Objective:To analyze the efficacy of enzyme replacement therapy and anti-drug antibody production in children with Fabry disease.Methods:The clinical data of 7 children with Fabry disease treated with enzyme replacement therapy for more than 1 year at Children′s Hospital of Zhejiang University School of Medicine from July 2021 to June 2024 were retrospectively analyzed. The basic information and the changes of related clinical indicators before and after treatment were collected. Paired sample t test was used to compare renal function, left heart mass index, pain score and other related indexes before and after treatment. The anti-drug antibodies were detected by enzyme-linked immunosorbent assay. Results:A total of 6 boys and 1 girl were included. The age of diagnosis was (12.2±1.8) years. After 1 year of enzyme replacement therapy, the abnormal substrate globotriaosylsphingosine and brief pain inventory scores of all children were significantly lower than those before treatment ((16±11) vs. (63±42) μg/L, 22±19 vs. 45±29, t=3.88, 3.43, both P<0.05). There were no significant differences in glomerular filtration rate, urinary microalbumin to creatinine and left heart mass index before and after treatment ((124±35) vs. (136±26) ml/(min·1.73 m 2), (9.3±8.3) vs. (3.8±2.5) mg/g, (38±9) vs. (33±6) g/m 2.7, t=1.33, 1.74, 1.19, all P>0.05). Patients 4, 5 and 6 developed anti-drug antibodies at 1 month, 4 months and 1 month after medication, respectively. Patient 4 had persistently high anti-drug antibody titers (absorbance 3.65-3.73) accompanied by urticaria, elevated globotriaosylsphingosine and worsening clinical symptoms. Conclusions:The enzyme replacement therapy can effectively improve the clinical symptoms and reduce the level of globotriaosylsphingosine in children with Fabry disease. The anti-drug antibody is common in patients after long-term enzyme replacement therapy and may diminish the efficacy, which needs dynamic monitoring.

The ethical basis of public health policy is relatively weakness, which is mainly reflected in the conflict between individual rights and public interests, the lack of moral norm, the short of ethical review, the low level of relevant legislation, the weak of ethical consciousness and so on. Therefore, it is necessary to take measures to improve relevant ethical policies and regulations, establish an ethical framework for evaluating public health policies, highlight ethical values and strengthen ethical awareness in public health policy-making. Make it carry a certain ethical dimension to achieve the purposes of maintaining and improving health.

With persistent breakthrough and maturity of surgical procedures and postoperative immunosuppressive therapy, the survival rate of liver transplant recipients and grafts has been significantly increased. The shortage of donor liver has become the main obstacle for clinical development of liver transplantation. How to expand the source of donor liver has become an urgent issue. Groundbreaking progresses have been made in the use of common marginal donor livers in clinical liver transplantation, such as elderly donor liver, steatosis donor liver, viral hepatitis donor liver and liver from donation after cardiac death. Nevertheless, multiple restrictions still exist regarding the use of marginal donor liver. Consequently, the definition of marginal donor liver and research progress in the application of common marginal donor livers were reviewed, and the opportunities and challenges of mariginal donoor liver were illustrated, aiming to provide reference for expanding the donor pool for clinical liver transplantation and bringing benefits to more patients with end-stage liver disease.

Objective:To investigate the hemodynamic changes in the ascending aorta (AAo) before and after interventricular septal myocardial resection in obstructive hypertrophic cardiomyopathy (HOCM) using cardiac magnetic resonance four-dimensional blood flow (CMR 4D Flow) technology.Methods:HOCM patients who underwent interventricular septal myocardial resection at Guangdong Provincial People′s Hospital from May 2021 to September 2022 were prospectively included. Age and gender matched healthy volunteers (control group) were included during the same period. Both the control group and HOCM patients underwent CMR examination (including cine sequence and 4D Flow sequence) before and 6 months after surgery. CMR 4D flow technology was used to evaluate changes in AAo preoperative and postoperative blood flow patterns (eddy currents, spiral flow), maximum energy loss (EL max), and average energy loss (EL avg). HOCM patients underwent laboratory tests, including N-terminal pro-brain natriuretic peptide (N-pro BNP) and high-sensitivity troponin T (hsTnT). At the same time, the correlation between postoperative energy loss in HOCM patients and the degree of improvement in laboratory biomarkers was explored. Results:A total of 15 HOCM patients and 15 healthy volunteers were included. (1) In terms of blood flow patterns, the preoperative spiral flow degree of HOCM patients was significantly higher than that of the control group ( P=0.001), but the postoperative difference was not statistically significant ( P=0.059). The degree of eddy currents in HOCM patients before and after surgery was higher than that in the control group (all P<0.05). (2) In terms of energy loss, the preoperative EL max [21.17(14.30-28.10)mW vs 10.17(7.66-13.07)mW, P<0.001] and EL avg [4.87(3.46-5.77)mW vs 2.27(2.19-2.27)mW, P=0.023] of HOCM patients were higher than those of the control group, but there was no statistically significant difference between the postoperative and control groups (all P>0.05). Compared with preoperative, the postoperative EL max [12.33(8.70-17.41)mW] and EL avg [3.10(2.25-4.40)mW] of AAo in HOCM patients were significantly reduced (mean P=0.001). (3) Correlation analysis showed that there was a positive correlation ( r=0.587, P=0.021) between the EL max of AAo and the degree of improvement in hsTNT after interventricular septum myocardial resection, but no significant correlation ( r=0.229, P=0.413) with the degree of improvement in NT-pro BNP. Conclusions:The degree of postoperative AAo blood flow disorder in HOCM patients is reduced, and EL max and EL avg are significantly reduced. The EL max of postoperative AAo is positively correlated with the degree of improvement in hsTNT, suggesting that EL max may be applicable for prognostic evaluation of patients.

Cell-free DNA (cfDNA) is the DNA fragment existing in human extracellular fluid. In specific physiological process (such as pregnancy) or pathological conditions (such as human malignancies), the contents of cfDNA in extracellular fluid will abnormally change. The contents and molecular characteristics of cfDNA make it have the potential as a kind of biomarker for diseases′ diagnosis. With the development of cfDNA detection technology such as sequencing and mass spectrometry, liquid biopsy based on cfDNA detection has been widely used in clinical tumor diagnosis, tumor treatment, prenatal examination, and research in autoimmune diseases. A systematic summary of the latest research progress in the development of cfDNA detection technology and the clinical application of liquid biopsy, as well as the research progress of cfDNA in the diagnosis and treatment of related diseases is summarized in this review.

Objective:The Capital′s Funds for Health Improvement and Research (referred to as ″CFH″), established to address clinical medical issues, have been operating for over a decade. This study aims to comprehensively evaluate the achievements of this fund and provide empirical support and recommendations for optimizing the operational model of the clinical research fund.Methods:An online questionnaire was used to investigate the following aspects of CFH: subject area, the person in charge, derived projects, theses, patents, transformation of results, and popularization and promotion.Results:A total of 745 projects were collected through the online survey, and after rechecking for outliers, 720 completed projects were ultimately included. The top three disciplinary domains, in terms of the number of completed projects, were oncology, cardiovascular diseases, and neurological disorders. The age distribution of project leaders exhibited a left-skewed pattern, with a median age of 45 years. 319 (44%) received support from other related projects subsequently. 95% of the projects resulted in paper publication. 211 (29%) projects applied for patents and copyrights, with 141 projects being granted patent authorization. 78 (11%) projects successfully achieved technology transfer, with transfer and licensing being the most common modes. 156 (22%) projects disseminated their outputs, often targeting secondary hospitals and community health institutions.Conclusions:CFH aligns with local needs, and the outputs are substantial. In the future, consideration can be given to establishing an evaluation mechanism, increasing efforts to promote high-quality outputs, strengthening the organizational model set by the project guidelines, and further improving the rolling support mechanism.

Objective: To investigate epidemiological characteristics of notifiable infectious diseases in Zhejiang Province in 2023, so as to provide the evidence for strengthening prevention and control of infectious diseases. Methods: Data pertaining to notifiable infectious diseases reported in Zhejiang Province in 2023 were retrieved from the Infectious Disease Surveillance System of Chinese Disease Prevention and Control Information System. The incidence and mortality were analyzed according to the classification of notifiable infectious diseases and transmission routes, and epidemiological characteristics of infectious diseases were descriptively analyzed. Results: Thirty types of notifiable infectious diseases with 2 955 699 cases and 427 deaths were reported in Zhejiang Province in 2023, with a reported incidence rate of 4 493.93/105 and a reported mortality rate of 0.649 2/105. A total of 679 notifiable infectious disease emergencies were reported, with 26 514 cases and 1 case death (rabies). The emergencies mainly occurred in schools and preschool institutions, with 621 cases accounting for 91.46%. There were 1 case of cholera reported in class A notifiable infectious diseases and no death, 22 types of class B notifiable infectious diseases, with a reported incidence rate of 552.46/105 and a reported mortality rate of 0.644 7/105, and 8 types of class C notifiable infectious diseases, with a reported incidence rate of 3 941.48/105 and a reported mortality rate of 0.004 6/105. The incidence rates of respiratory, intestinal, blood-borne and sexually transmitted, natural and insect-borne infectious diseases were 4 028.67/105, 381.59/105, 81.15/105 and 1.35/105, respectively, according to transmission routes. Influenza (3 561.78/105) and COVID-19 (423.77/105) reported the highest incidence, and AIDS (0.477 4/105) and tuberculosis (0.130 8/105) reported the highest mortality. Conclusion The incidence rates of respiratory and intestinal infectious diseases were high in Zhejiang Province in 2023, and schools and preschool institutions were the main places of diseases occurred.

Objective: To investigate the characteristics of public health emergencies in Zhejiang Province in 2023, so as to provide the reference for public health risk management. Methods: Data of public health emergencies and related information in Zhejiang Province from January 1 to December 31, 2023 was collected through Emergency Public Reporting System of Chinese Disease Prevention and Control Information System. Attack rates, disease types, distribution of time and places, and responses were descriptively analyzed. Results: A total of 718 public health emergencies were reported in 2023 in Zhejiang Province, and all were infectious disease events. There were 27 128 reported cases and 3 deaths, with an attack rate of 3.22%. The top five infectious diseases with the highest number of reported events were influenza, norovirus infection, monkeypox, varicella and hand, foot and mouth disease, accounting for 95.54% of total reported events. There were 355 public health emergencies with less than 30 cases each, accounting for 49.44%. The reported emergencies peaked from February to March (186 events, 25.91%) and from November to December (327 events, 45.54%), and mainly occurred in schools and preschool institutions (651 events, 90.67%). The median responding time, reporting time and duration of emergencies were 6.50 (interquartile range, 10.84) h, 0.53 (interquartile range, 0.63) h and 7.24 (interquartile range, 11.71) d, respectively. Conclusion Public health emergencies in Zhejiang Province in 2023 were mainly caused by influenza and norovirus infection, with February, March, November and December being the peak reporting periods, and schools and preschool institutions being the main places where these events occurred.

Objective To evaluate the effectiveness and safety of Yiyang Pill in preventing and treating cardiovascular adverse reactions in patients with traditional Chinese medicine(TCM)syndrome with qi and yin deficiency and thyroid stimulating hormone(TSH)inhibition after differentiated thyroid cancer(DTC)resection.Methods A randomized,double-blind,placebo-controlled clinical trial was conducted,and 120 patients with TSH inhibition after DTC surgery were enrolled and randomized into two groups in a 1∶1 ratio using SAS 9.4 software generated random tables.The control group received a placebo and TSH suppression therapy,whereas the treatment group received the Yiyang Pill and TSH suppression therapy.The treatment period was 3 months.The incidence of cardiovascular adverse reactions,blood pressure,blood lipids,thyroid function,the dosage of levothyroxine,the efficacy of TCM syndrome,and safety indicators were compared between the two groups.Multivariate Logistic regression was used to analyze the influencing factors of cardiovascular adverse reactions.Results The incidence of cardiovascular adverse reactions in the treatment group was lower than that in the control group(P<0.05),and the efficacy of TCM syndrome treatment was significantly higher than in the control group(P<0.05).The free tetraiodothyronine level in the treatment group was higher than that before treatment(P<0.05),and the systolic and diastolic blood pressure in the control group increased compared to those before treatment(P<0.05).No severe adverse events were observed in either group.Compared with the control group,the cardiovascular incidence in the treatment group was lower,and the cardiovascular incidence in the<100 μg/d group was lower than that in the group with≥100 μg/d before treatment.Conclusion The Yiyang Pill can reduce the incidence of cardiovascular adverse reactions in patients after DTC surgery,effectively improve TCM syndromes,and be safe to use.Yiyang Pill treatment is a protective factor for cardiovascular adverse reactions,and the dosage of levothyroxine≥100 μg/d was a risk factor.

Objective To explore the expression of serum Hepcidin,long non-coding RNA myocardial infarction-associated transcript(LncRNA MIAT)and creatinine(CRE)in patients with bipolar disorder(BPD)and their relationship with the severity of the disease.Method In this study,150 patients with BPD who were admitted to Baoji Central Hospital from March 2021 to March 2023 were selected as the study subjects and were included in the BPD group.Another 150 healthy subjects were selected as the control group.Hamilton Depression Scale(HAMD)was used to evaluate depression.Young mania ratings scale(YMRS)was used to evaluate the degree of mania.All subjects were tested for serum Hepcidin,LncRNA MIAT and CRE levels.The levels of serum Hepcidin,LncRNA MIAT and CRE were compared between control group and BPD group.The levels of serum Hepcidin,LncRNA MIAT and CRE in BPD groups with different depression/mania degrees were compared.Receiver operating characteristic(ROC)curve was used to analyze the diagnostic value of serum Hepcidin,LncRNA MIAT and CRE in BPD.The relationship between serum Hepcidin,LncRNA MIAT and CRE and the severity of BPD was analyzed.Results The levels of serum Hepcidin(46.17±9.17 ng/L),LncRNA MIAT(11.92±2.65)and CRE(74.02±11.89 μ mol/L)in BPD group were higher than those in control group(22.16±5.23 ng/L,4.11±0.95,57.16±8.61 μ mol/L),with significant differences(t=-27.856,-43.079,-14.066,all P＜0.05).The levels of serum Hepcidin,LncRNA MIAT and CRE increased with the increase of depression/mania in BPD patients(F=22.036,25.463,17.499;19.552,15.791,18.335,all P＜0.05).The combined detection of serum Hepcidin,LncRNA MIAT and CRE(95％CI:0.866～0.949)for BPD diagnosis had an area under the ROC curve of 0.914,with high specificity and sensitivity,which was significantly higher than that of serum Hepcidin alone(95％CI:0.693～0.816),LncRNAMIAT(95％CI:0.696～0.819)alone and CRE(95％CI:0.587～0.701)detection alone(AUC=0.759,0.762,0.614,all P＜0.05).Serum Hepcidin,LncRNA MIAT and CRE were positively correlated with depression severity in BPD patients(r=0.784,0.771,0.826,all P＜0.05).Serum Hepcidin,LncRNA MIAT and CRE were positively correlated with mania severity in BPD patients(r=0.806,0.793,0.831,all P＜0.05).Conclusion The levels of serum Hepcidin,LncRNA MIAT and CRE in BPD patients were higher than those in the normal population,and they were positively correlated with the severity of the disease.Combined detection may have a high diagnostic value.