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  • Frontiers Media SA  (5)
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  • Frontiers Media SA  (5)
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  • 1
    In: Frontiers in Pediatrics, Frontiers Media SA, Vol. 9 ( 2021-7-14)
    Abstract: Background: Infection is the most common complication of Idiopathic Nephrotic Syndrome (INS) and the main cause of INS recurrence, severe infection and even leading to mortality. The purpose of this study was to investigate the risk factors of severe infection in INS children and the clinical parameters influencing prognosis. Methods: Totally 147 children with INS and concomitant infections were enrolled and classified into the severe infection group (SIG) and Non-severe infection group (Non-SIG). The clinical characteristics and auxiliary examination results were compared between the two groups, and the early-warning parameters for severe infection and risk factors for poor prognosis were evaluated. Results: There were 49 patients in the SIG, 98 patients in the Non-SIG. In the SIG, the most common severe infections disease included severe pneumonia (63.6%), severe sepsis (30.6%), septic shock (4.1%). In SIG, Gram-positive bacteria (GPB) were more common, as was respiratory syncytial virus (RSV), and the three most common strains were Pseudomonas aeruginosa, Staphylococcus aureus (SA) and Staphylococcus epidermidis. There were more steroid-resistant nephrotic syndrome and combination of steroids and immunosuppressants in SIG, compared with the Non-SIG ( P = 0.000). Patients in the SIG has lower complement 3 (C3, ≤ 0.55 g/L,) and absolute lymphocyte count (ALC, ≤ 1.5 × 10 9 /L) ( P = 0.004). Logistic regression analysis revealed that the independent risk factors for severe infections were the combined use of immunosuppressants [95% confidence interval (CI):1.569–463.541, P = 0.023], steroid resistance (95% CI: 4.845–2,071.880, P = 0.003), C-reactive protein (CRP) ≥8 mg/L (95% CI: 43.581–959, 935.668, P = 0.001), and infections caused by GPB (95% CI: 27.126–2,118, 452.938, P = 0.002), influenza (95% CI: 2.494–1, 932.221, P = 0.012) and RSV (95% CI: 5.011–24 963.819, P = 0.007). The patients in the SIG were classified into the survival group ( N = 39) and the mortality group ( N = 5). Logistic regression analysis showed that white blood cell count (WBC) & gt;15 × 10 9 /L (95% CI: 1.046–2.844, P = 0.033) was an independent risk factor of poor prognosis for these patients. Conclusions: Resistance to steroids, combined with steroids and IS agents, and GPB infections (especially SA) are high-risk factors for severe infection in children with INS. We should monitor CRP ≥ 8 mg/L, C3 ≤ 0.55 g/L and ALC ≤ 1.5 × 10 9 /L to avoid developing severe infection. Accompanied by an increase in ANC, WBC significantly increased, suggesting a fatal infection.
    Type of Medium: Online Resource
    ISSN: 2296-2360
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2021
    detail.hit.zdb_id: 2711999-3
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  • 2
    Online Resource
    Online Resource
    Frontiers Media SA ; 2022
    In:  Frontiers in Genetics Vol. 13 ( 2022-3-31)
    In: Frontiers in Genetics, Frontiers Media SA, Vol. 13 ( 2022-3-31)
    Abstract: Background: PAX2 is a nuclear transcription factor gene that is highly conserved among species. Variants within PAX2 could result in optic nerve colobomas and kidney hypoplasia. However, little clinical and genetic information is currently available about PAX2 variants in Chinese children. Objective: This study aims to further understand the clinical manifestations and genetic characteristics of PAX2 variants in Chinese population. Methods: In this single-center retrospective study, we analyzed the clinical data of 10 children identified as carriers of PAX2 variants by gene sequencing. All the variants found in this study were analyzed using in silico prediction and American College of Medical Genetics and Genomics (ACMG) standards and guidelines. Results: The mean age for developing the first symptom in 10 unrelated children was 7.2 years old. Proteinuria and bilateral kidney dysplasia were found in every patient. Two children underwent kidney histological examination; one child showed high-intensity C1q deposition in the kidney, and the other child showed focal segmental glomerular sclerosis (FSGS). Three children had PAX2 -related ocular abnormalities, including nystagmus, retinal exudation, amblyopia, microphthalmia, microcornea, and total blindness. In addition, one patient had the comorbidity of oculocutaneous albinism (OCA). Eight different PAX2 variants were found in ten patients, three of which were reported for the first time. Conclusion: We reported some patients with unique manifestations and comorbidities, and we reported three variants that have not been previously identified. The PAX2 gene is prone to spontaneous variants, and the outcome of patients is unfavorable. Because of the lack of specific therapy, genetic testing should be recommended for individuals with obvious evidence of kidney dysplasia and eye abnormalities, and kidney protective treatment should be initiated early.
    Type of Medium: Online Resource
    ISSN: 1664-8021
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2022
    detail.hit.zdb_id: 2606823-0
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  • 3
    Online Resource
    Online Resource
    Frontiers Media SA ; 2023
    In:  Frontiers in Pediatrics Vol. 11 ( 2023-6-23)
    In: Frontiers in Pediatrics, Frontiers Media SA, Vol. 11 ( 2023-6-23)
    Abstract: Immunoglobulin A nephropathy (IgAN) is one of the most common kidney diseases leading to renal injury. Of pediatric cases, 25%–30% progress into end-stage kidney disease (ESKD) in 20–25 years. Therefore, predicting and intervening in IgAN at an early stage is crucial. The purpose of this study was to validate the availability of an international predictive tool for childhood IgAN in a cohort of children with IgAN treated at a regional medical centre. Methods An external validation cohort of children with IgAN from medical centers in Southwest China was formed to validate the predictive performance of the two full models with and without race differences by comparing four measures: area under the curve (AUC), the regression coefficient of linear prediction (PI), survival analysis curves for different risk groups, and R 2 D. Results A total of 210 Chinese children, including 129 males, with an overall mean age of 9.43 ± 2.71 years, were incorporated from this regional medical center. In total, 11.43% (24/210) of patients achieved an outcome with a GFR decrease of more than 30% or reached ESKD. The AUC of the full model with race was 0.685 (95% CI : 0.570–0.800) and the AUC of the full model without race was 0.640 (95% CI : 0.517–0.764). The PI of the full model with race and without race was 0.816 ( SE  = 0.006, P  & lt; 0.001) and 0.751 ( SE  = 0.005, P  & lt; 0.001), respectively. The results of the survival curve analysis suggested the two models could not well distinguish between the low-risk and high-risk groups ( P  = 0.359 and P  = 0.452), respectively, no matter the race difference. The evaluation of model fit for the full model with race was 66.5% and without race was 56.2%. Conclusions The international IgAN prediction tool has risk factors chosen based on adult data, and the validation cohort did not fully align with the derivation cohort in terms of demographic characteristics, clinical baseline levels, and pathological presentation, so the tool may not be highly applicable to children. We need to build IgAN prediction models that are more applicable to Chinese children based on their particular data.
    Type of Medium: Online Resource
    ISSN: 2296-2360
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2023
    detail.hit.zdb_id: 2711999-3
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  • 4
    Online Resource
    Online Resource
    Frontiers Media SA ; 2022
    In:  Frontiers in Oncology Vol. 12 ( 2022-9-28)
    In: Frontiers in Oncology, Frontiers Media SA, Vol. 12 ( 2022-9-28)
    Abstract: Tumor cells undergo epithelial-mesenchymal transition (EMT), however, there is a room of disagreement in role of EMT heterogeneity to colorectal cancer metastasis (mCRC) evolution. To uncover new EMT-related metastasis proteins and pathways, we addressed the EMT status in colorectal cancer liver metastasis patient-derived CTCs to identify proteins that promote their distant metastasis. And then, we performed a comparative proteomic analysis in matched pairs of primary tumor tissues, adjacent mucosa tissues and liver metastatic tissues. By integrative analysis we show that, unstable Epithelial/Mesenchymal (E/M)-type CTCs had the strongest liver metastases formation ability and the proportion of E/M-type CTCs correlated with distant metastases. Using an optimized proteomic workflow including data independent acquisition (DIA) and parallel reaction monitoring (PRM), we identified novel EMT-related protein cluster (GNG2, COL6A1, COL6A2, DCN, COL6A3, LAMB2, TNXB, CAVIN1) and well-described (ERBB2) core protein level changes in EMT-related metastasis progression, and the proteomic data indicate ERBB2, COL6A1 and CAVIN1 are promising EMT-related metastatic biomarker candidates. This study contributes to our understanding of the role that EMT plays in CRC metastasis and identifies heterogeneous EMT phenotypes as a key piece for tumor progression and prognosis. We further propose that therapies targeting this aggressive subset (E/M-type) of CTCs and related protein may be worthy of exploration as potential suppressors of metastatic evolution.
    Type of Medium: Online Resource
    ISSN: 2234-943X
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2022
    detail.hit.zdb_id: 2649216-7
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  • 5
    In: Frontiers in Molecular Neuroscience, Frontiers Media SA, Vol. 14 ( 2021-11-2)
    Abstract: The medial prefrontal cortex (mPFC), a key part of the brain networks that are closely related to the regulation of behavior, acts as a key regulator in emotion, social cognition, and decision making. Astrocytes are the majority cell type of glial cells, which play a significant role in a number of processes and establish a suitable environment for the functioning of neurons, including the brain energy metabolism. Astrocyte’s dysfunction in the mPFC has been implicated in various neuropsychiatric disorders. Glucose is a major energy source in the brain. In glucose metabolism, part of glucose is used to convert UDP-GlcNAc as a donor molecule for O-GlcNAcylation, which is controlled by a group of enzymes, O-GlcNAc transferase enzyme (OGT), and O-GlcNAcase (OGA). However, the role of O-GlcNAcylation in astrocytes is almost completely unknown. Our research showed that astrocytic OGT could influence the expression of proteins in the mPFC. Most of these altered proteins participate in metabolic processes, transferase activity, and biosynthetic processes. GFAP, an astrocyte maker, was increased after OGT deletion. These results provide a framework for further study on the role of astrocytic OGT/O-GlcNAcylation in the mPFC.
    Type of Medium: Online Resource
    ISSN: 1662-5099
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2021
    detail.hit.zdb_id: 2452967-9
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