Renal fibrosis represents the core pathway through which chronic kidney disease progresses to end-stage renal failure. Therapeutic strategies targeting renal fibrosis provide an important approach for clinically mitigating the transition from acute kidney injury to chronic kidney disease. Macrophages, owing to their high heterogeneity in origin and phenotype, play a significant role in various stages of acute inflammation, tissue repair, and fibrosis, thereby holding substantial promise for the treatment of renal fibrosis. This review summarizes the pivotal role of renal macrophages in the transition from acute kidney injury to chronic kidney disease, with a focus on their temporal phenotypic reprogramming characteristics and the fibrotic microenvironment interaction network constituted by injured tubules, macrophages, and fibroblasts. On this basis, we systematically outline four core categories of macrophage-targeted intervention strategies: modulation of upstream signaling pathways, induction of macrophage phenotypic reprogramming, blockade of profibrotic factors and their downstream pathways, and selective depletion or engineered modification of profibrotic macrophage subsets. This review aims to provide new insights for future exploration of the timing, precision, and efficacy of macrophage-targeted therapies.

Since its establishment in 2016, the National Rare Diseases Registry System of China (NRDRS) has accumulated valuable case data and bio-specimen for basic and clinical research on rare diseases in China. However, the emerging challenges in clinical diagnosis and treatment of rare diseases make it unable for data and resource platform to fully meet the diversified needs. Under this backdrop, we have developed a protocol to optimize and upgrade the system based on the core functions of the NRDRS platform. The goal is to leverage intelligent digital technologies to transform NRDRS into a new platform integrating multimodal data and auxiliary diagnostic and treatment functions. It is specified as the development and construction of "one platform and four intelligent tools." Currently, we have upgraded and developed NRDRS platform, intelligent tool for genotype-phenotype analysis of rare diseases, AI-assisted diagnostic tool for rare diseases, remote multidisciplinary diagnosis and teaching tool for rare diseases, drug screening and validation tool for rare diseases. The next step will focus on the promotion of the application of these tools in clinical settings in order to address the issue of severe imbalance in the allocation of resources for the diagnosis and treatment of rare diseases. This article provides an overview of the digital and intelligent upgrades of the NRDRS, the trials in applications in clinical settings, and direction in the future.

Objective:To construct an evaluation index system for the development of compact county medical communities, so as to provide evaluation tools for promoting the high-quality development of county medical communities in China.Methods:Based on the evaluation criteria and monitoring system related policy documents for the construction of county medical communities released at the national level, an evaluation index system for compact county medical community development was constructed through policy text analysis and expert discussions. The system was applied to evaluate the construction of medical communities in Shaxian County of Fujian Province, A County of Shanxi Province, and B County of Yunnan Province.Results:The evaluation index system included four primary indicators: responsibility community, management community, service community, and interest community, covering 11 secondary indicators and 32 tertiary indicators. The evaluation results showed that the construction level of the compact county medical community in Shaxian County (91.3 points) was slightly higher than that of A County and B County (83.1 and 83.9 points), and its advantages were mainly reflected in management community and interest community. There was still room for improvement in the construction of compact medical communities in all three regions.Conclusions:The evaluation system in this study combined scientific rigor and practical guidance value. It could provide guidance for promoting the construction of compact county medical communities in various regions of China, and provide a basis for relevant policy formulation and optimization.

Objective:To construct an evaluation index system for the development of compact county medical communities, so as to provide evaluation tools for promoting the high-quality development of county medical communities in China.Methods:Based on the evaluation criteria and monitoring system related policy documents for the construction of county medical communities released at the national level, an evaluation index system for compact county medical community development was constructed through policy text analysis and expert discussions. The system was applied to evaluate the construction of medical communities in Shaxian County of Fujian Province, A County of Shanxi Province, and B County of Yunnan Province.Results:The evaluation index system included four primary indicators: responsibility community, management community, service community, and interest community, covering 11 secondary indicators and 32 tertiary indicators. The evaluation results showed that the construction level of the compact county medical community in Shaxian County (91.3 points) was slightly higher than that of A County and B County (83.1 and 83.9 points), and its advantages were mainly reflected in management community and interest community. There was still room for improvement in the construction of compact medical communities in all three regions.Conclusions:The evaluation system in this study combined scientific rigor and practical guidance value. It could provide guidance for promoting the construction of compact county medical communities in various regions of China, and provide a basis for relevant policy formulation and optimization.

Objective: To analyze the prevalence and trend of screening myopia among primary and middle school students in Linfen Community of Shanghai from 2019 to 2023, so as to provide a reference for the prevention and control of myopia from the perspective of the community. Methods: From 2019 to 2023, all primary(5) and middle(2) school students aged 6-15 years in Linfen Community of Shanghai were screened. Statistical analysis was performed using the Chi square test and trend Chi square test. The curve fitting model was used to fit the model of the increase rate of screening myopia among primary and middle school students in 2019, 2021 and 2023. Results: The overall rate of screening myopia among primary and middle school students in Linfen community from 2019 to 2023 was 55.17%. The prevalence rate of screening myopia was 79.43% in boys and 81.92% in girls in middle school, and the difference was statistically significant ( χ 2=5.71, P =0.02). In 2019, 2021, and 2023, the peak age of screening myopia among primary and middle school students in Linfen Community gradually occurred earlier, at the age of 7(12.13%), 6( 12.28 %), and 6(14.99%) years old, respectively. The growth rate of screening myopia in students aged 8-12 years in 2023 was lower than that in 2019 and 2021. Conclusions The screening myopia rate of primary and middle school students aged 6-15 years in Linfen Community is relatively high, with primary school girls higher than boys, and growth spurt accelerates. It is suggested that prevention and control of myopia in the community should focus on preschool children and adolescent girls.

Schimke immuno-osseous dysplasia (SIOD)caused by SMARCAL1 gene mutations is a rare genetic disorder characterized by multi-system involvement, including T-cell dysfunction, skeletal dysplasia, disproportionate short stature, nephrotic syndrome, and kidney failure. This multidisciplinary consultation involved a 9-year-old boy with intrauterine growth retardation, presenting with short stature, T-cell lymphopenia, proteinuria, and degenerative bone and joint disease. Treatment primarily focused on symptomatic and supportive care. Through the multidisciplinary rare disease consultation, holistic support was provided to the patient, offering comprehensive care from various specialtiesx.We also aim to use this case report to enhance clinicians′ under-standing of SIOD and improve the management and treatment of rare diseases.

Tuberous sclerosis complex(TSC)is a rare genetic disease that can lead to benign dysplasia in multiple organs such as the skin, brain, eyes, oral cavity, heart, lungs, kidneys, liver, and bones. Its main symptoms include epilepsy, intellectual disabilities, skin depigmentation, and facial angiofibromas, whilst incidence is approximately 1 in 10 000 to 1 in 6000 newborns. This case presents a middle-aged woman who initially manifested with epilepsy and nodular depigmentation. Later, she developed a lower abdominal mass, elevated creatinine, and severe anemia. Based on clinical features and whole exome sequencing, the primary diagnosis was confirmed as TSC. Laboratory and imaging examinations revealed that the lower abdominal mass originated from the uterus. CT-guided biopsy pathology and surgical pathology suggested a combination of leiomyoma and abscess. With the involvement of multiple organs and various complications beyond the main diagnosis, the diagnostic and therapeutic process for this patient highlights the importance of rigorous clinical thinking and multidisciplinary collaboration in the diagnosis and treatment of rare and challenging diseases.

Rare diseases have been a major challenge for clinical medicine and public health challenge in China. One of the effective measures is to conduct proactive research on rare diseases to deal with the disease burden of the diseases. However, low prevalence, disperse distribution of patients, lack of knowledge about the disease course, and phenotype heterogeneity hamper the development of research for rare diseases. Recently, it has been found that patients registry is effective in understanding the course of the disease and accu- mulating the cases and data of clinical research or clinical trial design. At present, most of developed countries or regions in the world have promoted clinical research and clinical trials of new medications on rare diseases by using the registration of rare disease. In 2016, Peking Union Medical College Hospital established China's first registry system at the national level-National Rare Disease Registry System of China(NRDRS). NRDRS has accumulated 68 137 cases data registered by the researchers from China's 101 collaborating hospitals in 29 provinces/municipalities/autonomous regions, covering 171 different, and forming 188 cohorts. To date, NRDRS complete the initial stage of resources buildup.Nex stage will be focused on clinical research and clinical trials related to rare diseases based on NRDRS. This article is on the process of building NRDRS, the potential support for conducting clinical research and clinical trials related to rare diseases, and the challenges will be faced.

Objective:To evaluate whether dialysis modality will affect cognitive function in dialysis population.Methods:This was a cross-sectional study. Chronic dialysis patients in our center was screened from July 2013 to July 2014. All of the subjects received brain magnetic resonance imaging (MRI) examination and comprehensive cognitive function evaluation.Results:A total of 189 chronic dialysis patients were enrolled in this study, 122 cases on hemodialysis (HD) and 67 cases on peritoneal dialysis (PD). There was no significant difference in age between HD and PD groups [(56.4±13.2) years vs (56.4±16.1) years, t=0.004, P=0.997]. The dialysis vintage and serum albumin of HD patients was higher than those of PD patients[58.0(16.8, 107.5) months vs 31.0(7.0, 67.0) months, Z=-3.490, P<0.001; (39.6±3.9) g/L vs (35.3±3.8) g/L, t=7.328, P<0.001, respectively]. The prevalence of cerebral small vessel diseases (CSVDs) was comparable between HD and PD groups (all P>0.05). Compared with HD patients, PD patients presented a 11.90-fold risk of immediate memory impairment (95% CI 1.40-101.08, P=0.023) and a 6.18-fold risk of long-delayed memory impairment (95% CI 2.12-18.05, P=0.001). After adjusting for age, educational lever, dialysis vintage, serum creatinine, and CSVDs, the influence of dialysis modality on memory still worked. PD patients presented a 43% risk of executive function impairment of HD patients ( OR=0.43, 95% CI 0.17-1.04, P=0.061). Conclusions:HD patients manifested better memory than PD patients, while PD probably performed better in executive function than HD patients. There was no significant difference in language function between the two groups. The difference in cognitive function may not be related to CSVDs.

Objective:To investigate the influence of cognitive-behavior therapy on the psychological status of family members of terminal cancer patients. Method:A total of 60 families of terminal cancer patients were selected and randomly divided into observation group (30 cases) and control group (30 cases). The observation group was treated with cognitive-behavior therapy, while the control group was given general supportive psychological care. The Hamilton Depression Scale ( HAMD) , Hamilton Anxiety Scale ( HAMA) and Pittsburgh Sleep Quality Index ( PSQL) were used to evaluate the family members of the two groups of patients before and after the intervention. Results: Before the intervention, there was no statistical significance difference in the scores of HAMA, HAMD and PSQI between the two groups (P>0. 05). After the intervention, the scores of HAMA, HAMD and PSQI in the observation group were significantly lower than those before the intervention ( P <0 . 05 ); and the scores of HAMA, HAMD and PSQI in the observation group were significantly lower than those in the control group ( P<0 . 05 ) . Conclusion:Cognitive-behavior therapy can significantly improve the negative emotions of depression, anxiety and sleep disorder among family members of terminal tumor patients.