Guangzhou Women and Children's Medical Center (GWCMC) established a Rare Disease Medical Center in December 2023 and assumed responsibility for the construction of the Guangdong Provincial Rare Disease Quality Control Center. As a national children's regional medical center and a core institution for maternal and child health in the Guangdong-Hong Kong-Macao Greater Bay Area, GWCMC leverages its disciplinary strengths in maternal and pediatric specialties, centers on the diagnosis and treatment needs of rare diseases in women and children, and carries out a systematic layout in such aspects as clinical diagnosis, treatment and service system, technical system and platforms, scientific research innovation and academic influence, talent echelon and capacity building, as well as quality control and regional collaboration. This paper reviews the development trajectory of the GWCMC Rare Disease Center, summarizes its phased experience in high-quality development, and offers reflections on future directions.

Intervertebral disc degeneration （IVDD） is the core cause of chronic low back pain， which severely impairs patients’ quality of life and imposes a heavy social and medical burden. The hypoxia-pyroptosis-inflammation cascade mediated by hypoxia-inducible factor-1α （HIF-1α） is the core pathological mechanism driving the initiation and progression of IVDD. Natural active ingredients derived from traditional Chinese medicine （TCM） have become a research hotspot in the field of IVDD prevention and treatment due to their advantages of multi-target effects， favorable efficacy， and low toxicity. This paper systematically reviews the mechanism of HIF-1α-mediated hypoxia-pyroptosis-inflammation cascade in degenerative nucleus pulposus tissue and the intervention of related active ingredients. It is found that natural active ingredients such as baicalein， curcumin and resveratrol can intervene in the HIF-1α-mediated pathological cascade through four core links to delay IVDD progression： targeting the HIF-1α oxygen sensing pathway to block the initiation of pyroptosis cascade， inhibiting NOD-like receptor protein 3 inflammasome activation to cut off the cascade amplification of inflammatory signals， intervening in the Gasdermin D-mediated pyroptosis execution stage to protect cell membrane integrity， and regulating extracellular matrix metabolism to reconstruct intervertebral disc homeostasis.

ObjectiveTo observe the effect of heat-sensitive moxibustion on quality of life and immune function in non-small cell lung cancer （NSCLC） patients undergoing chemotherapy. MethodsSeventy NSCLC patients with qi deficiency and phlegm stasis syndrome were randomly divided into an intervention group and a control group， with 35 cases in each group. The control group received chemotherapy combined with routine symptomatic treatment， while the intervention group additionally received heat-sensitive moxibustion since the first day of chemotherapy. Acupoints included Dazhui （GV14）， bilateral Feishu （BL13）， Zhongwan （CV12）， Qihai （CV6）， and Guanyuan （CV4）. The site exhibiting the strongest heat-sensitization response was selected for moxibustion. Treatment was administered for 45 minutes per session， three times weekly for three consecutive weeks， totaling nine sessions. Before and after treatment， quality of life was assessed using the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire （EORTC QLQ-C30）， and traditional Chinese medicine （TCM） syndrome scores were evaluated. Peripheral blood levels of natural killer （NK） cells and T-lymphocyte subsets including CD3⁺， CD4⁺， and CD8⁺， and CD4⁺/CD8⁺ ratio were measured. Levels of programmed cell death protein-1 （PD-1）， including PD-1⁺CD4⁺ and PD-1⁺CD8⁺ cells， were also assessed. Liver and renal function were monitored before and after treatment， and adverse events were recorded. ResultsIn the intervention group， 1 participant withdrew and 1 was excluded， while in the control group， 2 participants withdrew. Ultimately， 33 participants in each group were included in the final analysis. The intervention group showed significant improvements in physical， role， emotional， cognitive， and social functioning， as well as global health status after treatment， while scores for fatigue， nausea and vomiting， dyspnea， appetite loss， diarrhea， and TCM syndrome scale were significantly decreased （P＜0.05）. Moreover， the intervention group demonstrated higher scores in physical functioning， role functioning， and global health status， as well as lower scores for fatigue， nausea and vomiting， dyspnea， appetite loss， diarrhea， and the TCM syndrome scale than the control group （P＜0.05）. After treatment， the levels of peripheral NK cells and PD-1⁺CD8⁺ T cells in the intervention group increased significantly； furthermore， the intervention group exhibited higher peripheral NK cell levels and lower PD-1⁺CD8⁺ T cell levels than the control group （P＜0.05）. No significant differences were found in liver or renal function between the two groups （P＞0.05）. In addition， no adverse events such as burns or moxibustion-induced syncope occurred during the study. ConclusionHeat-sensitive moxibustion as an adjunctive therapy may enhance immune function， alleviate clinical symptoms， and improve quality of life， while demonstrating a favorable safety profile in NSCLC patients with qi deficiency and phlegm stasis.

Triptolide （TP）， the core active component of the traditional Chinese medicine Tripterygium wilfordii ， exhibits remarkable pharmacological activities including anti-inflammatory， immunosuppressive and anti-tumor effects， and holds broad application prospects in the treatment of major diseases such as autoimmune diseases and malignant tumors. However， TP has a narrow therapeutic window and causes multi-organ toxicities including liver， kidney and reproductive toxicities， which severely restrict its safe clinical application and new drug development. Therefore， toxicity reduction and efficacy enhancement has become a core scientific problem urgently to be solved in this field. This paper systematically reviews the four core strategies for TP toxicity reduction and efficacy enhancement， including structural modification， dosage form improvement， herbal compatibility， and external therapies of traditional Chinese medicine. Among them， structural modification optimizes the toxic and efficacy characteristics of TP from the molecular structure level， with typica l derivatives including （5 R ）-5-hydroxy triptolide， ZT01， PG490-88， etc. Dosage form modification achieves toxicity reduction and efficacy enhancement via targeted and sustained-controlled drug release of diverse delivery systems. It includes triptolide preparations such as nanoparticles， liposomes， microemulsion gels and liquid crystals， possessing favorable clinical transformation potential. The herbal compatibility and external therapies of traditional Chinese medicine conform to the holistic view of traditional Chinese medicine and have a profound clinical application foundation， but their mechanisms of action are insufficiently elucidated， and they lack unified standardized specifications and high-quality evidence-based proof. In the future， we should rely on multi-omics technology to elucidate the toxic and efficacy mechanisms， integrate technologies to optimize preparations， improve the evaluation system and promote clinical transformation.

Objective : To investigate the effects of resveratrol(Res) on fibroblast-like synoviocytes(FLS) in patients with rheumatoid arthritis(RA), and to explore the possible mechanism of Res inhibiting the release of inflammatory factors from FLS. Methods : FLS from RA patients were culturedin vitroand treated with different concentrations of Res(0, 20, 40, 80, 160, 320 μmol/L). The viability of FLS cells was detected by CCK-8 assay after 12 and 24 h. The contents of inflammatory factor interleukin-6(IL-6) and tumor necrosis factor-α(TNF-α) in cell supernatant were detected by ELISA. The expression levels of cyclic guanosine monophosphate-adenosine monophosphate synthase(cGAS) and stimulator of interferon gene(STING) were measured by Western blot; After lentivirus infection with FLS caused the cells to overexpress cGAS, the cells were divided into Control group(blank control), cGAS group(cGAS overexpression), Res+cGAS group(Res 160 μmol/L+cGAS overexpression) and Res group(Res 160 μmol/L). The expression level of STING protein in cells of each group was determined by Western blot, the viability of FLS cells in each group was detected by CCK-8, and the contents of inflammatory factor IL-6 and TNF-α in the supernatant of cells of each group were detected by ELISA method. Results : The results of CCK-8 experiment showed that under 40, 80, 160 μmol/L Res treatment, FLS viability decreased significantly after 24 h compared with blank control group(P<0.01). ELISA results showed that the contents of IL-6 and TNF-α in cell supernatant were also significantly decreased after treatment with Res of 40, 80 and 160 μmol/L(P<0.01). Meanwhile, Western blot results showed that Res could significantly decrease the protein expression levels of STING and cGAS in FLS cells after treatment of 40, 80 and 160 μmol/L(P<0.05,P<0.01). Compared with the Control group, the expression level of STING protein in FLS increased after overexpression of cGAS(P<0.05); compared with the Res group, the content of inflammatory factors in the supernatant of FLS and the expression level of STING protein in FLS significantly increased after overexpression of cGAS(P<0.01,P<0.05). Conclusion The appropriate concentration of Res can inhibit the release of inflammatory cytokines in FLS cells, which may be related to the blocking of cGAS-STING signaling pathway.

With the rapid development of minimally invasive techniques in surgery, arti-ficial intelligence (AI), particularly deep learning, is playing an increasingly important role in mini-mally invasive surgery. By automated analysis of surgical videos, AI can efficiently perform key tasks such as instrument recognition, surgical phase identification, action analysis, anatomical structure recognition, intraoperative diagnosis, adverse event monitoring and smart desmoking. These appli-cations provide essential support for real-time monitoring, surgical navigation and skill assessment during surgery. The authors summarize the current research progress of AI in minimally invasive surgery, including its applications in the fields of hepatobiliary and pancreatic surgery, as well as gastrointestinal surgery. It also explores the potential of AI in enhancing surgical safety, efficiency and skill assessment. By synthesizing the latest research achievements of AI technology in the field of surgery, as well as analyzing its technical challenges and risks, it aims to provide guidance for future innovations and clinical applications, promoting the advancement and implementation of AI in minimally invasive surgery.

Objective:To investigate the current status of knowledge, attitude, and practice (KAP) of intensive care unit (ICU) medical staff for post-intensive care syndrome (PICS) and explore its influencing factors.Methods:The cross-sectional investigation study with stratified sampling was conducted. From June to September 2024, ICU medical staff from general hospitals in 5 regions (Chongqing, Beijing, Shaanxi, Jiangsu, and Gansu) were selected as the research subjects. The KAP of PICS questionnaire was distributed in the form of an electronic questionnaire. Observation indicators: (1) results of the questionnaire survey; (2) general information of ICU medical staff; (3) KAP scores of PICS and the correlation among various dimensions; (4) analysis of influencing factors for KAP of PICS. Comparison of measurement data with normal distribution between groups was conducted using the independent samples t test. One-way analysis of variance (ANOVA) was applied for com-parison among multiple groups, and post-hoc LSD test was used for pairwise comparison. Comparison of count data between groups was conducted using the chi-square test. Pearson correlation analysis was adopted for correlation analysis. Multiple linear regression analysis was used for univariate and multivariate analyses. Results:(1) Results of questionnaire survey. A total of 410 questionnaires were distributed and retrieved, among which 408 were valid, with an effective rate of 99.512%(408/410). (2) General information of ICU medical staff. Among the 408 ICU medical staff, there were 79 males and 329 females. Eight cases were under 25 years old, 248 cases were 25-35 years old, 132 cases were 36-40 years old, and 20 cases were over 40 years old. In terms of professional title, there were 10 junior nurses, 130 junior nurse practitioners, 228 intermediate nurse practitioners, and 40 senior nurse practitioners. About the educational background, 34 cases had a junior college degree, 347 cases had a bachelor's degree, and 27 cases had a master's degree or above. Regarding the hospital level, 25 nurses worked in secondary hospitals and 383 cases in tertiary hospitals. In terms of ICU type, 181 cases were from specialized ICU and 227 cases from general ICU. About working experience in ICU, 41 nurses had less than 5 years, 207 cases had 5-10 years, and 160 cases had more than 10 years. (3) KAP scores of PICS and the correlation among various dimensions. The total KAP score of PICS among the 408 ICU medical staff was 88.7±14.2, with 40.2±9.2 for the knowledge dimension, 22.0±5.6 for the attitude dimension, and 26.5±6.3 for the practice dimension. Pearson correlation analysis showed that the knowledge dimension of PICS among ICU medical staff was significantly positively correlated with both the attitude dimension and the practice dimension ( r=0.15, 0.69, P<0.05); the attitude dimension was positively correlated with the practice dimension ( r=0.23, P<0.05).(4) Analysis of influencing factors for KAP of PICS. Results of multivariate analysis showed that age (25-35 years old, 36-40 years old, over 40 years old), educational background and hospital level were independent influencing factors for the KAP of PICS among ICU medical staff ( t=2.23, 1.97, 2.84, 0.15, 2.04, P<0.05). Conclusions:The KAP of PICS among ICU medical staff is relatively good, while their practical ability still needs to be improved. Age, educational background, and hospital level are independent influencing factors for the KAP of PICS among ICU medical staff.

BACKGROUND:In the follow-up after unicompartmental knee arthroplasty,some patients have knee pain,among which postmenopausal obese women are the most common. As an important index to measure the degree of body obesity,whether body mass index is related to the curative effect after unicompartmental knee arthroplasty and whether obesity will affect the function of knee joint after operation are worthy of further study.OBJECTIVE:To evaluate the clinical efficacy of postmenopausal obese women undergoing medial unicompartmental knee arthroplasty,and to determine the influence of body mass index on the quality of life after unicompartmental knee arthroplasty.METHODS:Female postmenopausal patients who underwent medial unicompartmental knee arthroplasty for the first time due to medial knee pain from January 2017 to January 2019 in the Fourth Clinical Medical College of Xinjiang Medical University were enrolled. A total of 270 cases were included according to inclusion and exclusion criteria,and were divided into 4 groups according to preoperative body mass index:There were 42 cases in normal group (body mass index 18.5-22.9 kg/m2),58 cases in overweight group (body mass index 23.0-24.9 kg/m2),122 cases in obese group (body mass index 25.0-29.9 kg/m2),and 48 cases in severely obese group (body mass index ≥30 kg/m2). Hospital for Special Surgery score,Western Ontario and McMaster Universities Osteoarthritis Index score,knee range of motion,visual analog scale score,and hip-knee-ankle angle were compared before,after and at the last time in each group. Patients were followed up to record the time of use of prostheses after surgery and reasons for failure or revision. The effective utilization rate of prostheses was calculated and compared in each group. Survival curve was used for statistical analysis of the effective utilization rate of prostheses.RESULTS AND CONCLUSION:(1) There was no significant difference in postoperative follow-up time,knee joint range of motion,visual analog scale score,and hip-knee-ankle angle between the groups (P>0.05). (2) The Hospital for Special Surgery score and Western Ontario and McMaster Universities Osteoarthritis Index score of each group in final follow-up were significantly improved compared with those before surgery (P<0.05),and the postoperative effect was obvious in each group (P<0.05). Regarding Hospital for Special Surgery score,the improvement effect was worse in the severely obese group. (3) The comparison of hip-knee-ankle angle between all groups immediately after surgery and the last follow-up showed that there were significant differences between the other groups at two time points (P<0.05) except the normal group (P>0.05). (4) The effective utilization rate of prosthesis in normal,overweight,obesity,and severely obese groups was 100％,95％,94％,and 94％,respectively,and there was no significant difference between the groups (x2=2.532,P=0.469). (5) It is indicated that body mass index of postmenopausal obese women had no significant effect on the effective utilization rate of medial unicompartmental prosthesis. Obesity is an important factor affecting the postoperative knee function score and the effective utilization rate of prosthesis.Weight should be properly controlled before and after unicompartmental knee arthroplasty. At the same time,female body mass index ≥ 30 kg/m2 is not the best indication for unicompartmental knee arthroplasty. It is suggested that female patients undergoing unicompartmental knee arthroplasty should controlbody mass index below 30 kg/m2.

Objective:To analyze the current status of red blood cell transfusion in very preterm infants (VPI) (gestational age at birth <32 weeks) from Chinese Neonatal Network (CHNN) in 2022.Methods:This cross-sectional study was based on the CHNN VPI cohort. It included 6 985 VPI admitted to CHNN 89 participating centers within 24 hours after birth in 2022. VPI with major congenital anomalies or those transferred to non-CHNN centers for treatment or discharged against medical advice were excluded. VPI were categorized based on whether they received red blood cell transfusions, their gestational age at birth, the type of respiratory support received during transfusion, and whether the pre-transfusion hemoglobin levels exceeded the thresholds. General characteristics, red blood cell transfusion rates, number of transfusions, timing of the first transfusion, and pre-transfusion hemoglobin levels were compared among different groups. The incidence of adverse outcomes between the group of VPI who received transfusions above the threshold and those who received transfusions below the threshold were compared. Comparison among different groups was conducted using χ2 tests, Kruskal-Wallis H tests, Mann-Whitney U test, and so on. Trends by gestational age at birth were evaluated by Cochran-Armitage tests and Jonckheere-Terpstra tests for trend. Results:Among the 6 985 VPI, 3 865 cases(55.3%) were male, with a gestational age at birth of 30.0 (28.6, 31.0) weeks and a birth weight of (1 302±321) g. Overall, 3 617 cases (51.8%) received red blood cell transfusion, while 3 368 cases (48.2%) did not. The red blood cell transfusion rate was 51.8% (3 617/6 985), with rates of 77.7% (893/1 150) for those born before 28 weeks gestational age and 46.7% (2 724/5 835) for those born between 28 and 31 weeks gestational age. A total of 9 616 times red blood cell transfusions were administered to 3 617 VPI, with 632 times missing pre-transfusion hemoglobin data, and 8 984 times included in the analysis. Of the red blood cell transfusions, 25.6% (2 459/9 616) were administered when invasive respiratory support was required, 51.3% (4 934/9 616) were receiving non-invasive respiratory support, while 23.1% (2 223/9, 616) were given when no respiratory support was needed. Compared to the non-transfusion group, the red blood cell transfusion group had a higher rate of pregnancy-induced hypertension in mothers, lower rates of born via cesarean section and mother′s antenatal steroid administration, smaller gestational age, lower birth weight, a higher proportion of small-for-gestational-age, multiple births, and proportions of Apgar score at the 5 th minute after birth ≤3 (all P<0.05). They were also less likely to be female, born in hospital or undergo delayed cord clamping (all P<0.01). Additionally, higher transport risk index of physiologic stability score at admission were observed in the red blood cell transfusion group ( P<0.001). The number of red blood cell transfusion was 2 (1, 3) times, with the first transfusion occurring at an age of 18 (8, 29) days, and a pre-transfusion hemoglobin level of 97 (86, 109) g/L. For VPI ≤7 days of age, the pre-transfusion hemoglobin levels for invasive respiratory support, non-invasive respiratory support, or no respiratory support, respectively, with no statistically significant differences between groups ( H=5.59, P=0.061). For VPI aged 8 to 21 days and≥22 days, the levels with statistically differences between groups (both P<0.01). Red blood cell transfusions above recommended thresholds were observed in all respiratory support categories at different stages of life, with the highest prevalence in infants aged 8 to 21 days and≥22 days who did not require respiratory support, at 90.1% (264/273) and 91.1%(1 578/1 732), respectively. The rate of necrotizing enterocolitis was higher in the above-threshold group ( χ2=10.59, P=0.001), and the duration of hospital stay was longer in the above-threshold group ( Z=4.67, P<0.001) compared to the below-threshold group. Conclusions:In 2022, the red blood cell transfusion rate was relatively high among VPI from CHNN. Pre-transfusion hemoglobin levels frequently exceeded recommended transfusion thresholds.

OBJECTIVE To study the pharmacokinetic characteristics of ciprofol in pregnant and fetal rats， and provide reference for the application of ciprofol in cesarean section. METHODS Eight pregnant rats were selected. A single dose of 2.4 mg/kg of ciprofol was administered via the tail vein. One fetal rat was selected at 2， 4， 8， 12， 16， 25， 35， 45， 60， and 90 minutes respectively after ciprofol administration. Subsequently， whole blood samples were collected simultaneously from both the pregnant rats and fetal rats. HPLC-MS/MS method was used to determine the concentration of ciprofol in the bodies of pregnant and fetal rats. The ratios of fetal-to-maternal blood concentrations （F/M ratios） at each time point were calculated， and the F/M-time curves were plotted. Subsequently， non-compartmental pharmacokinetic parameters were computed using DAS 2.0 software. RESULTS Compared with pregnant rats， cmax， AUC0-90 min and AUC0-∞ of ciprofol in fetal rats were decreased significantly， while MRT was increased significantly （P＜0.05）. The F/M curve of ciprofol initially increased and then decreased， and between 0.16- 0.84， reaching a maximum value of 0.84 at 45 minutes. CONCLUSIONS Ciprofol can penetrate the placental barrier， and there are significant differences in pharmacokinetic parameters between pregnant and fetal rats. Moreover， the exposure level of ciprofol in fetal rats is much lower than that in pregnant rats. Therefore， ciprofol shows promise as an ideal anesthetic agent for cesarean section delivery.