Homozygous familial hypercholesterolemia (HoFH) is an extremely rare and severe hereditary lipid metabolism disorder, characterized by markedly elevated levels of plasma low-density lipoprotein cholesterol (LDL-C), significantly increasing the risk of atherosclerotic cardiovascular diseases. Among traditional lipid-lowering therapies, the combination of statins and ezetimibe is the basic treatment approach, but its efficacy is limited. In recent years, notable progress has been made in lipid-lowering therapy for HoFH. New drugs such as proprotein convertase subtilisin/kexin type 9 inhibitors and angiopoietin-like protein 3 inhibitors have demonstrated favorable LDL-C-lowering effects and play an active role in treatment. Lipoprotein apheresis can rapidly reduce LDL-C levels and has become an important adjuvant treatment modality. Although approaches like gene therapy and liver transplantation face many challenges, they offer hope for radical cure. Further research is still needed to optimize treatment strategies and regimens for more effective blood lipid management in patients with HoFH.

Objective To explore the research status,hotspots and trends in the field of traditional Chinese medicine in the treatment of dysfunctional uterine bleeding(DUB).Methods Relevant literatures on the treatment of DUB with traditional Chinese medicine were retrieved from China National Knowledge Infrastructure,Wanfang Data Knowledge Service Platform and VIP.Multi-dimensional visual analysis was conducted on the annual number of published papers,authors and institutions,and keywords using CiteSpace software.Results A total of 1649 literatures were included according to the inclusion and exclusion criteria.The top three authors in terms of the number of published articles are You Zhaoling,Ma Huirong,and Wu Yanan.Shandong University of Traditional Chinese Medicine is the institution that has published the most papers.The research on the treatment of DUB with traditional Chinese medicine mainly focuses on the integrated treatment of traditional Chinese and Western medicine,perimenopause,adolescence,therapeutic effect observation,Guchong decoction,sex hormones,and so on.Conclusion The research on the treatment of DUB with traditional Chinese medicine mainly focuses on the application of traditional Chinese medicine prescriptions,clinical observation and therapeutic effect indicators,etc.Subsequently,in-depth exploration of new traditional Chinese medical therapies and new observation indicators is still needed.In the future,the observation of new microscopic indicators and the research on pathological mechanisms may be potential research hotspots and directions.

Objective:To investigate the impact of receptor-interacting protein kinase 3 (RIPK3) on major adverse cardiovascular events (MACE) in patients with acute myocardial infarction (AMI) after percutaneous coronary intervention (PCI), as well as the predictive performance of RIPK3 combined with traditional cardiovascular risk factors.Methods:This study was a single-center prospective cohort study. It included patients with AMI who underwent PCI at Peking Union Medical College Hospital between September 2017 and November 2017. Baseline clinical data were collected, and plasma samples were obtained 6 hours after PCI to measure RIPK3 levels. Follow-up was conducted via outpatient visits or phone calls to record the occurrence of MACE, including cardiovascular death, hospitalization for heart failure, and vascular events (recurrent AMI or stroke). The predictive performance of RIPK3, traditional cardiovascular risk factors and their combination for MACE was compared using receiver operating characteristic (ROC) curves. Patients were divided into low- and high-RIPK3 level groups based on the optimal cutoff value of RIPK3. Multivariate Cox proportional hazards regression analysis was used to assess the impact of RIPK3 levels on MACE after PCI in AMI patients. Kaplan-Meier survival curves were plotted, and the log-rank test was used to compare MACE incidence between the low-and high-RIPK3 groups.Results:A total of 103 AMI patients who underwent PCI were included, aged 63.0 (56.0, 69.0) years, and 83 (80.6%) were male. The follow-up time was 5.17 (2.81, 5.17) years, during which 44 patients (42.7%) experienced MACE. The ROC curve analysis showed that the area under the curve ( AUC) for traditional cardiovascular risk factors was 0.68 (95% CI: 0.58-0.78), while the AUC for plasma RIPK3 was 0.72 (95% CI: 0.62-0.82). The combined AUC for traditional risk factors and RIPK3 was 0.75 (95% CI: 0.65-0.85). Multivariate Cox proportional hazards regression analysis indicated that plasma RIPK3 level is greater than or equal to the optimal cutoff value of 440.9 μg/L ( HR=3.31, 95% CI: 1.53-8.30, P=0.005) was an independent risk factor for MACE in AMI patients after PCI. Kaplan-Meier survival analysis demonstrated that the high-RIPK3 group had a significantly higher risk of MACE after PCI compared to the low-RIPK3 group (log-rank P=0.006). Conclusions:Elevated plasma RIPK3 level is an independent risk factor for MACE in AMI patients after PCI. Plasma RIPK3 combined with traditional cardiovascular risk factors can more effectively predict the occurrence of MACE in AMI patients after PCI. AMI patients with RIPK3≥440.9 μg/L have a higher risk of MACE after PCI.

Objective:To explore the relationship between retinol-binding protein (RBP) levels and disease severity in patients with transthyretin cardiac amyloidosis (ATTR-CA), as well as its impact on therapeutic response to tafamidis.Methods:This retrospective study utilized data from the China National Rare Disease Registry System and included ATTR-CA patients treated with tafamidis between January 2018 and September 2022. Patients were stratified into two groups based on baseline RBP levels: the normal RBP group (≥36 mg/L) and the reduced RBP group (<36 mg/L). Baseline characteristics and clinical data after one year of treatment were collected and compared between the groups. Within the reduced RBP group, patients were further subclassified by changes in RBP levels after treatment (ΔRBP=post-treatment RBP-baseline RBP) into ΔRBP>0 and ΔRBP<0 subgroups. Worsening of global longitudinal strain (GLS) after treatment was defined as the primary outcome, logistic regression analysis was used to identify risk factors influencing therapeutic response to tafamidis in ATTR-CA patients.Results:A total of 52 ATTR-CA patients were included (aged (58.5±12.0) years, 46 males (88%)). Among 39 patients who completed one-year tafamidis treatment, no statistically significant difference was observed in RBP levels post-treatment versus baseline ((27.0±14.3) mg/L vs. (25.9±15.4) mg/L, P=0.261). Compared to the normal RBP group, the reduced RBP group had significantly higher estimated glomerular filtration rate-adjusted N-terminal pro-B-type natriuretic peptide levels (2 316.0 (1 161.5, 6 027.8) ng/L vs. 806.2 (349.5, 1 735.8) ng/L), higher left ventricular mass index ((164.4±46.5) g/m2 vs. (123.9±31.8) g/m2), and lower left ventricular ejection fraction ((50.8±11.3)% vs. (58.8±6.2)%) (all P<0.05). Among 31 patients in the reduced RBP group who completed one-year tafamidis treatment, 23 were classified as ΔRBP>0 and 8 as ΔRBP<0. The ΔRBP<0 group exhibited greater GLS worsening than the ΔRBP>0 group (0.7 (-0.1, 1.4)% vs. -0.4 (-1.4, 0.2)%, P=0.027). Multivariate logistic regression analysis revealed that ΔRBP<0 was an independent risk factor for GLS worsening ( OR=8.584, 95%CI 1.186-62.150, P=0.033) in ATTR-CA patients. Conclusion:ATTR-CA patients with reduced RBP levels exhibit more severe left ventricular structural and functional impairment compared to those with normal RBP levels. Decline in RBP during treatment (ΔRBP<0) is associated with poorer response to tafamidis treatment. Monitoring RBP dynamics may assist clinicians in assessing disease severity and therapeutic response in ATTR-CA patients.

Objective:To screen risk factors for ICU-acquired weakness in patients with mechanical ventilation and construct a predictive model, so as to provide a basis for the health management of patients with mechanical ventilation.Methods:Convenience sampling was used to select 312 ICU patients with mechanical ventilation admitted to the Tenth People's Hospital of Tongji University from October 2019 to August 2020 for the study. Patients were divided into training set ( n=220) and test set ( n=92) in a 7∶3 ratio. Based on machine learning algorithms, decision random forest (DRF), extremely-randomized trees (XRT) and generalized linear model (GLM) were used to construct three ICU-acquired weakness risk prediction models for patients with mechanical ventilation, respectively. The performance of the prediction model was evaluated using the area under the receiver operating characteristic curve ( AUC), the area under the precision-recall curve ( AUPRC), and the root mean square error ( RMSE) . Results:There were 7 predictors of risk of ICU-acquired weakness in patients with mechanical ventilation, including age, gender, braking, duration of mechanical ventilation, blood glucose, lactic acid, and parenteral nutrition. Test set and training set validation showed that AUC and AUPRC of GLM prediction model were greater than those of DRF, XRT prediction model. Test set validation indicated that the RMSE, logarithmic loss of GLM prediction model was less than those of DRF, XRT prediction model. Conclusions:Machine learning algorithm based GLM prediction model has good prediction performance. Healthcare professionals can construct evidence-based decisions for interventions in areas such as braking, duration of mechanical ventilation, and blood glucose management.

Objective To explore the research status,hotspots and trends in the field of traditional Chinese medicine in the treatment of dysfunctional uterine bleeding(DUB).Methods Relevant literatures on the treatment of DUB with traditional Chinese medicine were retrieved from China National Knowledge Infrastructure,Wanfang Data Knowledge Service Platform and VIP.Multi-dimensional visual analysis was conducted on the annual number of published papers,authors and institutions,and keywords using CiteSpace software.Results A total of 1649 literatures were included according to the inclusion and exclusion criteria.The top three authors in terms of the number of published articles are You Zhaoling,Ma Huirong,and Wu Yanan.Shandong University of Traditional Chinese Medicine is the institution that has published the most papers.The research on the treatment of DUB with traditional Chinese medicine mainly focuses on the integrated treatment of traditional Chinese and Western medicine,perimenopause,adolescence,therapeutic effect observation,Guchong decoction,sex hormones,and so on.Conclusion The research on the treatment of DUB with traditional Chinese medicine mainly focuses on the application of traditional Chinese medicine prescriptions,clinical observation and therapeutic effect indicators,etc.Subsequently,in-depth exploration of new traditional Chinese medical therapies and new observation indicators is still needed.In the future,the observation of new microscopic indicators and the research on pathological mechanisms may be potential research hotspots and directions.

Objective:To screen risk factors for ICU-acquired weakness in patients with mechanical ventilation and construct a predictive model, so as to provide a basis for the health management of patients with mechanical ventilation.Methods:Convenience sampling was used to select 312 ICU patients with mechanical ventilation admitted to the Tenth People's Hospital of Tongji University from October 2019 to August 2020 for the study. Patients were divided into training set ( n=220) and test set ( n=92) in a 7∶3 ratio. Based on machine learning algorithms, decision random forest (DRF), extremely-randomized trees (XRT) and generalized linear model (GLM) were used to construct three ICU-acquired weakness risk prediction models for patients with mechanical ventilation, respectively. The performance of the prediction model was evaluated using the area under the receiver operating characteristic curve ( AUC), the area under the precision-recall curve ( AUPRC), and the root mean square error ( RMSE) . Results:There were 7 predictors of risk of ICU-acquired weakness in patients with mechanical ventilation, including age, gender, braking, duration of mechanical ventilation, blood glucose, lactic acid, and parenteral nutrition. Test set and training set validation showed that AUC and AUPRC of GLM prediction model were greater than those of DRF, XRT prediction model. Test set validation indicated that the RMSE, logarithmic loss of GLM prediction model was less than those of DRF, XRT prediction model. Conclusions:Machine learning algorithm based GLM prediction model has good prediction performance. Healthcare professionals can construct evidence-based decisions for interventions in areas such as braking, duration of mechanical ventilation, and blood glucose management.

Objective:To investigate the impact of receptor-interacting protein kinase 3 (RIPK3) on major adverse cardiovascular events (MACE) in patients with acute myocardial infarction (AMI) after percutaneous coronary intervention (PCI), as well as the predictive performance of RIPK3 combined with traditional cardiovascular risk factors.Methods:This study was a single-center prospective cohort study. It included patients with AMI who underwent PCI at Peking Union Medical College Hospital between September 2017 and November 2017. Baseline clinical data were collected, and plasma samples were obtained 6 hours after PCI to measure RIPK3 levels. Follow-up was conducted via outpatient visits or phone calls to record the occurrence of MACE, including cardiovascular death, hospitalization for heart failure, and vascular events (recurrent AMI or stroke). The predictive performance of RIPK3, traditional cardiovascular risk factors and their combination for MACE was compared using receiver operating characteristic (ROC) curves. Patients were divided into low- and high-RIPK3 level groups based on the optimal cutoff value of RIPK3. Multivariate Cox proportional hazards regression analysis was used to assess the impact of RIPK3 levels on MACE after PCI in AMI patients. Kaplan-Meier survival curves were plotted, and the log-rank test was used to compare MACE incidence between the low-and high-RIPK3 groups.Results:A total of 103 AMI patients who underwent PCI were included, aged 63.0 (56.0, 69.0) years, and 83 (80.6%) were male. The follow-up time was 5.17 (2.81, 5.17) years, during which 44 patients (42.7%) experienced MACE. The ROC curve analysis showed that the area under the curve ( AUC) for traditional cardiovascular risk factors was 0.68 (95% CI: 0.58-0.78), while the AUC for plasma RIPK3 was 0.72 (95% CI: 0.62-0.82). The combined AUC for traditional risk factors and RIPK3 was 0.75 (95% CI: 0.65-0.85). Multivariate Cox proportional hazards regression analysis indicated that plasma RIPK3 level is greater than or equal to the optimal cutoff value of 440.9 μg/L ( HR=3.31, 95% CI: 1.53-8.30, P=0.005) was an independent risk factor for MACE in AMI patients after PCI. Kaplan-Meier survival analysis demonstrated that the high-RIPK3 group had a significantly higher risk of MACE after PCI compared to the low-RIPK3 group (log-rank P=0.006). Conclusions:Elevated plasma RIPK3 level is an independent risk factor for MACE in AMI patients after PCI. Plasma RIPK3 combined with traditional cardiovascular risk factors can more effectively predict the occurrence of MACE in AMI patients after PCI. AMI patients with RIPK3≥440.9 μg/L have a higher risk of MACE after PCI.

Objective:To explore the relationship between retinol-binding protein (RBP) levels and disease severity in patients with transthyretin cardiac amyloidosis (ATTR-CA), as well as its impact on therapeutic response to tafamidis.Methods:This retrospective study utilized data from the China National Rare Disease Registry System and included ATTR-CA patients treated with tafamidis between January 2018 and September 2022. Patients were stratified into two groups based on baseline RBP levels: the normal RBP group (≥36 mg/L) and the reduced RBP group (<36 mg/L). Baseline characteristics and clinical data after one year of treatment were collected and compared between the groups. Within the reduced RBP group, patients were further subclassified by changes in RBP levels after treatment (ΔRBP=post-treatment RBP-baseline RBP) into ΔRBP>0 and ΔRBP<0 subgroups. Worsening of global longitudinal strain (GLS) after treatment was defined as the primary outcome, logistic regression analysis was used to identify risk factors influencing therapeutic response to tafamidis in ATTR-CA patients.Results:A total of 52 ATTR-CA patients were included (aged (58.5±12.0) years, 46 males (88%)). Among 39 patients who completed one-year tafamidis treatment, no statistically significant difference was observed in RBP levels post-treatment versus baseline ((27.0±14.3) mg/L vs. (25.9±15.4) mg/L, P=0.261). Compared to the normal RBP group, the reduced RBP group had significantly higher estimated glomerular filtration rate-adjusted N-terminal pro-B-type natriuretic peptide levels (2 316.0 (1 161.5, 6 027.8) ng/L vs. 806.2 (349.5, 1 735.8) ng/L), higher left ventricular mass index ((164.4±46.5) g/m2 vs. (123.9±31.8) g/m2), and lower left ventricular ejection fraction ((50.8±11.3)% vs. (58.8±6.2)%) (all P<0.05). Among 31 patients in the reduced RBP group who completed one-year tafamidis treatment, 23 were classified as ΔRBP>0 and 8 as ΔRBP<0. The ΔRBP<0 group exhibited greater GLS worsening than the ΔRBP>0 group (0.7 (-0.1, 1.4)% vs. -0.4 (-1.4, 0.2)%, P=0.027). Multivariate logistic regression analysis revealed that ΔRBP<0 was an independent risk factor for GLS worsening ( OR=8.584, 95%CI 1.186-62.150, P=0.033) in ATTR-CA patients. Conclusion:ATTR-CA patients with reduced RBP levels exhibit more severe left ventricular structural and functional impairment compared to those with normal RBP levels. Decline in RBP during treatment (ΔRBP<0) is associated with poorer response to tafamidis treatment. Monitoring RBP dynamics may assist clinicians in assessing disease severity and therapeutic response in ATTR-CA patients.

Humans ; Consensus ; Immunoglobulin Light-chain Amyloidosis/therapy* ; Heart ; Immunoglobulin Light Chains ; China