Congenital ectopialentis(CEL)is a significant cause of blindness-related disease in children and adolescents, with a highly heterogeneous etiology. It can manifest either as a prominent ocular phenotype of connective tissue disorders such as Marfan syndrome or as an isolated condition. Emerging evidence indicates that zonular microfibrils and extracellular matrix homeostasis represent the core pathological basis. Variants in genes including FBN1, ADAMTSL4, LTBP2, ADAMTS10/17, ASPH, and SUOX lead to lens malposition and anterior segment remodeling by disrupting microfibril assembly, anchoring, and signaling regulation. Clinically, after excluding secondary causes, a stratified diagnosis based on phenotypic presentation should be pursued. Genetic testing can adopt a stepwise strategy of “panel-first, supplemented by whole-exome/whole-genome sequencing(WES/WGS)”, combined with family-based follow-up and reassessment. This review synthesizes current knowledge on the pathogenic basis, genetic spectrum and genotype-phenotype correlations, diagnostic workflows, and testing strategies, and presents a clinically oriented stratified diagnostic framework aimed at improving early recognition and systemic risk management.

ObjectiveTo establish three standardized rehabilitation unit models covering acute, critical and comprehensive recovery stages for traumatic brain injury (TBI) that fit the whole-course characteristics, clarify their multidisciplinary collaborative framework, standardized procedures, and information management system, and verify the clinical effectiveness of this staged rehabilitation unit on long-term functional prognosis and in-hospital complications of TBI patients. MethodsThis study was conducted at two stages: model construction and efficacy verification. At the first stage, TBI rehabilitation units for acute, critical and recovery stages were established in the Department of Neurosurgery, Intensive Care Unit and Department of Rehabilitation Medicine, respectively, with unified multidisciplinary team configuration, standardized assessment and treatment protocols, collaborative workflows, and a dedicated information management system. At the second stage, 324 TBI patients admitted to the newly built rehabilitation units from July, 2023 to July, 2025 were enrolled as the observation group, and 167 TBI patients receiving traditional management from January, 2018 to December, 2022 were retrospectively selected as the control group. The primary outcomes were 1-year post-injury Glasgow Outcome Scale (GOS) and Disability Rating Scale (DRS) scores. Secondary outcomes included the incidence of in-hospital complications and the mean indwelling time of tracheal intubation, gastric tube and urinary catheter. ResultsThe 1-year GOS score was significantly higher (t = -7.836, P < 0.001), and the DRS score was significantly lower (t = -6.742, P < 0.001) in the observation group than in the control group. The overall incidence of complications was significantly lower in the observation group than in the control group (χ² = 18.762, P < 0.001). The incidences of pulmonary infection, urinary tract infection, pressure ulcer, lower extremity venous thrombosis and joint contracture deformity were all significantly reduced (χ² > 4.328, P < 0.05). The indwelling time of tracheal intubation, gastric tube and urinary catheter was significantly shorter in the observation group than in the control group (t > 10.276, P < 0.001). ConclusionThe acute-critical-comprehensive three-stage standardized TBI rehabilitation unit could significantly improve the long-term functional outcomes of TBI patients, reduce the incidence of complications, and shorten the indwelling time of invasive tubes through multidisciplinary collaboration, whole-course standardized management and information-based quality control. This model conforms to the pathological and functional laws of TBI, realizes seamless whole-course connection from acute treatment to recovery rehabilitation, and has good clinical feasibility and promotion value. It can provide evidence-based evidence and practical reference for the homogenized and standardized development of TBI rehabilitation in China.

Background and Objectives: The Fractional Flow Reserve and Intravascular UltrasoundGuided Intervention Strategy for Clinical Outcomes in Patients with Intermediate Stenosis (FLAVOUR) trial demonstrated non-inferiority of fractional flow reserve (FFR)-guided percutaneous coronary intervention (PCI) compared with intravascular ultrasound (IVUS)-guided PCI. We sought to investigate the cost-effectiveness of FFR-guided PCI compared to IVUS-guided PCI in Korea. Methods: A 2-part cost-effectiveness model, composed of a short-term decision tree model and a long-term Markov model, was developed for patients who underwent PCI to treat intermediate stenosis (40% to 70% stenosis by visual estimation on coronary angiography).The lifetime healthcare costs and quality-adjusted life-years (QALYs) were estimated from the healthcare system perspective. Transition probabilities were mainly referred from the FLAVOUR trial, and healthcare costs were mainly obtained through analysis of Korean National Health Insurance claims data. Health utilities were mainly obtained from the Seattle Angina Questionnaire responses of FLAVOUR trial participants mapped to EQ-5D. Results: From the Korean healthcare system perspective, the base-case analysis showed that FFR-guided PCI was 2,451 U.S. dollar lower in lifetime healthcare costs and 0.178 higher in QALYs compared to IVUS-guided PCI. FFR-guided PCI remained more likely to be cost-effective over a wide range of willingness-to-pay thresholds in the probabilistic sensitivity analysis. Conclusions Based on the results from the FLAVOUR trial, FFR-guided PCI is projected to decrease lifetime healthcare costs and increase QALYs compared with IVUS-guided PCI in intermediate coronary lesion, and it is a dominant strategy in Korea.

Background and Objectives: The Fractional Flow Reserve and Intravascular UltrasoundGuided Intervention Strategy for Clinical Outcomes in Patients with Intermediate Stenosis (FLAVOUR) trial demonstrated non-inferiority of fractional flow reserve (FFR)-guided percutaneous coronary intervention (PCI) compared with intravascular ultrasound (IVUS)-guided PCI. We sought to investigate the cost-effectiveness of FFR-guided PCI compared to IVUS-guided PCI in Korea. Methods: A 2-part cost-effectiveness model, composed of a short-term decision tree model and a long-term Markov model, was developed for patients who underwent PCI to treat intermediate stenosis (40% to 70% stenosis by visual estimation on coronary angiography).The lifetime healthcare costs and quality-adjusted life-years (QALYs) were estimated from the healthcare system perspective. Transition probabilities were mainly referred from the FLAVOUR trial, and healthcare costs were mainly obtained through analysis of Korean National Health Insurance claims data. Health utilities were mainly obtained from the Seattle Angina Questionnaire responses of FLAVOUR trial participants mapped to EQ-5D. Results: From the Korean healthcare system perspective, the base-case analysis showed that FFR-guided PCI was 2,451 U.S. dollar lower in lifetime healthcare costs and 0.178 higher in QALYs compared to IVUS-guided PCI. FFR-guided PCI remained more likely to be cost-effective over a wide range of willingness-to-pay thresholds in the probabilistic sensitivity analysis. Conclusions Based on the results from the FLAVOUR trial, FFR-guided PCI is projected to decrease lifetime healthcare costs and increase QALYs compared with IVUS-guided PCI in intermediate coronary lesion, and it is a dominant strategy in Korea.

Background and Objectives: The Fractional Flow Reserve and Intravascular UltrasoundGuided Intervention Strategy for Clinical Outcomes in Patients with Intermediate Stenosis (FLAVOUR) trial demonstrated non-inferiority of fractional flow reserve (FFR)-guided percutaneous coronary intervention (PCI) compared with intravascular ultrasound (IVUS)-guided PCI. We sought to investigate the cost-effectiveness of FFR-guided PCI compared to IVUS-guided PCI in Korea. Methods: A 2-part cost-effectiveness model, composed of a short-term decision tree model and a long-term Markov model, was developed for patients who underwent PCI to treat intermediate stenosis (40% to 70% stenosis by visual estimation on coronary angiography).The lifetime healthcare costs and quality-adjusted life-years (QALYs) were estimated from the healthcare system perspective. Transition probabilities were mainly referred from the FLAVOUR trial, and healthcare costs were mainly obtained through analysis of Korean National Health Insurance claims data. Health utilities were mainly obtained from the Seattle Angina Questionnaire responses of FLAVOUR trial participants mapped to EQ-5D. Results: From the Korean healthcare system perspective, the base-case analysis showed that FFR-guided PCI was 2,451 U.S. dollar lower in lifetime healthcare costs and 0.178 higher in QALYs compared to IVUS-guided PCI. FFR-guided PCI remained more likely to be cost-effective over a wide range of willingness-to-pay thresholds in the probabilistic sensitivity analysis. Conclusions Based on the results from the FLAVOUR trial, FFR-guided PCI is projected to decrease lifetime healthcare costs and increase QALYs compared with IVUS-guided PCI in intermediate coronary lesion, and it is a dominant strategy in Korea.

ObjectiveTo analyze the epidemiological characteristics of foodborne disease outbreaks in Wuhan from 2006 to 2023, and to provide a scientific basis for the development of foodborne disease prevention and control measures. MethodsDescriptive statistical analyses were performed on foodborne disease outbreaks confirmed by the district and municipal center for disease control and prevention (CDC) in Wuhan from 2006 to 2023, and the attack rate and case fatality rate were calculated as well. ResultsA total of 182 foodborne disease outbreaks were reported in Wuhan from 2006 to 2023, with a cumulative of 2 820 cases. Among which, 3 cases were dead, with an annual average attack rate of 1.22% and a case fatality rate of 0.11%. The highest number of outbreaks occurred in collective canteens (43.96%, 80/182), the highest attack rate was observed in catering facilities (11.03%), and the highest case fatality rate was found in households (1.45%). Among the foodborne disease outbreaks with identified etiologies, microbial factors were the leading causes (36.26%, 66/182), with the main pathogens being norovirus, Bacillus cereus, and other unspecified bacteria. Fungal factors were mainly attributed to poisonous mushrooms, with a relatively high fatality rate of 2.22% (2/90). Outbreaks caused by bacterial factors were more common in the central urban area (30.28%, 33/109), while fungal-related outbreaks were more frequent in the outlying urban areas (24.66%, 18/73). ConclusionCollective canteens are the main venues for foodborne disease outbreaks in Wuhan. Microbial factors are the main pathogenic factors, and poisonous mushrooms are the leading causes to death. It is necessary to strengthen the supervision on collective canteens, carry out various forms of public awareness campaigns on poisonous mushroom poisoning, and, if required, cooperate with the gardening department to eradicate wild poisonous mushrooms in the green belts. A collaborative cooperation involving multiple departments is essential to reduce the occurrence of foodborne disease outbreaks.

Objective To analyze the efficacy of CT-guided alcohol ablation of splanchnic nerve block(SNB)in the treatment of pain in patients with pancreatic cancer,and to discuss the related factors influencing the effectiveness in treating pancreatic cancer pain.Methods The clinical data of patients with pancreatic cancer pain,who was unable to receive surgery and underwent CT-guided SNB at Hai'an People's Hospital from June 2018 to September 2023,were retrospectively analyzed.According to postoperative one-month VAS pain score and the used dosage of opioids,the improvement of pain was assessed.Logistic regression analysis was used to evaluate the demographic characteristics,tumor features,and pain-related factors so as to determine the predictive factors for effective analgesia after SNB,and the survival outcomes were compared between the patients with effective SNB analgesia and the patients with ineffective SNB analgesia.Results A total of 133 patients were included in this analysis.One week after SNB,74.4％of patients achieved effective pain relief,and one month after the procedure,54.9％of patients experienced effective pain relief.One month after treatment,multivariate logistic regression analysis showed that the diabetes(OR=0.637,95％CI=0.397-0.876,P=0.034),serum CA199(OR=1.531,95％CI=1.030-2.370,P=0.042),serum MMP-1(OR=0.703,95％CI=0.457-0.983,P=0.038),preoperative ECOG score(OR=2.693,95％CI=1.234-6.609,P=0.021),and preoperative daily morphine consumption(OR=2.797,95％CI=1.148-6.695,P=0.019)were the independent predictive factors for achieving a good analgesic efficacy after SNB.Patients with poor analgesic outcomes after SNB had a significantly lower median survival time when compared to patients with good analgesic outcomes(82 days vs 149 days,P＜0.05).No serious SNB-related adverse events occurred.Conclusion For the treatment of refractory pancreatic cancer pain,CT-guided SNB is clinically safe and effective.The diabetes,ECOG score,serum MMP-1 level,CA199 level,and preoperative daily used dosage of opioids are the independent predictive factors for the efficacy of CT-guided SNB in treating pancreatic cancer pain.

Objective:To investigate whether sivelestat sodium (SV) mitigates paraquat (PQ)-induced acute lung injury (ALI)/acute respiratory distress syndrome (ARDS)-associated pulmonary fibrosis in mice by suppressing the NLRP3 inflammasome signaling pathway.Methods:Male C57BL/6J mice were randomly divided into four groups ( n=8 per group): Control group, PQ group, PQ+SV group, and SV group. The PQ and PQ+SV groups received an intraperitoneal injection of PQ solution (20 mg/kg) to establish a PQ poisoning model, while the Control and SV groups received an equivalent volume of saline. One hour later, the PQ+SV and SV groups were administered SV solution (100 mg/kg) intraperitoneally, whereas the Control and PQ groups received saline. After 48 hours, the mice were euthanized, and lung tissues were collected. Pathological changes were assessed via hematoxylin-eosin (HE) and Masson staining, followed by Smith and Ashcroft scoring. Immunohistochemistry was performed to evaluate the expression of NLRP3 inflammasome, caspase-1, α-smooth muscle actin (α-SMA), and collagen I. Western blotting was used to measure NLRP3 protein levels. Intergroup comparisons were conducted using one-way ANOVA with LSD post-hoc correction. Results:The Control and SV groups exhibited normal lung morphology, whereas the PQ+SV group showed reduced hemorrhage, congestion, and edema compared to the PQ group. Both PQ and PQ+SV groups exhibited significant weight loss post-intervention compared to the Control group (both P<0.001). HE and Masson staining revealed thickened alveolar septa, congestive and edematous alveolar walls, extensive inflammatory cell infiltration, and collagen deposition in the PQ group. In contrast, the PQ+SV group demonstrated alleviated alveolar wall congestion, reduced inflammatory infiltration, and decreased collagen deposition, with significantly lower Smith and Ashcroft scores [(5.92±1.34) vs. (10.88±1.88), P<0.001; (3.42±1.35) vs. (5.75±0.79), P<0.001]. Immunohistochemistry indicated reduced expression percentages of NLRP3 and caspase-1 in the PQ+SV group compared to the PQ group [(12.79%±0.43%) vs. (16.59%±0.40%), P<0.001; (17.71%±0.92%) vs. (19.84%±0.71%), P<0.001]. Similarly, α-SMA and collagen I expression in lung interstitium was significantly lower in the PQ+SV group [(11.79%±0.58%) vs. (16.14%±0.74%), P<0.001; (16.43%±0.56%) vs. (18.86%±0.60%), P<0.001]. Western blotting confirmed decreased NLRP3 protein expression in the PQ+SV group [(0.54±0.12) vs. (0.81±0.24), P<0.05]. Conclusions:SV attenuates PQ-induced ALI/ARDS-associated pulmonary fibrosis progression by inhibiting the NLRP3 inflammasome, suggesting that NLRP3 may be a key therapeutic target for early intervention in PQ-induced pulmonary fibrosis.

Strengthening the construction of platforms,talents,and disciplines is a crucial strategy to enhance the core competitiveness and promote the high-quality development of national regional medical centers.It outlines the theoretical framework and implementation path for building the"Platform-Talent(Team)-Discipline"develop-ment system during the establishment of the National Regional Medical Center at Henan Children's Hospital Zheng-zhou Children's Hospital.By creating a collaborative innovation platform integrating medical services,education,and research,implementing talent development programs,optimizing mechanisms for talent recruitment,cultivation,and retention,and advancing discipline development projects,the center has achieved significant progress in its core competencies.This exploration provides valuable insights and references for the development of national regional medical centers and the high-quality growth of public hospitals.

Objective To develop an assessment scale for post-intensive care syndrome in pediatrics(PICS-p)and evaluate its reliability and validity,aiming to provide a scientifically sound and feasible tool for clinical assessment.Methods Based on the PICS-p conceptual framework,combined with literature review,semi-structured interviews,and Delphi expert consultation,a preliminary scale was developed.From June to December 2023,a survey was conducted among 330 pediatric patients who were discharged from a tertiary children's hospital in Hangzhou,followed by a two-week retest with 30 children to assess the reliability and validity of the scale.Results Finally 304 children completed the survey.The Post-Intensive Care Syndrome Scale for Children consists of 4 dimensions of physical dysfunction,cognitive dysfunction,psychological dysfunction and social dysfunction,with a total of 29 entries,with a cumulative variance contribution of 61.705％.The content validity index for individual items ranged from 0.800 to 1.000,and the content validity index for the scale as a whole was 0.98.The dimensions of the scale showed positive correlations with the Functional Status Scale(r=0.438-0.581,P＜0.001).The overall Cronbach's α coefficient of the scale was 0.847;the split-half reliability was 0.868;the test-retest reliability was 0.832.Conclusion The scale demonstrates good reliability and validity,and it is suitable for assessing the severity of PICS-p in pediatric patients discharged from the PICU.