in 20 (22.2%) patients. In clinical characteristics, serum IgG, ratio of IgA/C3 and recurrent mucosal infection rate were increased obviously in high serum IgA group compared with normal serum IgA group (serum IgG, 14.90±3.50 g/L vs. 10.27±3.49 g/L, P<0.001, IgA/C3, 4.45±1.21 vs. 2.77±0.75, P<0.001, recurrent mucosae infection rate, 40.0% vs. 14.3%, P=0.027). In kidney biopsy, mesangial proliferation was significantly more common in normal serum IgA group (81% vs. 50% in high serum IgA group, P=0.028). The proportion of crescent less than 25% more often occurred in elevated IgA group (81.3% vs. 63.8% in normal IgA group). The Kaplan-Meier curves showed that proteinuria remission rate for patients with high serum IgA was 80%, 85%, 90%, 95% and 95% after 3, 6, 9, 12 and 15 months compared with patients with normal serum IgA (proteinuria remission rate, 45%, 64%, 75%, 86% and 93%, P=0.020). Cox proportional hazard regression model indicated that elevated serum IgA (RR=1.984, P=0.040) and steroids therapy (RR=2.192, P=0.030) were independent predictors for proteinuria remission in IgA nephropathy patients. In view of our data, more active treatments may improve outcome of IgA nephropathy patients with elevated serum IgA. We conclude that elevated serum IgA may indicate a higher proteinuria remission rate within a shorter period of time in IgA nephropathy patients.

Relationship between MCP-1 promoter -2518 A/G gene polymorphism (rs1024611) and systemic lupus erythematosus/lupus nephritis.

Results from the published studies on the association between monocyte chemoattractant protein-1 (MCP-1) promoter -2518 A/G (rs1024611) gene polymorphism and systemic lupus erythematosus (SLE)/lupus nephritis (LN) are still conflicting. This meta-analysis was performed to evaluate the relationship between MCP-1 A/G gene polymorphism and SLE/LN and to explore whether MCP-1 A allele, AA genotype or GG genotype could become a predictive marker for SLE/LN risk. Association studies were identified from the databases of PubMed, Embase, Cochrane Library and CBM-disc (China Biological Medicine Database) as of 1 January 2014, and eligible investigations were synthesized using meta-analysis method. Results were expressed with odds ratios (OR) for dichotomous data, and 95% confidence intervals (CI) were also calculated. Sixteen investigations were identified for the analysis of association between MCP-1 A/G gene polymorphism and SLE, consisting of 2425 patients with SLE and 2567 controls. In the overall populations, Asians, Caucasian population, the association between MCP-1 A/G gene polymorphism and SLE susceptibility was not found. Interestingly, a trend toward an association between A allele/AA genotype and LN risk was observed in overall populations, although there was no statistical difference. However, this meta-analysis indicated that AA genotype was associated with LN risk in Caucasians (OR = 0.71; 95% CI: 0.54-0.93; p = 0.01). In conclusion, our results indicate that AA homozygous might be a significant genetic molecular marker to predict the SLE patients developing into LN in Caucasians. However, more investigations are required to further clarify this association.

Prevalence and risk factors of CKD in Chinese patients with periodontal disease.

BACKGROUND: Periodontal disease is common among adults and is associated with an increasing risk of chronic kidney disease (CKD). We aimed to investigate the prevalence and risk factors of CKD in patients with periodontal disease in China. METHODS: In the current cross-sectional study, patients with periodontal disease were included from Guangdong Provincial Stomatological Hospital between March 2011 and August 2011. CKD was defined as estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m(2), the presence of albuminuria, or hematuria. All patients with periodontal disease underwent a periodontal examination, including periodontal probing pocket depth, gingival recession, and clinical attachment level by Florida Probe. They completed a questionnaire and had blood and urine samples taken. The adjusted prevalence of indicators of kidney damage was calculated and risk factors associated with CKD were analyzed. RESULTS: A total of 1392 patients with periodontal disease were invited to participate this study and 1268 completed the survey and examination. After adjusting for age and sex, the prevalence of reduced eGFR, albuminuria, and hematuria was 2.7% (95% CI 1.7-3.7), 6.7% (95% CI 5.5-8.1) and 10.9% (95% CI 9.2-12.5), respectively. The adjusted prevalence of CKD was 18.2% (95% CI 16.2-20.3). Age, male, diabetes, hypertension, history of CKD, hyperuricemia, and interleukin-6 levels (≥7.54 ng/L) were independent risk factors for reduced eGFR. Female, diabetes, hypertension, history of CKD, hyperuricemia, high level of cholesterol, and high sensitivity C-reactive protein (hsCRP) (≥ 1.03 mg/L) and TNF-α levels (≥ 1.12 ng/L) were independently associated with an increased risk of albuminuria. Female, lower education (