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.

Objective·To preliminarily investigate the value of high-order functional magnetic resonance imaging in the evaluation of benign and malignant bone and soft tissue tumors and the changes after chemotherapy.Methods·Patients clinically diagnosed with bone and soft tissue tumors at the Department of Orthopaedics,Shanghai Ninth People's Hospital,Shanghai Jiao Tong University School of Medicine,from October 2014 to December 2024 were enrolled.The patients were divided into a control group and an amide proton transfer-weighted imaging(APTw)group according to the imaging method.All patients underwent conventional magnetic resonance imaging(MRI),diffusion-weighted imaging(DWI),and dynamic contrast-enhanced imaging(DCE)before surgery.Patients in the APTw group received additional APTw imaging.Both groups were divided into non-malignant and malignant lesion subgroups according to pathological results.According to whether the patients received chemotherapy before enrollment,the patients with malignant lesions in the APTw group were further divided into malignant group without chemotherapy and malignant group with chemotherapy.Clinical and imaging data,including APT values,apparent diffusion coefficient(ADC),and time-intensity curves(TICs)from the largest tumor section,were collected and analyzed to assess the diagnostic performance of APTw,DWI,and DCE,and to evaluate changes after chemotherapy.Results·Eighty-five patients were enrolled,including 51 males and 34 females,with ages ranging from 10 to 84 years,and a mean age of(43.05±17.62)years.There were 51 patients in the control group(16 with non-malignant lesions and 35 with malignant lesions)and 34 patients in the APTw group(5 with non-malignant lesions and 29 with malignant lesions;23 malignant lesions without chemotherapy and 6 malignant lesions with chemotherapy).The clinical and imaging data showed that only the tumor margin of the control group and the maximum tumor diameter of the APTw group had statistically significant differences in their malignant and non-malignant lesion groups(P<0.05).In the APTw group,there was a statistically significant difference in APT values between the malignant lesion group and the non-malignant lesion group(P<0.001).Further analysis showed that the APT values in the malignant group without chemotherapy were significantly lower than that in the malignant group with chemotherapy(P<0.001).However,there were no statistically significant differences in APT values between the malignant group with chemotherapy and the non-malignant lesion group(P>0.05).There were no significant differences in ADC values and TIC types between malignant and non-malignant lesion groups in the control group and the APTw group(P>0.05).The area under the curve(AUC)of the diagnostic model in the APTw group(MRI+DWI+DCE+APTw)for distinguishing malignant from benign tumors was significantly higher than that of the control group(MRI+DWI+DCE)(P<0.05).The Youden index and specificity of the diagnostic model in the APTw group were higher than those in the control group.Conclusion·As a high-order functional MRI technique,APTw imaging is capable of evaluating the nature(benign or malignant)of bone and soft tissue tumors and detecting changes after chemotherapy.It serves as a valuable supplement to conventional MRI,DWI,and DCE imaging,providing a novel noninvasive tool for the diagnosis and treatment evaluation of bone and soft tissue tumors.

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.

After ostomy surgery, patients lose the ability to control their anal sphincter, relying on ostomy pouches for excretion. Convex baseplates offer several advantages, including preventing leakage, reducing the risk of stoma and peripheral skin complications, and adapting to the dynamic stoma shapes caused by peristaltic expansion and contraction of the stoma. This article reviews the structure and features of convex baseplates, usage considerations, existing challenges, and future prospects, aiming to provide a reference for the standardized clinical use of convex baseplates and to improve patient health outcomes.

The classification of stoma skin barriers varies based on their specific features. The curvature design of convex skin barriers provides a secure and effective seal for patients with flat, retracted stomas or peristomal skin folds. The secure sealing ability of convex skin barriers is attributed to several critical structural components. Although convex skin barriers offer many clinical advantages, there is currently no unified standard for measuring their characteristics, resulting in confusion among healthcare professionals when selecting stoma care products. To address this issue, the 2021 International Stoma Care Expert Meeting proposed the Expert Consensus on Characteristics of Convex Skin Barriers and Clinical Application, which clearly defines five essential properties and clinical application guidelines for convex barriers. However, as most consensus contributors are from Europe and North America, its applicability in Chinese healthcare settings may be limited. Therefore, this paper provides a detailed interpretation of the five characteristics and clinical application statements of convex skin barriers, aiming to offer practical guidance to clinical nurses in selecting appropriate convex products and managing stoma-related complications.

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.

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.

After ostomy surgery, patients lose the ability to control their anal sphincter, relying on ostomy pouches for excretion. Convex baseplates offer several advantages, including preventing leakage, reducing the risk of stoma and peripheral skin complications, and adapting to the dynamic stoma shapes caused by peristaltic expansion and contraction of the stoma. This article reviews the structure and features of convex baseplates, usage considerations, existing challenges, and future prospects, aiming to provide a reference for the standardized clinical use of convex baseplates and to improve patient health outcomes.