This study investigated the role of the nuclear factor of activated T cells c3 (NFATc3) in vascular smooth muscle cells (VSMCs) during aortic aneurysm and dissection (AAD) progression and the underlying molecular mechanisms. Cytoplasmic and nuclear NFATc3 levels were elevated in human and mouse AAD. VSMC-NFATc3 deletion reduced thoracic AAD (TAAD) and abdominal aortic aneurysm (AAA) progression in mice, contrary to VSMC-NFATc3 overexpression. VSMC-NFATc3 deletion reduced extracellular matrix (ECM) degradation and maintained the VSMC contractile phenotype. Nuclear NFATc3 targeted and transcriptionally upregulated matrix metalloproteinase 9 (MMP9) and MMP2, promoting ECM degradation and AAD development. NFATc3 promoted VSMC phenotypic switching by binding to eukaryotic elongation factor 2 (eEF2) and inhibiting its phosphorylation in the VSMC cytoplasm. Restoring eEF2 reversed the beneficial effects in VSMC-specific NFATc3-knockout mice. Cabamiquine-targets eEF2 and inhibits protein synthesis-inhibited AAD development and progression in VSMC-NFATc3-overexpressing mice. VSMC-NFATc3 promoted VSMC switch and ECM degradation while exacerbating AAD development, making it a novel potential therapeutic target for preventing and treating AAD.

Allogeneic haemopoietic stem cell transplantation (allo-HSCT)-related nephrotic syndrome is a rare complication, recognized as a clinical manifestation of chronic graft versus host disease (GVHD). T cell dysfunction is thought to play a significant role in the pathogenesis of allo-HSCT-related nephrotic syndrome, but the precise mechanism remains unclear. This paper reported a case of X-linked adrenoleukodystrophy (X-ALD) who had good control of the disease after allo-HSCT, but developed proteinuria and progressed to nephrotic syndrome after immunosuppressive therapy was tapered. Kidney biopsy revealed secondary membranous nephropathy, which responded well to treatment with glucocorticoids and tacrolimus. Limited literature exist on allo-HSCT-related nephrotic syndrome in children. This study provides a comprehensive summary of its mechanism, clinical features, pathology, diagnosis,and treatment, offering valuable insights for diagnosing and managing allo-HSCT-related nephrotic syndrome in pediatric patients.

Allogeneic haemopoietic stem cell transplantation (allo-HSCT)-related nephrotic syndrome is a rare complication, recognized as a clinical manifestation of chronic graft versus host disease (GVHD). T cell dysfunction is thought to play a significant role in the pathogenesis of allo-HSCT-related nephrotic syndrome, but the precise mechanism remains unclear. This paper reported a case of X-linked adrenoleukodystrophy (X-ALD) who had good control of the disease after allo-HSCT, but developed proteinuria and progressed to nephrotic syndrome after immunosuppressive therapy was tapered. Kidney biopsy revealed secondary membranous nephropathy, which responded well to treatment with glucocorticoids and tacrolimus. Limited literature exist on allo-HSCT-related nephrotic syndrome in children. This study provides a comprehensive summary of its mechanism, clinical features, pathology, diagnosis,and treatment, offering valuable insights for diagnosing and managing allo-HSCT-related nephrotic syndrome in pediatric patients.

ObjectiveTo explore the suitability of four qualitative research methods in the development of TCM scale. MethodsTaking the development of "Seven Emotions Scale" as an example， we conducted semi-structured interviews with 31 patients of emotional disorders and 10 healthy people by objective sampling， and collected psychological feelings and emotional cognition data related to seven emotions according to the interview outline. Two qualitative methods， descriptive qualitative research and descriptive phenomenology， were used to analyze the data and construct the item library of the scale. The conceptual framework of the scale was constructed by using commonly used grounded theory and frame analysis. ResultsDuring data analysis， it is found that the themes extracted from descriptive phenomenology were not easily understood by the interviewees， and it is difficult for the researchers to truly achieve the "suspension" required by phenomenology. Considering the feasibility and convenience of the researchers' actual operation， as well as whether the initial purpose of the scale research can be intuitively included in the interviewees' views and feelings， descriptive phenomenology is not suitable for the formation of scale items. Using descriptive qualitative research method to analyze the interview data of healthy people and patients with emotional disorders， 306 and 476 scale items were obtained respectively. Through grounded theory， five selective codes were obtained： physical symptoms， external manifestations， psychological feelings， behaviors and emotional control. Using frame analysis， four themes including physical symptoms， psychological feelings， behavior and emotional cognition were constructed. Both methods can be used to construct the conceptual frame of scale， but the framework analysis is more convenient and can better ensure the transparency of the research. ConclusionDescriptive qualitative research methods can be used to form the item library of TCM scales. Framework analysis is more suitable for the construction of the conceptual framework of the scale than grounded theory， while descriptive phenomenology is not suitable for the development of TCM scales.

Aim Preliminarily construct a classification system for Yi medicine diseases,and establish a relatively well-defined and clear hierarchy of disease terms and their classification system.Methods Adopting the research methods of Yi medicine,terminology and standard science,and drawing on the idea of standardisation of Chinese medicine's disease classification system,through in-depth excavation of the content characteristics of the 52 excavated ancient Yi medicine books on diseases and medicines,systematically collate the terms and terminology of Yi medicine diseases,and formulate the'Three-level Class List of Yi Medicine Diseases'on the basis of the original classification of ancient books,and construct a knowledge database of Yi medicine diseases.Constructing a knowledge database of Yi medical conditions.Results To establish the principle of classification of diseases and conditions in Yi medicine,with the first level of categories being the categories of diseases and conditions in Yi medicine,the second level of categories being the categories of sections,and the third level of categories being the subcategories of speciality systems,and to establish the method of classifying the various diseases and conditions in Yi medicine into categories and systems in accordance with the established principles.Conclusion The Yi medicine disease classification system is a systematic integration and standardised classification of disease terms from Yi medicine texts and literature over the ages,which helps to lead Yi medicine clinics to carry out medical activities in accordance with their own academic and clinical trajectories,and promotes the standardised research of Yi medicine.

Aim Preliminarily construct a classification system for Yi medicine diseases,and establish a relatively well-defined and clear hierarchy of disease terms and their classification system.Methods Adopting the research methods of Yi medicine,terminology and standard science,and drawing on the idea of standardisation of Chinese medicine's disease classification system,through in-depth excavation of the content characteristics of the 52 excavated ancient Yi medicine books on diseases and medicines,systematically collate the terms and terminology of Yi medicine diseases,and formulate the'Three-level Class List of Yi Medicine Diseases'on the basis of the original classification of ancient books,and construct a knowledge database of Yi medicine diseases.Constructing a knowledge database of Yi medical conditions.Results To establish the principle of classification of diseases and conditions in Yi medicine,with the first level of categories being the categories of diseases and conditions in Yi medicine,the second level of categories being the categories of sections,and the third level of categories being the subcategories of speciality systems,and to establish the method of classifying the various diseases and conditions in Yi medicine into categories and systems in accordance with the established principles.Conclusion The Yi medicine disease classification system is a systematic integration and standardised classification of disease terms from Yi medicine texts and literature over the ages,which helps to lead Yi medicine clinics to carry out medical activities in accordance with their own academic and clinical trajectories,and promotes the standardised research of Yi medicine.

Objective:To investigate the potential of the antineutrophil cytoplasmic antibody (ANCA) renal risk score (ARRS) in predicting the prognosis of children with ANCA-associated glomerulonephritis (AAGN).Methods:Laboratory testing, renal pathology results, treatment and prognosis of 61 children with AAGN diagnosed by renal biopsy from June 2007 to May 2022 in General Hospital of Eastern Theater Command were retrospectively analyzed.The Kaplan-Meier method was used to evaluate the overall and renal survival of children with AAGN, and risk factors of progression to end stage renal disease (ESRD) were analyzed by Cox regression analysis. Results:Among the 61 children with AAGN, there were 14 males and 47 females with the age of (15.65±3.74) years.According to ARRS, AAGN children were assigned into low-risk group (27 cases), medium-risk group (21 cases) and high-risk group (13 cases). During a median follow-up duration of 46.36 (14.58, 95.62) months, the number of ESRD cases in the high-risk group (9 cases) was significantly higher than that of low-risk group (2 cases) and medium-risk group (3 cases) ( χ2=13.079, P<0.001). Kaplan-Meier survival analysis showed that AAGN children in the high-risk group had the worst renal prognosis ( χ2=5.796, P=0.016), while no significant difference was detected in the overall survival among the 3 groups ( χ2=2.883, P=0.237). Multivariate Cox regression showed that estimate glomerular filtration rate(eGFR)≤15 mL/(min·1.73 m 2) ( HR=9.574, 95% CI: 4.205-25.187, P=0.015) and ARRS ( HR=2.115, 95% CI: 1.206-4.174, P=0.012) were independent risk factors for children with AAGN progress to ESRD.Receiver operating characteristic (ROC) curve analysis results showed that the area under the curve of ARRS for predicting the risk of progressing to ESRD in AAGN children was 0.880 (95% CI: 0.759-1.000), and the optimal cutoff value of ARRS was 5.50, with the sensitivity and specificity of 85.71% and 82.98%, respectively. Conclusions:ARRS was an independent risk factor for children with AAGN progress to ESRD, which had a predictive value for the progression of AAGN to ESRD.

The paper reports two cases of lipoprotein glomerulopathy (LPG) in children. The Sanger sequencing results in 2 cases indicated apolipoprotein E gene mutation[c.127 (exon3) C>T, p.R43C (p.Arg43Cys); c.494 (exon4) G>C, p.R165P (p.Arg165Pro),respectively]. Renal pathological presentation of two children showed that a large number of lipoprotein emboli were formed in the glomerular capillary loop, and the diagnosis of LPG was confirmed. The onset of LPG has no specific clinical manifestation, which is easy to be undiagnosed or misdiagnosed. Renal biopsy is a diagnostic means, glucocorticoid treatment is ineffective, and long-term lipid-lowering treatment may be required for LPG.

Objective: To investigate the protective effect of Paeoniflorin-6 '-o-benzene sulfonate ( CP-25) on methotrexate (MTX) -induced liver injury in rats． Methods: 40 SD rats were randomly divided into normal group，model group，CP-25 treatment group，CP-25 prevention group and resveratrol prevention group．A rat model of liver injury was established by single intraperitoneal injection of MTX (20 mg / kg) ，the levels of serum alanine aminotransferase (ALT) ，aspartate aminotransferase ( AST) ，superoxide dismutase ( SOD) ，glutathione ( GSH) and malondialdehyde (MDA) were determined by ELISA，the levels of interleukin-1 β (IL-1 β) ，interleukin-6 (IL-6) ， tumor necrosis factor-α (TNF-α) ，B-lymphocyte tumor-2 (Bcl-2) ，Bcl-2-associated X (Bax) ，cytochrome C ( Cyt- C) ，cleaved cysteinyl aspartate specific proteinase8 ( cleaved-cas8 ) ，cleaved cysteinyl aspartate specific proteinase 9 (cleaved-cas9) and reduced nicotinamide adenine dinucleotide phosphate oxidase 4 (NOX4) containing cysteine were assessed by Western blot. Results: Compared with the normal group，the levels of ALT and AST in MTX-induced liver injury model rats increased (F = 70. 23，P＜0. 01 ; F = 11. 09，P＜0. 05) ，and the liver histopathology showed extensive inflammatory cell infiltration，hepatic lobular structure disorder and hepatocyte swelling. Compared with MTX model group，the levels of ALT and AST in CP-25 prevention group and treatment group were lower (F = 62. 32，86. 86，P＜0. 01 ; F = 16. 35，7. 14，P ＜0. 01 ) ，and the pathological score of liver tissue was lower (F = 18. 33，P＜0. 01 ; F = 15. 47，P＜0. 05) ．Further studies showed that compared with MTX model group， both CP-25 prevention group and treatment group could significantly down-regulate the expression of pro-apoptotic proteins Bax(F = 21. 37，P＜0. 01 ; F = 19. 35，P＜0. 05) ，Cyt-C (FCP-25 prevention group = 7. 21，P＜0. 01) ，cleavedcas8 (FCP-25 prevention group = 12. 32，P＜0. 05) and cleaved-cas9(F = 8. 41，P＜0. 01 ; F = 6. 91，P＜0. 05) ，and upregulate the expression of anti-apoptotic protein Bcl-2 (F = 13. 11，P＜0. 01 ; F = 9. 93，P＜0. 05) . At the same time，compared with MTX model group，CP-25 prevention group and treatment group decreased the levels of IL- 1 β , IL-6 and TNF-α(FCP-25 prevention group = 160. 7，46. 55，28. 89，P＜0. 01) ; FCP-25 treatment group = 127. 4，53. 70，26. 46， P ＜ 0. 01 ) ，down-regulated the levels of MDA and NOX4 ( FCP-25 prevention group = 31. 71，38. 45 ，P ＜ 0. 01 ; FCP-25 treatment group = 27. 89，31. 27，P＜0. 01) ，and up-regulated the level of GSH ( F = 39. 65，29. 04，P ＜0. 01) . Conclusion CP-25 has a good therapeutic and protective effect on hepatotoxicity induced by MTX，and this effect may be related to its inhibition of oxidative stress，inflammatory response，and reduction of apoptosis in liver tissue.

Objective:To analyze the clinicopathologic features and prognosis of children with Henoch-Sch?nlein purpura nephritis (HSPN).Methods:The clinicopathological data of children with HSPN who were followed up for more than 5 years and underwent renal biopsy in Jinling Hospital affiliated to Medical School of Nanjing University from January 2001 to June 2015 were retrospectively analyzed. The follow-up endpoint event was defined as estimated glomerular filtration rate (eGFR)<90 ml·min -1·(1.73 m 2) -1. Participants were divided into two groups according to whether the children had reached the primary endpoint event or not. Cox proportional hazards model was used to analyze the influencing factors of renal poor prognosis in children with HSPN. Kaplan-Meier survival curve method was used for survival analysis, and log-rank test was used to compare the difference of renal cumulative survival rate between segmental sclerosis/adhesion (S1) group and non-segmental sclerosis/adhesion (S0) group. Receiver operating characteristic curve (ROC curve) and area under the curve ( AUC) were used to evaluate the diagnostic value. Results:A total of 130 children with HSPN were enrolled in the study. The median onset age was 11.7(8.6, 13.3) years old, of whom 71 cases were males (54.6%). At a median follow-up time of 100.0(75.8, 119.0) months, 12 cases (9.23%) with HSPN reached the primary endpoint event. Compared with the non-endpoint event group, the endpoint event group had higher proportion of hypertension, higher levels of 24-hour urinary protein, serum cholesterol, serum uric acid, and serum creatinine, and lower levels of serum albumin (all P<0.05). There was no statistical difference in treatment between the two groups (all P>0.05). In terms of pathological features, compared with the non-endpoint event group, the endpoint event group had higher proportion of mesangial hyperplasia (M1), S1, tubular atrophy/interstitial fibrosis (T1/T2) and Glomerulus-Bowman's capsule adhesion (all P<0.05). Multivariate Cox regression model showed that S1 was significantly correlated with renal poor prognosis ( HR=7.739, 95% CI 1.422-42.114, P=0.018). As was revealed in a Kaplan-Meier plot, renal cumulative survival rate in the S1 group was significantly lower than that in the S0 group (log-rank χ2=17.069, P<0.001). The ROC curve showed S1 accurately predicted the outcome ( AUC=0.710, 95% CI 0.549-0.872) with specificity of 0.667(95% CI 0.349-0.901) and specificity of 0.754(95% CI 0.667-0.829). Conclusions:S1 is an independent risk factor affecting renal poor prognosis and has a diagnostic value.