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A digital library is a digital information resource system supported by modern high technology, a next-generation information resource management model on the Internet, and the result of the digitization of library collections, and with the development of society and the accelerated pace of people’s lives, people cannot spend too much time classifying and finding books, so the study of book classification and quick finding in university libraries is very important. This paper mainly researches and analyzes the classification and quick search of books in the university library through the algorithms and methods of digital information technology and finds a better algorithm. This paper mainly conducts experiments on automatic text and support vector machine (one-to-many and global optimization) methods and compares the obtained experimental data, such as classification accuracy, classification time, search time, and other data. The experimental results show that the classification accuracy of these three classification methods is in the range of 86%–94%. However, compared with the two methods of automatic text classification and one-to-many classification, the global optimization classification has the highest accuracy in the sample size of each interval. Among them, the classification time is the lowest for automatic text classification, which is less than 30s, and the one-to-many classification sample takes the most time, and their average fitness is in the range of 24%–27%.

This study systematically and comprehensively analyzed the characteristics of matrix metalloproteinases (MMPs) in gastric cancer (GC) and revealed the relationship between MMPs and prognoses, clinicopathological features, tumor microenvironment, gene mutations, and drug therapy response in patients with GC. Based on the mRNA expression profiles of 45 MMP-related genes in GC, we established a model that classified GC patients into three groups based on cluster analysis of the mRNA expression profiles. The 3 groups of GC patients showed significantly different prognoses as well as tumor microenvironmental characteristics. Next, we used Boruta’s algorithm and PCA method to establish an MMP scoring system and found that lower MMP scores were associated with better prognoses, lower clinical stages, better immune cell infiltration, lower degrees of immune dysfunction and rejection, and more genetic mutations. Whereas a high MMP score was the opposite. These observations were further validated with data from other datasets, showing the robustness of our MMP scoring system. Overall, MMP could be involved in the tumor microenvironment (TME), clinical features, and prognosis of GC. An in-depth study of MMP patterns can better understand the indispensable role of MMP in the development of GC and reasonably assess the survival prognosis, clinicopathological features, and drug efficacy of different patients, thus providing clinicians with a broader vision of GC progression and treatment.

Background This study investigated the association between urinary epidermal growth factor (EGF) and complete remission (CR) of proteinuria in children with IgA nephropathy (IgAN). Methods We included 108 patients from the Registry of IgA Nephropathy in Chinese Children. The urinary EGF at the baseline and follow-up were measured and normalized by urine creatinine (expressed as uEGF/Cr). The person-specific uEGF/Cr slopes were estimated using linear mixed-effects models for the subset of patients with longitudinal data of uEGF/Cr. Cox models were used to analyze the associations of baseline uEGF/Cr and uEGF/Cr slope with CR of proteinuria. Results Patients with high baseline uEGF/Cr were more likely to achieve CR of proteinuria (adjusted HR 2.24, 95% CI: 1.05–4.79). The addition of high baseline uEGF/Cr on the traditional parameters significantly improved the model fit for predicting CR of proteinuria. In the subset of patients with longitudinal data of uEGF/Cr, high uEGF/Cr slope was associated with a higher likelihood of CR of proteinuria (adjusted HR 4.03, 95% CI: 1.02–15.88). Conclusions Urinary EGF may be a useful noninvasive biomarker for predicting and monitoring CR of proteinuria in children with IgAN. Impact High levels of baseline uEGF/Cr (>21.45 ng/mg) could serve as an independent predictor for CR of proteinuria. The addition of baseline uEGF/Cr on the traditional clinical pathological parameters significantly improved the fitting ability for the prediction of CR of proteinuria. Longitudinal data of uEGF/Cr were also independently associated with CR of proteinuria. Our study provides evidence that urinary EGF may be a useful noninvasive biomarker in the prediction of CR of proteinuria as well as monitoring therapeutic response, thus guiding treatment strategies in clinical practice for children with IgAN.

Background Neutrophil extracellular traps (NETs) is the key means for neutrophils to resist bacterial invasion. Sepsis is a systemic inflammatory response syndrome caused by infection. Methods In our study, qRT-PCR was used to detect the gene expression in neutrophils, Western blot was used to detect the protein expression in mouse tissues and neutrophils, flow cytometry was used to detect the purity of neutrophils in the whole blood and immunofluorescence was used to detect the NETs formation. Results In this study, we analyzed the NETs formation in the blood of patients with sepsis. The results showed that a large number of NETs appeared. And the expression of GPR109A in neutrophils of patients with sepsis was significantly up regulated. Then we collected neutrophils from WT mice and GPR109A−/− mice and found that GPR109A knockout could significantly inhibit the early NETs formation of neutrophils. The results also showed that knockout of GPR109A or inhibition of the NETs formation could increase the inflammatory response of liver, spleen, lung and kidney in mice, thus affecting the disease process of sepsis. Then we observed the death of mice in 16 days. The results showed that inhibiting the NETs formation could significantly affect the early mortality of mice, while knocking out GPR109A could directly affect the mortality of the whole period. Conclusions This study confirmed the regulatory effect of GPR109A on early NETs formation for the first time, and provided a new target for the treatment of sepsis.

BackgroundPatients with diabetic kidney disease (DKD) often have gastrointestinal dysfunction such as inflammatory bowel disease (IBD). This study aims to investigate the genetic mechanism leading to IBD in DKD patients through data mining and bioinformatics analysis.MethodsThe disease-related genes of DKD and IBD were searched from the five databases of OMIM, GeneCards, PharmGkb, TTD, and DrugBank, and the intersection part of the two diseases were taken to obtain the risk genes of DKD complicated with IBD. A protein–protein interaction (PPI) network analysis was performed on risk genes, and three topological parameters of degree, betweenness, and closeness of nodes in the network were used to identify key risk genes. Finally, Gene Ontology (GO) analysis and Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis were performed on the risk genes to explore the related mechanism of DKD merging IBD.ResultsThis study identified 495 risk genes for DKD complicated with IBD. After constructing a protein–protein interaction network and screening for three times, six key risk genes were obtained, including matrix metalloproteinase 2 (MMP2), hepatocyte growth factor (HGF), fibroblast growth factor 2 (FGF2), interleukin (IL)-18, IL-13, and C–C motif chemokine ligand 5 (CCL5). Based on GO enrichment analysis, we found that DKD genes complicated with IBD were associated with 3,646 biological processes such as inflammatory response regulation, 121 cellular components such as cytoplasmic vesicles, and 276 molecular functions such as G-protein-coupled receptor binding. Based on KEGG enrichment analysis, we found that the risk genes of DKD combined with IBD were associated with 181 pathways, such as the PI3K-Akt signaling pathway, advanced glycation end product–receptor for AGE (AGE-RAGE) signaling pathway and hypoxia-inducible factor (HIF)-1 signaling pathway.ConclusionThere is a genetic mechanism for the complication of IBD in patients with CKD. Oxidative stress, chronic inflammatory response, and immune dysfunction were possible mechanisms for DKD complicated with IBD.

ObjectiveCblC deficiency, the most common cobalamin metabolic abnormality, is caused by pathogenic variants in the MMACHC gene. The renal complications of this disease have been described only in a small number of cases. This study aimed to better delineate renal phenotype and genetic characteristics in Chinese children with cblC defect.MethodsChildren with cblC deficiency who manifested as kidney damage were enrolled. Clinical, renal pathological, and genetic data were reviewed in detail.ResultsSeven cases were enrolled. Ages at disease onset ranged from 9 months to 5 years. All patients presented with hematuria and proteinuria, and 2/7 cases presented with nephrotic syndrome. Renal dysfunction was observed in 4/7 cases. Renal biopsy was performed in 5/7 cases, and all of them had renal thrombotic microangiopathy. Macrocytic anemia was detected in all seven patients. Six out of seven cases had hypertension, and 2/7 cases presented with pulmonary hypertension. Two of them had a mild intellectual disability, and one suffered from epilepsy. Increased urine methylmalonic acid and plasma homocysteine were detected in seven cases, while two patients had normal levels of urine methylmalonic acid at the initial evaluation. After diagnosis, all seven cases were treated with hydroxocobalamin IM. Six cases were followed-up for 3–8 years. After treatments, anemia was the first to be recovered, followed by proteinuria. Renal function recovered after 1 year in two cases, whereas patient 2 progressed to stage 2 chronic kidney disease 13 years after onset. While a case presented with end-stage kidney disease because of late diagnosis, one case died 3 months after disease onset due to giving up treatment. Three MMACHC pathogenic variants c.80A > G (8/14), c.609G > A (4/14), and c.658_660delAAG (2/14) were detected in all seven children.ConclusionMMACHC variant c.80A > G may be associated with prominent renal complications in Chinese cblC patients. Macrocytic anemia and hyperhomocysteinemia are useful clues for patients with hematuria and proteinuria caused by cblC defect. The most frequent renal pathological manifestation is thrombotic microangiopathy. Early diagnosis and treatment resulted in improving renal and hematological signs.

Salinity is regarded as the main environmental stress, which affects plant growth and physiological functions. miRNAs play crucial roles in plants salt stress response. Melilotus officinalis (L.) Pall. (MO) is an excellent forage and honey plant. In addition to its high medicinal values and outstanding ecological effects, MO also has the ability of salt tolerance. However, the miRNA expression mechanism of MO in response to salt stress is still unclear. To reveal the salt tolerance related miRNAs and predict their target genes in MO, we extracted a small RNA library from the roots of MO seedlings without salt treatment and used 300 mM NaCl treated roots of MO seedlings to construct another small RNA library. We identified 93 novel and 98 known miRNAs in control, 100 novel and 115 known miRNAs in case. 27 up-regulation and 20 down-regulated miRNAs were selected, 466 potential target miRNAs and 520 sites were predicted by differential expression analysis. 9 miRNAs were verified by quantitative reverse transcription polymerase chain reaction (qRT-PCR). Our findings summary the miRNAs and their targets for regulating salt defense reactions in MO. The discussion will be helpful to study the molecular mechanisms of salt resistance of MO and other Leguminosae species.

The effect of recombinant human GH (rhGH) in Chinese children with chronic kidney disease (CKD) is unclear.This was a 52-week, multicenter, randomized, open-label, negative-controlled phase 3 study. Prepubertal subjects were randomized 1:1 to either daily subcutaneous injections of rhGH 0.05 mg/kg/day or no treatment for 52 weeks.A total of 68 subjects with a mean age of 7.8 ± 3.27 years were enrolled. At week 52, the height standard deviation score (HT-SDS) in the treated group increased by 0.75 ± 0.58, which was significantly higher compared with 0.17 ± 0.47 in the untreated group (least squares mean 0.58, 95% confidence interval, 0.32-0.84; P 0.001). At week 52, significant improvements were observed in other growth parameters (height velocity [P 0.001]), insulin-like growth factor 1 (IGF-1) SDS [P 0.001], IFG-1/insulin-like growth factor binding protein-3 molar ratio [P 0.001], and height [P 0.001]) compared with the untreated control. Seven patients reported treatment-related adverse events (TRAEs) and most TRAEs were mild in severity. Most subjects recovered without further intervention.Daily rhGH for 52 weeks in children with CKD-induced growth retardation significantly improved HT-SDS and other growth parameters without compromising safety.The efficacy and safety of growth hormone (GH) therapy in Chinese children with chronic kidney disease (CKD) are unclear. This study found that giving short stature Chinese children with CKD daily recombinant human growth hormone (rhGH) for 52 weeks improved growth parameters without compromising safety. This study's information can give physicians the confidence to treat these patients in their clinical practice.

Photosystem II (PSII) of grapevine leaves is easily damaged under heat stress, but no such injury is observed when the leaves are heated in low light. To elucidate the mechanisms, we compared the photosynthetic characteristics of grapevine seedlings under heat treatments (42 °C) for 4 h in the dark or low light (200 μmol m−2 s−1). At 42 °C in the dark, the PSII maximum quantum yield (Fv/Fm) decreased significantly with the increase in time but did not change much in low light. The JIP (chlorophyll a fluorescence rise kinetics) test results showed that low light significantly alleviated the damage to the oxygen evolving complexes (OECs; the K-step was less visible) by heat stress. Further, in the presence of de novo D1 protein synthesis inhibitor chloramphenicol, Fv/Fm did not differ significantly between dark and light treatments under heat stress. The 50% re-reduction (RR50) of P700+ on cessation of far-red illumination was faster after light treatment than that in the dark. After exposure to 25 °C in a low light for 15 min, Y(NO) (the constitutive non-regulatory non-photochemical quenching) treated by heat stress and darkness was higher than that by heat stress and light. Overall, our results suggested that enhanced CEFs around PSI in low light could assist PSII against heat damage by maintaining the rate of PSII repair and inhibiting the non-radiative charge recombination in PSII reaction centers.

What is the water source for ecological restoration plants in arid region is still up to debate. To address this issue, we conducted an in-situ experiment in the Ulan Buh Desert of China. We selected Tamarisk, a common drought-salt-tolerance species in the desert for ecological restoration as our research subject, used a new designed lysimeter to monitor precipitation infiltration, a sap flow system to track reverse sap flow that occurred in shoot, branch, and stem during the precipitation event, and observed the precipitation redistribution process of the Tamarisk plot. The results showed that Tamarisk indeed directly absorb precipitation water, when precipitation occurs, the main stem, lateral branch, and shoot all show the signs of reversed sap flow, and the reversed sap flow accounted for 21.5 % of the annual sap flow in the shoot and branch, and 13.6 % in the stem. Precipitation event in desert was dominated by light precipitation events, which accounted for 81 % of the annual precipitation events. It was found that light precipitation can be directly absorbed by the Tamarisk leaves, especially in nighttime or cloudy days. Even when the precipitation is absent, it was found that desert plants can still absorb unsaturated atmospheric vapor, as reversed sap flow was observed when the atmospheric relative humidity reached 75 %. This study indicated that the effect of light precipitation on desert plants was significant and should not be overlooked in terms of managing the ecological and hydrological systems in arid regions.

Purpose Colorectal cancer is a high incidence tumor, immunotherapy has become a hot topic of research in recent years, at the same time, the dysregulation of intestinal microbiota is also gaining attention because it can lead to changes in body immunity. So, we want to explore the relationship between gut microbiota and colorectal cancer. Methods We performed 16S rRNA high-throughput sequencing of tumors and paraneoplastic tissues from 30 patients, followed by at least 5 years of follow-up. Microbiome analysis, survival analysis, and then immunohistochemistry and its correlation with microbiota were performed in 19 of these patients. Results OTU13530 and OTU17086, both representing g_Sediminibacterium, showed significant differences in normalized abundance in tumor and paraneoplastic tissues (FDR 2FC > 1). Both of them correlated with T-stage (p = 0.016, p = 0.024) and Her-2 expression (p = 0.034, p = 0.001). The former correlated with HIF expression (p = 0.017), and the latter with EGFR expression (p = 0.003). OTU3504, representing o_Myxococcales, had a correlation with patient survival (p = 0.015, HR = 28.5(1.9–425.0)), its abundance correlated with tumor neural invasion (p = 0.005) and vascular invasion (p = 0.005). Conclusion There is no statistical difference in the diversity and homogeneity of the microbiota between tumor and paraneoplastic tissues. But the abundance of some specific microbiota differed significantly between tumor and paraneoplastic tissues, and these microbiota correlated with patient survival, Her-2, HIF and other proteins expression, T-stage, neurovascular invasion and other clinical traits.

Colorectal cancer (CRC) is a common type of malignant digestive tract tumor with a high incidence rate worldwide. Currently, the clinical treatment of CRC predominantly include surgical resection, postoperative chemotherapy, and radiotherapy. However, these treatments contain severe limitations such as drug side effects, the risk of recurrence and drug resistance. Some natural compounds found in plants, fungi, marine animals, and bacteria have been shown to inhibit the occurrence and development of CRC. Although the explicit molecular mechanisms underlying the therapeutic effects of these compounds on CRC are not clear, classical signaling transduction pathways such as NF-kB and Wnt/β-catenin are extensively regulated. In this review, we have summarized the specific mechanisms regulating the inhibition and development of CRC by various types of natural compounds through nine signaling pathways, and explored the potential therapeutic values of these natural compounds in the clinical treatment of CRC.

Understanding soil seed banks (SSBs) of Nitrariatangutorum nebkhas is critical for vegetation restoration and ecological management in desert–oasis ecotones. In this study, we conducted a survey in the field and a seed germination experiment in the laboratory to investigate the characteristics of SSB in different sizes and parts of nebkhas and to examine their relationship with aboveground vegetation. The results showed that there were 17 species of plants in six families in the SSB of Nitraria tangutorum nebkhas in a desert–oasis ecotone, dominated by Chenopodiaceae. The life forms were mainly annual herbs, accounting for 58.82%–71.43%. The number of species in the SSB were in the order of large nebkha > medium nebkha > small nebkha, and there was no significant difference in SSB density. However, SSB densities in different parts of each nebkha were in the order bottom > middle > top. The densities were significantly different and showed a decreasing trend as soil depth increased. Most of the plant seeds were distributed in the 0–5 cm soil layer. The similarity coefficient between the SSB of each nebkha and the aboveground vegetation was high. From the bottom to the top, the number of common species and similarity coefficient between the aboveground vegetation and the SSB decreased, and the number of species in the SSB was greater than that of the aboveground vegetation. Vegetation plays a decisive role in the initial development of a nebkha. When the nebkha develops to a certain extent, it reacts to the vegetation, thereby intensifying the differences in the composition of vegetation species in different parts of the nebkha and further affecting the SSB.

Knowledge of the microclimate and wind regimes of different land landscapes can be used to support natural resource development and agricultural productivity in desert areas. The aim of this study is to provide a theoretical basis and technical support for the management of Ulan Buh Desert Oasis and the comprehensive prevention and control of sandstorms from the surrounding desert in northern China. We examined the microclimate and wind regime of three landscapes—desert, desert–oasis ecotone, and oasis—and discussed the factors underlying the observed variations. Similar patterns were found in annual temperature, annual relative humidity, and wind speed between the desert, desert–oasis, and the oasis. Compared with the desert and the desert–oasis ecotone, the average annual temperature and wind speed in the oasis were lower by 2.56%–5.38% and 32.99%–37.05%, respectively. Average annual relative humidity in the oasis was higher than in other areas by 1.31%–2.57%. The desert, desert–oasis ecotone, and the oasis were dominated by westerly winds. Wind direction was relatively stable in the oasis and variable in the desert and desert–oasis ecotone. There were intermediate wind energy fields in the desert and the desert–oasis ecotone and a low wind energy field in the oasis. In the desert–oasis ecotone, drift potential (DP), resultant drift potential (RDP), and resultant drift direction (RDD) were 231.87 VU, 97.21 VU, and 104.33°, respectively. In the desert, DP was 228.45 VU, RDP was 92.65 VU, and RDD was 76.05°. In the oasis, the DP was 61.85 VU, RDP was 38.04 VU, and RDD was 92.97°. In the desert and the desert–oasis ecotone, sediment transport potential was the highest in spring, second highest in autumn, third highest in winter, and lowest in summer. In the oasis, it was the highest in winter, second highest in autumn, third highest in spring, and lowest in summer. There are complex variations in the dominant sediment transport directions in the desert and desert–oasis ecotone in the study area. Variations in the dominant sediment transport direction in the oasis are less complicated and are associated with the seasons. The oasis is primarily threatened by sandstorms from the south-southeast in spring.

Colorectal cancer (CRC) is a common type of malignant digestive tract tumor with a high incidence rate worldwide. Currently, the clinical treatment of CRC predominantly include surgical resection, postoperative chemotherapy, and radiotherapy. However, these treatments contain severe limitations such as drug side effects, the risk of recurrence and drug resistance. Some natural compounds found in plants, fungi, marine animals, and bacteria have been shown to inhibit the occurrence and development of CRC. Although the explicit molecular mechanisms underlying the therapeutic effects of these compounds on CRC are not clear, classical signaling transduction pathways such as NF-kB and Wnt/β-catenin are extensively regulated. In this review, we have summarized the specific mechanisms regulating the inhibition and development of CRC by various types of natural compounds through nine signaling pathways, and explored the potential therapeutic values of these natural compounds in the clinical treatment of CRC.

The prognosis of acute kidney injury (AKI) varies in children with nephrotic syndrome (NS), data on factors predicting the recovery and recurrence of AKI in children with NS are limited. This study aimed to explore the possible factors predicting the recovery from and recurrence of AKI in children with primary NS. Children with primary NS complicated with AKI from 1993 to 2017 in a single centre were reviewed retrospectively. The clinical pictures and possible factors predicting the recovery from and recurrence of AKI in children with primary NS were investigated. Sixty-eight episodes of AKI in 59 children with NS were analysed: 88.2% of AKI recovered within 3 months, and 2.9% of AKI did not recover after 3 months. Survival analysis revealed that leucocyturia is significantly related to the AKI recovery time (P = 0.001), and children with leucocyturia [22 (4, 79) days] recovered significantly slower than did children without leucocyturia [12.0 (2, 39) days]. Renal tubular and interstitial injury were prominent in children with leucocyturia, and 11.9% of children with index AKI experienced the recurrence of AKI. Most episodes of AKI that occurred in children with NS recovered completely. Leucocyturia is a significant factor predicting the recovery time of AKI.

To explore the genetic etiology and clinical outcome of a child with steroid-resistant nephrotic syndrome and diffuse mesangial sclerosis.Genomic DNA was extracted from peripheral blood leukocytes of the proband and his parents. Targeted capture - next generation sequencing and Sanger sequencing were carried out. Candidate variant was verified by segregation analysis in his family.A heterozygous missense variant of the TRPC6 gene, namely c.325GA (p.Gly109Ser), was detected in the proband. The same variant was not detected in either parent. According to the guidelines for the interpretation of sequence variants developed by American College of Medical Genetics and Genomics, the variant was predicted as pathogenic.The missense variant of the TRPC6 gene probably underlay the diffuse mesangial sclerosis in this patient. Above finding has expanded the phenotypic spectrum of the TRPC6 gene.

To detect the expression of HER-2 and P53 patients with gastric cancer and to analyze their correlation.A total of 249 gastric cancer patients with complete clinical data who received surgical treatment from China-Japan Union Hospital of Jilin University were selected. The expression of Her-2 and P53 were detected by immunohistochemistry using the streptavidin-biotin-peroxidase method. The correlations between HER-2 and P53 in gastric cancer were analyzed.The positive rate of Her-2 and P53 expression was 37.3% (93/249) and 100% in all the specimens, respectively. The intensity of Her-2 expression was significantly different in patients with different degrees of gastric cancer cell differentiation (P = 0.012). Meanwhile, the expression of her-2 was closely related to whether the pathological type of gastric cancer was a signet-ring cell carcinoma (P = 0.022). Different percentage of positive P53 expression was closely related to the grade of tumor differentiation (P = 0.035) and positive Ki67 expression (P = 0.001). There was a significant positive correlation between HER-2 and P53 expression in gastric cancer (P = 0.003). These findings suggest that HER-2 and P53 have synergistic effects in gastric cancer.Her-2 and P53 are important markers for invasion and metastasis of gastric cancer. Combined detection of P53 and Her-2 expression in gastric cancer tissue can be used to assess prognosis and screen cancer patients at high risk of metastasis.

Purpose: Adolescents are easily attacked by potential inherited metabolic disorders. cblC deficiency is the most common type of methylmalonic aciduria in China. The late-onset patients present with varied non-specific symptoms and usually being misdiagnosed. The purpose of this study is to investigate the clinical features of patients with adolescence-onset cblC deficiency and explore the prevention and control strategies. Methods: Fifty-seven patients (34 males and 23 females) with adolescence-onset cblC deficiency were admitted in our clinic from 2002 to September 2021. The diagnosis was confirmed by metabolic and genetic tests. The clinical and biochemical features, disease triggers, outcome and genotypes-phenotypes correlation were examined.Results: The onset ages ranged from 10 to 25 years old (median age was 12 years). 16 cases (28.0%) presented with symptoms after infection or sports training. 46 patients (80.7%) had neuropsychiatric diseases. 14 patients (24.6%) displayed cardiovascular diseases. Five cases (8.9%) showed pulmonary hypertension. Renal damage was observed in seven cases (12.3%). 23 mutations were identified from the MMACHC gene of 57 patients. 37 patients demonstrated c.482G>A (64.9%) and 16 cases had c.609G>A (26.3%). Among 13 patients that exhibited spastic paraplegia as a main manifestation, 10 patients had c.482G>A (76.9%). Five patients presented with psychotic disorders and spastic paraplegia with c.482G>A. All patients improved after metabolic treatment with cobalamin, L-carnitine, and betaine. 30 school-aged patients returned to school. Two patients were married and had healthy babies.Conclusion: Patients with adolescence-onset cblC deficiency presented with varied neuropsychiatric symptoms or multiple organ damage. Metabolic studies and individualized treatment are keys to improve the outcome of the patients.

Complexity of forest structure is an important factor contributing to uncertainty in aboveground biomass estimates. In this study, we present a new method for reducing uncertainty in forest aboveground biomass (AGB) estimation based on plot-level terrestrial laser scanner (TLS) point clouds reconstruction. The method estimates the total AGB of plots with complex structures after automatically performing the steps of ground point filtering, single tree segmentation, and three-dimensional (3D) structure reconstruction. We used plot data from temperate and tropical forest ecosystems to verify the effectiveness of the method, reconstructing a 1300 m2 temperate plantation plot and a 5000 m2 mingled forest plot, respectively. The total biomass of 153 trees in the plantation plot was overestimated by 17.12 %, and the total biomass of 61 trees in the mingled forest plot was underestimated by 10.88 %. We found that the uncertainty of aboveground biomass estimation in tropical forests with more complex structures is not necessarily greater than in plantations. Therefore, in large-scale remote sensing observations of forest biomass, the number or area of plots can be increased to reduce the uncertainty of the results caused by the complex structure. The focus of this study is to explore TLS point clouds modeling methods to reduce the uncertainty in AGB estimation caused by the complexity of forest structures, and to provide reference cases for plot-level point clouds reconstruction methods. Forest ecologists can use this method to regularly observe forest growth and obtain indicators related to forest ecology without destroying trees.

Background cblC deficiency is the most common type of methylmalonic aciduria in China. Late-onset patients present with various non-specific symptoms and are usually misdiagnosed. The purpose of this study is to investigate the clinical features of patients with late-onset cblC deficiency and explore diagnosis and management strategies around puberty. Results This study included 56 patients (35 males and 21 females) with late-onset cblC deficiency who were admitted to our clinic between 2002 and September 2021. The diagnosis was confirmed by metabolic and genetic tests. The clinical and biochemical features, disease triggers, outcome, and associated genetic variants were examined. The onset age ranged from 10 to 20 years (median age, 12 years). Fifteen patients (26.8%) presented with symptoms after infection or sports training. Further, 46 patients (82.1%) had neuropsychiatric diseases; 11 patients (19.6%), cardiovascular diseases; and 6 patients (10.7%), pulmonary hypertension. Renal damage was observed in 6 cases (10.7%). Genetic analysis revealed 21 variants of the MMACHC gene in the 56 patients. The top five common variants detected in 112 alleles were c.482G > A (36.6%), c.609G > A (16.1%), c.658_660delAAG (9.8%), c.80A > G (8.0%), and c.567dupT (6.3%). Thirty-nine patients carried the c.482G > A variant. Among 13 patients who exhibited spastic paraplegia as the main manifestation, 11 patients carried c.482G > A variants. Six patients who presented with psychotic disorders and spastic paraplegia had compound heterozygotic c.482G > A and other variants. All the patients showed improvement after metabolic treatment with cobalamin, l-carnitine, and betaine, and 30 school-aged patients returned to school. Two female patients got married and had healthy babies. Conclusions Patients with late-onset cblC deficiency present with a wide variety of neuropsychiatric symptoms and other presentations, including multiple organ damage. As a result, cb1C deficiency can easily be misdiagnosed as other conditions. Metabolic and genetic studies are important for accurate diagnosis, and metabolic treatment with cobalamin, l-carnitine, and betaine appears to be beneficial.

A. fruticosa (Amorpha fruticosa L.) is widely used for revegetation in semiarid lands that undergo secondary salinization. Understanding A. fruticosa plants response to soil water and salt stress is essential for water irrigation management and proper revegetation practices. In this study, we measured sap flow, stomatal conductance, meteorological and soil characteristics in an A. fruticosa community that recently experienced secondary salinization in northwestern China. Results of our study showed that daytime and nocturnal sap flows averaged 804.37 g·cm−2·day−1 and 46.06 g·cm−2·day−1, respectively, during the growing season. Within individual days, the highest sap flow appeared around noon local time and followed a similar pattern of photosynthetically active radiation (PAR). Despite the significant effect of meteorological factors on the characteristics of sap flow, our study highlighted that the sap flow of A. fruticosa is strongly regulated by the availability of soil relative extractable water (REW). The daytime sap flow, which is predominant compared to nocturnal sap flow, was strongly affected by PAR, air temperature and vapor-pressure deficit. With water stress in the top 40 cm of the soil (REW0–40 cm SWC) (positive) and soil electrical conductivity (EC) (negative) in the relatively shallow soil profile (up to 40 cm). For the nocturnal sap flow, our results suggest that in the absence of soil water stress (REW0–40 cm > 0.4), the nocturnal sap flow is mainly used to replenish the stem water content and sustain nocturnal transpiration. Under soil water stress, nocturnal sap flow is mainly used to replenish stem water content. The results of our study indicate that it is necessary to shorten the irrigation cycle during the primary growing period (May–July) of A. fruticosa. Moreover, in the absence of soil water stress (REW0–40 cm > 0.4), A. fruticosa can survive well in an saline environment with soil EC −1.

The aim of this study is to clarify the association between lymphovascular invasion (LVI) and/or perineural invasion (PNI) and the clinical characteristics and prognostic importance of rectal cancer, to provide a basis for early adjuvant treatment of rectal cancer. We retrospectively analyzed patients diagnosed with rectal cancer. This study involved rectal cancer tissue samples were obtained by surgical methods. Data on histological form, tumor classification, tumor size, gross growth pattern, blood and lymphatic vessel invasion, and PNI of the slice by HE staining were obtained from pathological examination. Immunohistochemical analysis of tissue samples was performed to determine p53 and EGFR expressions. There were 330 rectal cancer patients included in the study. LVI and/or PNI can be used as a high-risk factor for the prognosis of rectal cancer, predict prognostic survival, and guide adjuvant therapy. The detection rates of LVI and PNI were 32.1% and 16.1%. Differentiation grade, Union for International Cancer Control staging, tumor-lymph node-metastasis staging are significantly related to LVI or PNI. Multivariate logistic regression analysis shows that poor differentiation and N ≥ 1 can be used as independent risk factors and predictive factors for LVI. At the same time, poor differentiation and T 3 is an independent risk factor for PNI. Only poor differentiation is the risk factor for poor prognosis in Cox risk regression analysis. In addition, the simultaneous occurrence of LVI and PNI is an independent prognostic factor.

Background Both Pierson syndrome (PS) and isolated nephrotic syndrome can be caused by LAMB2 biallelic pathogenic variants. Only 15 causative splicing variants in the LAMB2 gene have been reported. However, the pathogenicity of most of these variants has not been verified, which may lead to incorrect interpretation of the functional consequence of these variants. Methods Using high‐throughput DNA sequencing and Sanger sequencing, we detected variants in a female with clinically suspected PS. A minigene splicing assay was performed to assess the effect of LAMB2 intron 20 c.2885‐9C>A on RNA splicing. We also performed the immunohistochemical analysis of laminin beta‐2 in kidney tissues. Results Two novel LAMB2 heteroallelic variants were found: a paternally inherited variant c.2885‐9C>A in intron 20 and a maternally inherited variant c. 3658C>T (p. (Gln1220Ter)). In vitro minigene assay showed that the variant c.2885‐9C>A caused erroneous integration of a 7 bp sequence into intron 20. Immunohistochemical analysis revealed the absence of glomerular expression of laminin beta‐2, the protein encoded by LAMB2. Conclusion We demonstrated the impact of a novel LAMB2 intronic variant on RNA splicing using the minigene assay firstly. Our results extend the mutational spectrum of LAMB2., We demonstrated the impact of a novel LAMB2 intronic variant on RNA splicing using the minigene assay firstly. Our results extend the mutational spectrum of LAMB2.

Renal hypodysplasia and cystic kidney diseases, the common non-glomerular causes of pediatric chronic kidney disease (CKD), are usually diagnosed by their clinical and imaging characteristics. The high degree of phenotypic heterogeneity, in both conditions, makes the correct final diagnosis dependent on genetic testing. It is not clear, however, whether the frequencies of damaged alleles vary among different ethnicities in children with non-glomerular CKD, and this will influence the strategy used for genetic testing. In this study, 69 unrelated children (40 boys, 29 girls) of predominantly Han Chinese ethnicity with stage 2–5 non-glomerular CKD caused by suspected renal hypodysplasia or cystic kidney diseases were enrolled and assessed by molecular analysis using proband-only targeted exome sequencing and array-comparative genomic hybridization. Targeted exome sequencing discovered genetic etiologies in 33 patients (47.8%) covering 10 distinct genetic disorders. The clinical diagnoses in 13/48 patients (27.1%) with suspected renal hypodysplasia were confirmed, and two patients were reclassified carrying mutations in nephronophthisis (NPHP) genes. The clinical diagnoses in 16/20 patients (80%) with suspected cystic kidney diseases were confirmed, and one patient was reclassified as carrying a deletion in the hepatocyte nuclear factor-1-beta gene (HNF1B). The diagnosis of one patient with unknown non-glomerular disease was elucidated. No copy number variations were identified in the 20 patients with negative targeted exome sequencing results. NPHP genes were the most common disease-causing genes in the patients with disease onsets above 6 years of age (14/45, 31.1%). The children with stage 2 and 3 CKD at onset were found to carry causative mutations in paired box gene 2 (PAX2) and HNF1B gene (11/24, 45.8%), whereas those with stage 4 and 5 CKD mostly carried causative mutations in NPHP genes (19/45, 42.2%). The causative genes were not suspected by the kidney imaging patterns at disease onset. Thus, our data show that in Chinese children with non-glomerular renal dysfunction caused by renal hypodysplasia and cystic kidney diseases, the common causative genes vary with age and CKD stage at disease onset. These findings have the potential to improve management and genetic counseling of these diseases in clinical practice.

The study aims to reveal the clinical significance of perineural invasion (PNI) for gastric cancer prognosis and determine the risk factors of PNI in gastric cancer. This study retrospectively analyzed 350 patients who were diagnosed with GC and underwent curative surgical resection. Variables used to analyze survival included gender, age, degree of differentiation, T classification, lymph node metastasis, lymphovascular invasion, nerve invasion, mucinous adenocarcinoma component, and signet ring cell carcinoma component. The tumors of all patients were surgically resected. All resected specimens were stained with hematoxylin-eosin and immunohistochemical. The data for the patient's lymphovascular invasion and PNI came from the collected pathological reports. The results of the survival analysis showed that T staging (P.001), lymph node metastasis (P.001), lymphovascular invasion (P = .013), PNI (P = .001), and signet ring cell carcinoma components (P = .046) affect the survival time and have a statistically significant difference. Multivariate analysis indicated that the positivity of PNI was an independent prognostic factor (P = .014). T staging (P = .006) and lymph node metastasis (P = .013) were independent prognostic parameters too. Using the Spearman correlation analysis, the following clinicopathological indicators were associated with PNI positivity, such as tumor differentiation, T staging, lymph node metastasis, vascular invasion, and signet ring cell carcinoma components (P.05). PNI is an independent marker of poor prognosis in patients with gastric cancer.

Northwest China is significantly affected by sandstorm disasters. To mitigate the negative impacts of sandstorm events, it is critical to understand the spatio-temporal variations in typical sand and dust storms and their influencing factors. In this work, using ground-based measurements of particulate matter and remote sensing data such as MODIS, OMI, and CALIPSO data, the sources of aerosol pollution and aerosol optical properties of a typical sandstorm event that occurred in Northwest China in 2018 was studied. In addition, the HYSPLIT model was used to explore the air mass trajectories in order to analyze the sand and dust migration process during the sandstorm event. Furthermore, the wind erosion sensitivity of Northwest China was analyzed via single factor analysis and multi-factor superposition of wind field intensity, soil drought index, vegetation coverage, and relief amplitude. Finally, the region of the study area having a high comprehensive wind erosion sensitivity was identified. The results showed that the PM10 concentrations exceeded 400 µg/m3 and the PM2.5/PM10 ratio did not exceeded 0.6 during the sandstorm event, indicating that natural particulate matter was dominant in the ambient air. At the epicenter of pollution, the aerosol optical depth (AOD) at 550 nm was 0.75–1. By combining AOD data with wind speed and direction data from field observation stations, it was found that the sandstorm event in 2018 mainly occurred between 1 April and 3 April, and affected all of Northwest China on 2 April and 3 April. The absorbed aerosol index (AAI) ranged between 2.5 and 4, indicating that the Taklimakan Desert was the main source of sandstorm events in Northwest China. The CALIPSO total attenuated backscatter coefficient at 532 nm indicated that the main component of tropospheric aerosol in this region was distributed in the range of 0–12.5 km. The simulated airflow track showed that it had the same dust source regions as AAI index studies. Moreover, investigation of wind erosion sensitivity in the study areas indicated that the Taklimakan Desert and other desert regions were the main ecologically sensitive areas. These conclusions can provide references and suggestions for the mitigation of damage caused by sandstorm events, in addition to the enhancement of ecological governance.

Purpose: Vesicoureteral reflux (VUR) is a risk factor for various renal problems like recurrent urinary tract infections (UTIs), pyelonephritis, renal scarring, hypertension, and other renal parenchymal defects. The interventions followed by pediatricians include low-dose antibiotic treatment, surgical correction, and endoscopy. This meta-analysis aimed to assess the advantages and drawbacks of various primary VUR treatment options. Search Strategy: The Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, reference lists of journals, and abstracts from conference proceedings were all used to find randomized controlled trials. The articles were retrieved from 1985 till 2020. Twenty articles were used for the data analysis. Criteria for Selection: Surgery, long-term antibiotic prophylaxis, noninvasive techniques, and any mix of therapies are also options for treating VUR. Collection and Interpretation of Data: Two authors searched the literature separately, determining research qualifications, assessing accuracy, and extracting and entering results. The odds ratio (OR) of these studies was used to construct the forest plot. The random-effects model was used to pool the data. Also, the random-effects model was used with statistical significance at a p value < 0.05 to assess the difference in side effects after treatment of VUR using different modalities. Results: We found no statistically significant differences between surgery plus antibiotics and antibiotic alone-treated patients in terms of recurrent UTIs (OR = 0.581; 95% confidence interval [CI] 0.259–1.30), renal parenchymal defects (OR = 1.149; 95% CI 0.75–1.754), and renal scarring (OR = 1.042; 95% CI 0.72–1.50). However, the risk of developing pyelonephritis after surgical treatment of VUR was lesser than that in the conservative approach, that is, antibiotics (OR = 0.345; 95% CI 0.126–0.946.), positive urine culture (OR = 0.617; 95% CI 0.428–0.890), and recurrent UTIs were more common in the placebo group than in the antibiotic group (p < 0.05; OR = 0.639; 95% CI 0.436–0.936) which is statistically significant. Conclusion: Based on current research, we recommend that a child with a UTI and significant VUR be treated conservatively at first, with surgical care reserved for children who have issues with antimicrobials or have clinically significant VUR that persists after several years of follow-up.

Background: The coronavirus disease 2019 (COVID-19) pandemic has posed a great challenge to the healthcare system. This study evaluated the impact of the pandemic on the utilization of primary healthcare (PHC). Methods: The outpatient data from 158 PHC institutions in Yinchuan from May 1, 2017 to April 30, 2020 were used. The difference in difference (DID) model was used to analyze the difference in the number of outpatient visits per day, total outpatient expenditure per day, and outpatient expenditure per visit between December 2019 and February 2020 compared with the same periods in two previous years. The autoregressive integrated moving average (ARIMA) modelling was used to investigate the association between the outpatient volume and the number of the last week’s new COVID-19 cases in Yinchuan, Ningxia, and China. Results: From December 2019 to February 2020, the decline in the number of outpatient visits per day (DID: -367.21 times, P=.004) was larger than that in two previous years, and a similar trend can be seen in the outpatient expenditure per day. However, the rise in the outpatient expenditure per visit (DID: 19.06 thousand yuan, P=.003) was larger than that in two previous years. In 2020, the outpatient visits for most types of diseases decreased from week 3 and rebounded after week 5. The decline and rebound of outpatient visits in the population aged 45 years and older were steeper than in those younger. The outpatient volume was negatively associated with the number of the last week’s new COVID-19 cases. Conclusion: This study indicated a significant impact of the pandemic on PHC service utilization. Since PHC service is the foundation of the healthcare system in most developing countries, measures should be taken to make PHC help cope with the crisis and relieve the burden of hospital care.

Shelterbelts, also termed windbreaks, play a significant role in reducing soil erosion, sand drift, and protecting crops, livestock and farmstead. Cropland shelterbelts are known to improve the microclimate and provide protection against sand-related damages to crops. However, the protection of shelterbelts to crops may be complicated by fine roots near the edge of the shelterbelt, which directly affects the absorption of soil water and the accumulation of soil organic carbon. In this study, we investigated the effects of shelterbelt fine roots on farmland soil water content (SWC) and soil organic carbon density in an agricultural system located in northern China. The distribution characteristics of fine-root biomass density, soil water content and soil organic carbon density were measured at the 0-200 cm soil depth in a farmland shelterbelt system at distances of 0.3H (H is the windbreak height), 0.5H, and 0.7H from the shelterbelt with three replicates. Soil samples were randomly collected in the center of the cropland. The results showed that fine roots of shelterbelts concentrated at the depth of the 20-60 cm soil layer, whereas the fine roots of the crop summer squash (Cucurbita pepo L.) were mainly found within the top 20 cm of the soil profile. With the presence of shelterbelt fine roots, strong SWC deficiency that occurred up to100 cm in the soil profile was observed. The SWC was deficient at all sampling points, the overall shallow layer deficient was larger than the deep layer. In contrast, the soil organic carbon was cumulative, and the overall deep accumulation was greater than the shallow layer. The effects of fine roots on SWC and soil organic carbon density variations in different soil layers were inconsistent. The information accrued in this study can be used to evaluate the effect of farmland shelterbelt on soil water and soil carbon in the Hetao irrigation area of Inner Mongolia, northern China.

The windbreak system is a major component of successful agricultural systems in arid deserts throughout the world. Ulan Buh Desert is one of the eight biggest deserts in China, and the oases there offer residence and cropland for over 90% of the local residents. However, due to climate change and human disturbances, the Ulan Buh Desert continues spreading to the south, bringing more pressure on the windbreak systems there. Meanwhile, the Chinese government put much effort into greening the desert, establishing artificial shrubs to prevent dune movement and soil loss. How microclimate in the cropland-windbreak-desert system responded to human activities and climate change has rarely been studied. In this study, we investigated the microclimate change dynamics across the cropland-windbreak-desert transition zone during the past 38 years. Two 50 m climatological towers, located in the same distance inner and outside a shelterbelt, have continuously monitored climatic factors, including air temperature, soil temperature, relative humidity, precipitation, evaporation, layered wind speeds, etc., and aeolian erosion related factors, such as layered dustfall. The long-time fluctuations of the inside and outside climatic factors have been analyzed, and the global climate change data, local land-use history, as well as the record of afforestation activities implemented by government and local people, were also collected. The results revealed that both the inside and outside windbreak air temperatures and soil temperatures increased during the past 38 years, which agrees with the global warming phenomenon. The inner windbreak air temperature is consistently lower than the outer windbreak areas, and the temperature difference is biggest in summer and smallest in winter. However, the soil temperature difference between the outside and inner windbreak is unstable. In 1995, 2002, and 2004, the dune areas even had lower soil temperature than the inner cropland. The precipitation is 0.5~100.7mm higher in cropland and the evaporation is lower in cropland when comparing to outside dune areas, but their annual variations changed greatly. The wind speed and erosion rate are significantly lower in cropland than desert dune areas, and the seasonal change exhibited a bimodal curve pattern. The results suggest that the cropland-windbreak-desert transition zone responded to global climate change simultaneously. Although the shelterbelt still creates a favorable regional climatic condition for the cropland, the differences between the inner and outer windbreak areas narrowed during the past 10 years. The aeolian erosion rate reduced significantly in outside windbreak dune areas, which may largely attribute to the artificial Haloxylon ammodendron communities planted at the southeastern margin of the desert.

The inappropriate selection of measurement points and measurement times in an ecosystem may easily lead to the underestimation or overestimation of soil respiration due to spatial and temporal heterogeneity. To assess the law of spatial and temporal heterogeneity and more accurately determine the soil respiration rate, we measured the soil respiration rate of a forest in the plant growing season from 2011 to 2013 on Changbai Mountain in 8 directions and 7 distances from each tree trunk. Neglecting the direction of the measuring point may overestimate or underestimate the soil respiration rate by 29.81% and 26.09%, respectively, neglecting the distance may overestimate or underestimate the soil respiration rate by 41.36% and 20.28%, respectively, and ignoring the measurement time may overestimate and underestimate the soil respiration rate by 41.71% and 57.64%, respectively. In addition, choosing a measurement point in the eastern direction at a 1.8 m distance and conducting the measurement in September may relatively accurately reflect the soil respiration rate of the ecosystem. These findings can deepen our understanding of soil respiration rate heterogeneity and may provide new ideas for improving the measurement method of soil respiration.

Water, nutrient and light deficiency during the co-growth period are the major factors limiting the productivity of the apple–maize alley cropping system in Loess Plateau, China. Therefore, it is crucial to optimize the management of water and fertilizer in the region. The effects of different irrigation and fertilizer levels on photosynthetic characteristics, growth, yield, water consumption (ET) and water use efficiency were studied in a young apple-maize intercropping system during 2014–2016. The treatments included three levels of maximum irrigation, namely 50% (W1), 65% (W2), and 85% (W3) of field capacity (Fc), and three different NPK fertilization levels (289.0-118.0-118.0, 412.4-168.8-168.8 and 537.0-219.0-219.0 kg∙ha−1), which were designated F1, F2 and F3, respectively. In addition, the rain-fed (neither irrigation nor fertilizer) crop was included as the control (CK). The F1 significantly increased the net rate of photosynthesis (Pn) and leaf water-use efficiency (LWUE) of both apple and maize. For maize, the effect of irrigation was greater than that of fertilizers on water use, but smaller on growth indicators and yield. The ET of the alley cropping systems increased with the amount of irrigation but decreased as the dose of fertilizers increased. Growth and yield of maize increased when the amount of irrigation increased, but were inversely related to fertilizer dose. The canopy architecture appeared to be regulated through differential absorption of water and nutrients. The Pn and transpiration (Tr) in maize decreased as the distance from the tree line increased, and the extent of decrease grew over time, suggesting greater interspecific competition. It is recommended that dwarf crops should be planted after intercropping for 5 years according to the change in photosynthetic index with intercropping years under different treatments. Multiple regression analysis indicated the following regime to be optimal during the first 3–5 years of the intercropping in terms of the yield of maize and water use in the alley cropping system: maximum watering (85% Fc) and minimum fertilization (289.0-118.0-118.0 kg ha−1 NPK).

In arid areas precipitation patterns and soil nutrient determine plant survival. To study the effect of fertilizer addition on plant traits and on biological soil crusts under different rainfall intervals we collected and sowed the seeds of three typical psammophyte species from a natural community in China’s Mu Us Desert, and raised their seedlings in pots. During the growing season we added commercial seaweed fertilizer to the pots and irrigated the plants at different intervals. After one growing season we measured plant traits and the biomass of biological soil crusts, and soil nutrient contents. Seaweed fertilizer increased biomass production. The combined effects of seaweed fertilizer and rainfall interval were related to the life history attributes of the species. For a given fertilizer addition, changing the rainfall interval from 3 to 15 days increased the Agriophyllum squarrosum and Achnatherum splendens biomass, but decreased Glycyrrhiza uralensis biomass. Seaweed fertilizer significantly affected plant traits, but the effects of the rainfall interval on plant traits were not significant. Fertilizer addition increased the soil organic carbon and total nitrogen. Fertilizer addition also promoted biological soil crusts and enriched their total nitrogen content. Structural equation modeling showed that the organic carbon, total nitrogen, and chlorophyll a content of the biological crusts played different roles in biomass production of the three species. Soil carbon and nitrogen affected biological soil crusts more than they affected plant biomass production. Our results show that seaweed fertilizer can promote plant growth and biological crust development, making it useful for ecosystem restoration in arid and semi-arid regions.

Amorpha fruticosa is often used to revegetate saline wasteland in the YinNan Irrigation Area, northwest China. However, the survival rate of transplanted A. fruticosa seedlings is constrained by its low tolerance to high soil salinity, drought, and low soil temperatures (Tsoil) at the early transplanting stage. This study aimed to evaluate the effects of three different mulches, including plastic mulch (PM), sand mulch (SM) and rice straw mulch (RSM), and control (CK) on the soil microenvironment and A. fruticosa seedling growth. The results indicated that PM (19.1–26.2 °C), followed by SM (18.6–26.1 °C), provided the highest Tsoil at 0–20 cm depth during the whole growing period, while RSM (16.5–21.5 °C) exhibited lower Tsoil than CK (15.8–22.0 °C) at all time except the later growing stage. RSM (7.1%–26.4%) retained soil water most effectively as compared to CK, followed by SM (3.2%–22.5%), and then PM (-0.6%–19.7%). PM (11.4%–18.5%) had the best performance in reduction of ECe at 0–80 cm depth, followed by RSM (10.5%–15.5%), and then SM (3.7%–7.4%). Still, PM exhibited the greatest improvement in soil structure. A. fruticosa seedlings grown in PM achieved the best stand establishment and growth characteristics, in which its root reached to 40–60 cm depth, while the counterparts in RSM showed the poorest performance in three mulching treatments. The results above suggest that PM is a promising strategy to promote the growth of transplanted halophytes in saline wasteland of the YinNan Irrigation Area and other areas with similar ecologies.

A field trial consisting of cotton grown employing a combination of ridge planting, mulching with film, and drip irrigation was laid out on a plot with severely saline soil in a typical inland arid area of Xinjiang. The effect of five levels of soil matric potential set up 0.2 m below the drip emitter, namely −5 kPa, −10 kPa, −15 kPa, −20 kPa, and −25 kPa, were studied in terms of changes in soil salinity (ECe), sodicity (SAR), crop growth and yield components. Drip irrigation increased the leaching of soil salts and decreased the ECe and SAR of each soil layer. Although the levels of soil salt rose again, in spring and winter, after irrigation was discontinued, the root zone (0–40 cm) remained less saline: the ECe and SAR value under the soil matric potential of −5 kPa and −10 kPa were 63% and 49% of its values in 2009 respectively, before the land was brought under cultivation (p ≤ 0.05), showing maximum leaching. The yield of cotton peaked at the soil matric potential of −5 kPa. The germination rate, which was the main factor that influenced the cotton yield, was 67% of that in non-saline soil in the first two years, and increased to 84% in the third year. After three years, the rate of germination in all the treatments exceeded 67%, and the highest rate (78%) was at −5 kPa; in the same treatment, boll yield was 4.40 g per plant. Except for germination rate and the yield of lint and seed, all the yield components increased significantly (p ≤0.05) as ECe and SAR decreased in 2010 and 2011. The correlation between soil salt (salinity and sodicity) and other components such as the number of cotton bolls per plant, the average weight of a boll, and lint percentage varied, probably because water supply was being regulated and, as a result, the physicochemical properties of the soil kept changing constantly. Taking into account the extent of leaching, crop growth, and yield, the lower limit for the soil matric potential should be −5 kPa at 20 cm below the dripper for the first three years during reclamation to promote cotton cultivation on the saline-sodic soil of Xinjiang.

Camptothecin (CPT) is a cytotoxic quinoline alkaloid isolated from the bark and branches of the Chinese tree Camptotheca acuminata. CPT inhibits topoisomerase I. It possesses various antitumor activities and is mainly used in the treatment of colon, ovarian, liver, and bone cancers as well as leukemia. CPT inhibits the expressions of inflammatory genes and can prevent death from chronic inflammation. Therefore, we investigated the effect of CPT treatment in ulcerative colitis (UC) using DSS-induced UC mouse model; after that, we explored its potential mechanisms. Here, we found that CPT exerted protection on DSS-induced UC in rats. In addition, the administration prominently reduced the disease activity index as well as colon length of the model rats and remarkably reduced the inflammatory cytokines. Further, CPT significantly reduced several vital proinflammatory proteins in LPS-induced RAW264.7 cells. In summary, our findings demonstrate that CPT is hopefully to act as a therapeutic agent for UC.

BackgroundThromboembolism is a life-threatening, limb-threatening or organ-threatening complication that occurs in patients with primary nephrotic syndrome (NS). There are few studies on the spectrum, complications and outcomes of thrombosis in children with NS. This study aimed to determine the spectrum of thrombosis and its relationship with the nephrotic state, treatment and outcomes in children and adolescents with primary NS.MethodsThe medical records of subjects aged 1–18 years with NS complicated with thromboembolism treated at our centre within the last 26 years were retrieved. Data on the status of NS, site, symptoms and signs, laboratory investigations, diagnosis, treatment, complications and outcomes of thrombosis were collected and reviewed retrospectively. A severe complication was defined as a condition associated with thrombosis requiring a special diagnostic modality to confirm or a specific treatment such as surgical intervention. The outcome of thrombosis was defined as the status of thrombosis, as determined by imaging methods and the functional status with respect to the anatomic sites of thrombosis at the last follow-up. The permanent dysfunction of an organ or limb related to thrombosis was defined as a sequela.ResultsWe observed thrombosis in 1.4% (27/1995) of subjects with NS during the study period. There were 27 subjects with thrombosis, including 21 males and 6 females. Thrombosis was observed in 51.9% (14/27) of the study participants with steroid resistant NS. Most episodes of thrombosis occurred during the active stage of NS; however, 7.4% of thrombosis cases occurred during the remission of proteinuria. Renal vein thrombosis (33.3%) and pulmonary embolism (25.9%) were the most common types of thrombosis. Among the 17 subjects biopsied, minimal change disease and membranous nephropathy were the two most common findings. Six (22.2%) subjects experienced severe complications or sequelae; 1 had persistent intracranial hypertension, 1 had intestinal perforation, 1 had hypoxemia and pulmonary hypertension, 1 had lameness, 1 had epilepsy, and 1 had an askew mouth due to facial paralysis. In 19 (70.4%) subjects, the symptoms resolved completely or improved without severe complications or sequelae.ConclusionsThrombosis mostly occurred in males of school age during the active stage of NS. Renal vein thrombosis and pulmonary embolism were the most common types of thrombosis. In most patients with thrombosis, the symptoms improved completely without complications with standard anticoagulation therapy. However, 22.2% had severe complications or sequelae requiring an advanced diagnostic modality and aggressive treatment.

Background: A false interpretation of homozygosity for pathogenic variants causing autosomal recessive disorders can lead to improper genetic counseling. The aim of this study was to demonstrate the underlying etiologies of presumed homozygous disease-causing variants harbored in six unrelated children with five different genetic renal diseases when the same variant was identified in a heterozygous state in only one of the two parents from each family using direct sequencing. Methods: Peripheral blood genomic DNA samples were extracted. Six short tandem repeats were used to verify the biological relationships between the probands and their parents. Quantitative PCR was performed to detect mutant exons with deletions. Single nucleotide polymorphism analysis and genotyping with polymorphic microsatellite markers were performed to identify uniparental disomy (UPD). Results: Each proband and his/her parents had biological relationships. Patients 2, 4, and 6 were characterized by large deletions encompassing a missense/small deletion in DGKE, NPHP1, and NPHS1, respectively. Patients 1 and 5 were caused by segmental UPD in NPHS2 and SMARCAL1, respectively. In patient 6, maternal UPD, mosaicism in paternal sperm or de novo variant in NPHP1 could not be ruled out. Conclusions: When a variant analysis report shows that a patient of non-consanguineous parents has a pathogenic presumed homozygous variant, we should remember the need to assess real homozygosity for the variant, and a segregation analysis of the variants within the parental DNAs and comprehensive molecular tests to evaluate the potential molecular etiologies, such as a point variant and an overlapping exon deletion, UPD, germline mosaicism and de novo variant, are crucial.

Pediatric native kidney diseases are common worldwide. The pathological diagnosis of kidney lesions is crucial for clinical treatment and prognosis. The aim of the current study was therefore to evaluate the value of electron microscopy (EM) to the final diagnosis of native kidney biopsies in children.A retrospective evaluation of 855 pediatric kidney biopsies obtained from the Department of Pediatrics in Peking University First Hospital between November 2010 and December 2017 was performed to assess the contribution of EM to the final diagnosis.The role of EM in the final diagnosis was determined to be crucial in 300 cases (35.1%), important in 280 cases (32.7%), and auxiliary in 275 cases (32.2%). EM is considered most valuable in a large percentage of glomerular diseases, mainly including minimal change disease, early-stage membranous nephropathy, postinfectious glomerulonephritis, Alport syndrome, thin basement membrane nephropathy, and thrombotic microangiopathy. EM also provided helpful diagnostic information in cases of focal segmental glomerulosclerosis, lupus nephritis, IgA nephropathy, and IgA vasculitis (Henoch-Schonlein purpura nephritis). Additionally, EM was crucial in 90.0% of cases of subtle pathological changes observed with light microscopy (LM) and immunofluorescence (IF) and in 69.3% of the IF-negative specimens. Patients with nephrotic syndrome or hematuria also benefit from ultrastructural examination.The present study demonstrated the crucial or important role of EM in the diagnosis of a majority of native kidney biopsies in children. The application of EM should be integrated together with LM and IF as a routine method of assessing pediatric kidney specimens. Graphical abstract.

Background To characterize the phenotypic spectrum and assess the antialbuminuric response to angiotensin converting enzyme (ACE) inhibitor and/or angiotensin receptor blocker (ARB) therapy in a cohort of children with Dent disease. Methods The patients’ clinical findings, renal biopsy results, genetic and follow‐up data were analyzed retrospectively. Mutations in CLCN5 or OCRL were detected by next‐generation sequencing or Sanger sequencing. Results Of 31 Dent disease boys, 24 carried CLCN5 and 7 carried OCRL mutations. Low molecular weight proteinuria and albuminuria were detected in all cases. Nephrotic‐range proteinuria and severe albuminuria were identified in 52% and 62% of cases, respectively; by 7 years of age, 6 patients had hematuria and nephrotic‐range proteinuria, and 7 patients had hematuria and moderate to severe albuminuria. In addition to disease‐related renal features, patients with Dent‐1 disease also presented with congenital cataract (1/9) and developmental delay (2/7). Seventeen of 31 patients underwent renal biopsy. Glomerular changes included mild glomerular lesions, mesangial proliferative glomerulonephritis and focal segmental glomerular sclerosis. Thirteen of the 31 patients had follow‐up records and received ACE inhibitor and/or ARB treatment for more than 3 months. After a median 1.7 (range 0.3–8.5) years of treatment, a reduction in the urinary microalbumin‐to‐creatinine ratio was observed in 54% of children. Conclusions Hematuria with nephrotic‐range proteinuria or moderate to severe albuminuria was common in Dent disease patients. Extrarenal manifestations were observed in Dent‐1 patients, which extends the phenotypic spectrum. In addition, ACE inhibitors and ARBs are well tolerated, and they are partially effective in controlling albuminuria., Hematuria with nephrotic‐range proteinuria or moderate to severe albuminuria was common in Dent disease patients.Patients with Dent‐1 disease may also present with congenital cataract and developmental delay.The percentage of urinary albumin in the total protein ranged from 24.2% to 47.6%; it was usually smaller than that of LMW proteinuria. ACE inhibitors and ARBs are partially effective in controlling albuminuria and they are well tolerated.

Alport syndrome is a hereditary progressive chronic kidney disease caused by mutations in type IV collagen genes COL4A3/4/5. X-linked Alport syndrome (XLAS) is caused by mutations in the COL4A5 gene and is the most common form of Alport syndrome. A strong correlation between the type of COL4A5 mutation and the age developing end-stage renal disease in male patients has been found. Mutation to the α (IV) chain causes retention of the protein to the endoplasmic reticulum lumen, which causes endoplasmic reticulum stress (ERS) and subsequent exertion of deleterious intracellular effects through the activation of ERS. The exact time point that mutant type IV collagen α chain exerts its deleterious effects remains elusive. In this study, we explored the relationship between the COL4A5 genotype and cell type in ERS activation. We obtained skin fibroblasts from Alport syndrome patients with different COL4A5 mutation categories [i.e., a missense mutation (c.4298G > T, p.Gly1433Val) in exon 47, a splicing mutation (c.1949–1G > A) in intron 25 and an insertion (c.573_c.574insG, p. Pro193Alafs*23) in exon 10], and then reprogrammed these fibroblasts into induced pluripotent stem cells (iPSCs). Interestingly, no significant dysregulation of ERS pathway markers was observed for the three COL4A5 mutant iPSCs; however, significant activation of ERS in COL4A5 mutant fibroblasts was observed. In addition, we found that the activation levels of some ERS markers in fibroblasts varied among the three COL4A5 mutation types. Mutant COL4A5 proteins were demonstrated to have different effects on cells at different stages of ontogenesis, providing a theoretical basis for choosing the timing of intervention. The observed differences in activation of ERS by the COL4A5 mutant fibroblasts may contribute to the intracellular molecular mechanisms that describe the correlation between genotype and clinical features in XLAS.

Background Alport syndrome is a rare hereditary kidney disease manifested with progressive renal failure. Considerable variation exists in terms of disease progression among patients with Alport syndrome. Identification of patients at high risk of rapid progression remains an unmet need. Urinary epidermal growth factor (uEGF) has been shown to be independently associated with risk of progression to adverse kidney outcome in multiple independent adult chronic kidney disease (CKD) cohorts. In this study, we aim to assess if uEGF is associated with kidney impairment and its prognostic value for children with Alport syndrome. Methods One hundred and seventeen pediatric patients with Alport syndrome and 146 healthy children (3–18 years old) were included in this study. uEGF was measured in duplicates in baseline urine samples using ELISA (R&D) and concentration was normalized by urine creatinine (uEGF/Cr). In patients with longitudinal follow-up data (n = 38), progression was defined as deteriorated kidney function (CKD stage increase) during follow-up period (follow-up length is about 31 months in average). The association of baseline uEGF/Cr level with estimated glomerular filtration rate (eGFR) slope and Alport syndrome patients’ progression to a more advanced CKD stage during the follow-up period was used to evaluate the prognostic value of the marker. Results We found that uEGF/creatinine (uEGF/Cr) decreases with age in pediatric patients with Alport syndrome with a significantly faster rate than in healthy children of the same age group. uEGF/Cr is significantly correlated with eGFR (r = 0.75, p

Aim The TTC21B gene is now known as causative of nephronophthisis-related ciliopathies (NPHP-RC). We reported two Chinese pediatric cases with end-stage renal disease and other phenotypes caused by the TTC21B gene mutations. Methods The clinical features of Chinese pediatric cases with NPHP-RC were summarized. Mutation analysis of the TTC21B gene was performed using next-generation sequencing. Results The two cases both had nephrotic proteinuria, renal failure, hypertension and abnormal liver function (or hepatic fibrosis). One case also presented situs inversus and short phalanges. They developed end-stage renal disease (ESRD) at 1 year old and 8 years old, respectively, when renal pathology both showed focal segmental glomerular sclerosis (FSGS) with tubulointerstitial lesions including interstitial fibrosis and atrophic tubules. Three novel disease-causing TTC21B mutations were identified. One case carried homozygous mutation c.2211 + 3A > G, while the other case carried compound heterozygous mutations c.1552 T > C (p.C518R) and c.1456dupA (p.R486KfsX22). Conclusion Mutations in TTC21B cause a range of ciliopathy phenotypes in humans. We identified 3 novel TTC21B mutations in two Chinese pediatric cases that both presented end-stage renal disease and other different features. This is the first TTC21B mutations ever reported in China.