OBJECTIVE To evaluate the quality of diagnosis and treatment guidelines and expert consensuses on childhood immune thrombocytopenic purpura （ITP） published domestically and internationally， in order to provide reference for clinical practice and future guideline/expert consensus development and improvement. METHODS A systematic search was conducted across multiple databases， including PubMed， Cochrane Library， Embase， CNKI， Wanfang data， VIP， CBM； additionally， supplementary searches were carried out on websites such as Medlive， the Chinese Medical Association’s official website， and National Institute for Health and Clinical Excellence in the UK. The retrieval time ranged from the inception to September 2， 2024. Researchers who had undergone systematic training independently evaluated the methodology and report quality included in the guideline/consensus using the Appraisal of Guidelines Research and Evaluation Ⅱ （AGREE Ⅱ） and the Reporting Items for Practice Guidelines in Healthcare （RIGHT）. RESULTS A total of 11 guidelines/consensuses were included. The average scores for the six domains of AGREE Ⅱ tool respectively were “range and purpose” （[ 66.67±17.98）% ]， “participants” [58.33% （13.89%，73.61%）]， “rigor” （[ 41.81±23.85）% ]， “clarity”（[ 69.57±19.35）%]， “applicability” （[ 35.98±17.83）%]， and “independence” [27.08% （0，75.00%）]； out of 11 articles， 9 had a recommendation level of B， 2 had a recommendation level of C， and there were no A-level articles. The average reporting rates of the 7 areas in the RIGHT tool were “basic information” （[ 72.35±12.95）% ]， “background” （[ 54.55±15.40）%]，“ evidence” （[ 36.36±24.81）%]，“ recommended opinions” （[ 53.25±19.20）%]，“ review and quality assurance” [0 （0， 25.00%）]， “funding and conflict of interest statement and management” [12.50%（0，25.00%）]， and other aspects [8.33%（0， 50.00%）]. In addition， there was no statistically significant difference in the AGREE Ⅱ and RIGHT scores between the guidelines and consensuses （P＞0.05）. CONCLUSIONS The overall quality of the guidelines and consensuses included in this study is not high， with a recommended level of B or C. It is recommended that clinical decision-making prioritize referring to the relatively high-quality guideline/consensus among them. The quality of evidence in the existing traditional Chinese medicine guidelines for children with ITP needs to be improved， and there is no integrated guideline/consensus for traditional Chinese and Western medicine. It is recommended to revise or write relevant guideline/consensus according to the requirements of AGREE Ⅱ and RIGHT in various fields to guide clinical practice.

AIM:To study the effect of hepatitis B combined with hepatic fibrosis on endogenous and exogenous metabolism of liver and the effect of glycyrrhizic acid combined with tenofovir(TFV)es-ter on anti-HBV efficacy.METHODS:Hepatitis B mouse was induced by chronic CCI4 to form a mod-el of hepatitis B with hepatic fibrosis.H&E staining,Sirius Red Staining,α-SMA immunohistochemistry were used to detect pathological changes in liver tissue.The changes of liver endogenous metabo-lism in mice with hepatitis B and hepatic fibrosis were detected by metabolomics.LC-MS/MS was used to investigate the plasma and liver concentra-tions of TFV and its active metabolite(TFV-DP),for investigating the changes of exogenous metabo-lism.RESULTS:HBV+CCl4 mice showed fibrosis symptoms such as liver injury and collagen deposi-tion.Hepatitis B combined with hepatic fibrosis af-fected nucleotide metabolism,amino acid metabo-lism,tricarboxylic acid cycle,pentose phosphate pathway and other endogenous metabolism,low-ered the hepatic level of TFV-DP,and decreased the antiviral efficacy.By combining with glycyrrhizic acid or forming a self-assembled preparation,the hepatic level of TFV-DP was improved,and the anti-viral efficacy was enhanced.CONCLUSIONS:Hepati-tis B combined with hepatic fibrosis affected both endogenous and exogenous metabolism of liver.Different forms of combination of glycyrrhizic acid and TFV could elevate the level of TFV-DP in liver and improve the antiviral efficacy in HBV+CCI4 mice.

Objective To investigate the correlation between the total load of cerebral small vessel disease(CSVD)and the early prognosis of patients with acute cerebral infarction(ACI).Methods A total of 150 patients with ACI admitted to the Department of Neurology,the First Hospital of Jiaxing from October 2020 to June 2022 were included,according to modified Rankin scale(mRs)score,patients were classified good prognosis group(mRs≤2 points)and poor prognosis group(mRs≥3 points),baseline data and clinical characteristics were compared between the 2 groups,and total CSVD load was assessed by cranial MRI,single factor and multi-factor analysis were performed with Logistic model.The relativity of CSVD load and early prognosis was analyzed with Pearson method.Results Age,length of stay,National Institute of Health stroke scale(NIHSS),hypersensitivity C-reactive protein,triglyceride and homocysteine were significantly higher in poor prognosis group than those in good prognosis group,the difference was significant(P<0.05).The scores of lacunar,cerebral microhemorrhage,white matter hyperintensity and CSVD in poor prognosis group were significantly higher than those in good prognosis group(P<0.05).Multivariate Logistic regression analysis showed that length of stay(OR=1.278,95％CI=1.054-1.548,P=0.012),admission NIHSS score(OR=1.354,95％CI=1.113-1.647,P=0.002),CSVD total load(OR=2.494,95％CI=1.666-3.735,P<0.001)were independent risk factors for poor early prognosis in patients with acute cerebral infarction.Conclusion CSVD load is associated with poor prognosis in patients with acute cerebral infarction.

Objective To investigate the clinical manifestations,molecular genetics and gonadal pathol-ogy characteristics of patients with disorders of sex development(DSD),and to summarize the clinical experi-ence of identifying rare diseases from common symptoms.Methods The clinical data of 416 patients with DSD diagnosed and treated in the multidisciplinary center of DSD of Guangzhou Women and Children's Medical Cen-ter from May 2018 to August 2023 were retrospectively analyzed,summarized and discussed.Results Accord-ing to chromosome karyotype,416 cases of DSD were classified into three types:92 cases(22.1％)of abnormal sex chromosome karyotype,285 cases(68.5％)of 46,XY karyotype and 39 cases(9.4％)of 46,XX karyotype.Among the 92 patients with abnormal sex chromosome karyotype,59 cases were raised as males,18 cases(30.5％)complained of short penis with hypospadias and cryptorchidism.The most common karyotype was 45,X/46,XY(58 cases,63.0％).Among the 285 patients with 46,XY karyotype,238 cases were raised as males,and 63 cases(26.5％)complained of short penis and hypospadias;47 cases were raised as females,and 13 ca-ses(27.7％)complained of inguinal mass.A total of 216 patients with 46,XY karyotype were subjected to whole exome gene detection,and 155 cases(71.8％)were found to have molecular pathogenesis with the clinical phe-notype.Among the 39 patients with 46,XX karyotype,19 cases were raised as males,and 8 cases(42.1％)com-plained of short penis and hypospadias.In the 18 cases of gonad biopsy,17 cases showed testicular tissue in go-nads.Whole exome sequencing was performed in 14 cases.NR5A1 gene heterozygous mutation,SRY gene muta-tion and SOX3 gene mutation were found in 2 cases,respectively(14.3％).Twenty cases were raised as females,and 14 cases(70.0％)complained of clitoral hypertrophy.Gonad biopsy was performed in 8 cases,with 7 cases of ovotestis(87.5％)and 1 case of NR5A1 gene heterozygous mutation(14.3％).Conclusions The etiologies of DSD are complex and diverse,and the clinical manifestations are various,which can be manifested as hypospa-dias,micropenis,cryptorchidism and other common symptoms of the urinary system.Different etiologies have dif-ferent treatment options.Therefore,chromosome karyotype,molecular genetic testing and gonadal pathology can be used to clarify the cause of disease,especially for rare diseases,improve the detection rate,reduce the rate of missed diagnosis,and ensure reasonable treatment,especially sex selection.

Neuropeptide Y receptor Y8(NPY8R)is a fish-specific receptor with two subtypes,NPY8AR and NPY8BR.Changes in expression levels during physiological processes or in vivo regulation after ventricular injection suggest that NPY8BR plays an important role in feeding regulation;this has been found in only a few fish,at present.In order to better understand the physiological function of npy8br,especially in digestion,we used clustered regularly interspaced short palindromic repeats(CRISPR)/CRISPR-associated protein 9(Cas9)technology to generate npy8br-/-Japanese medaka(Oryzias latipes).We found that the deletion of npy8br in medaka larvae affected their feeding and digestion ability,ultimately affecting their growth.Specifically,npy8br deficiency in medaka larvae resulted in decreased feed intake and decreased expression levels of orexigenic genes(npy and agrp).npy8br-/-medaka larvae fed for 10 d(10th day of feeding)still had incompletely digested brine shrimp(Artemia nauplii)in the digestive tract 8 h after feeding,the messenger RNA(mRNA)expression levels of digestion-related genes(amy,lpl,ctra,and ctrb)were significantly decreased,and the activity of amylase,trypsin,and lipase also significantly decreased.The deletion of npy8br in medaka larvae inhibited the growth and significantly decreased the expression of growth-related genes(gh and igf1).Hematoxylin and eosin(H&E)sections of intestinal tissue showed that npy8br-/-medaka larvae had damaged intestine,thinned intestinal wall,and shortened intestinal villi.So far,this is the first npy8br gene knockout model established in fish and the first demonstration that npy8br plays an important role in digestion.

Objective:To study the effect of blood volume feedback control system on improving intradialytic-hypotension (IDH) in maintenance hemodialysis (MHD) patients.Methods:It was a prospective cohort study. Thirty MHD patients with recurrent IDH in the Dialysis Center of the First Affiliated Hospital of Zhejiang University School of Medicine from March 2021 to March 2022 were selected. A self-control study was conducted in MHD patients. The patients were treated with routine hemodialysis in both baseline phase (A1) and reversal phase (A2), while with hemodialysis under the blood volume feedback control system in intervention phase (B). Each phase lasted for 4 weeks (12 hemodialysis sessions). The average occurrences of IDH and IDH-related adverse events (IDH-RAE, stopping dehydration for more than 10 minutes or getting off the hemodialysis machine 10 minutes earlier due to IDH) of each patient between phase A1, B, and A2 were calculated and compared. In a total of 1 080 dialysis records, a logistic regression analysis model was established with age, sex and intervention as independent variables and with the occurrence of IDH-RAE as the outcome.Results:A total of 30 eligible patients were included in the study, including 14 males (46.7%) and 16 females (53.3%), aged 63.0 (56.5, 72.5) years old, with a median dialysis age of 84.0 (37.2, 120.0) months. The average times of IDH in 30 MHD patients decreased from 1.17 (0.83, 1.67) in stage A1 (before intervention) to 0.33 (0.25, 0.58) in stage B (after intervention) ( P<0.05). The frequency of IDH-RAE decreased significantly from 0.29 (0.19, 0.47) in stage A1 to 0.17 (0,0.25) in stage B ( P<0.05). Logistic regression analysis results indicated that the use of blood volume feedback control system reduced the risk of IDH-RAE by 53% ( OR=0.47, 95% CI 0.34-0.64, P<0.001). Conclusions:The application of blood volume feedback control system can effectively reduce the occurrences of IDH and the risk of IDH-RAE in MHD patients.

BackgroundThe treatment of patients with depressive disorders is short of targeted outcome assessment. As a secondary outcome that is guided by patient values， quality of life is thus of relatively high evaluative value. In China， there exists a lack of large sample prospective cohort studies evaluating the effect of different treatment protocols on quality of life in patients with acute depressive disorder. ObjectiveTo explore the effects of monotherapy and combination therapy on the quality of life of patients with depressive disorder in acute phase， so as to provide references for optimizing the outcome of treatment for such patients. MethodsA prospective follow-up cohort study from August 24， 2020 to November 29， 2021 was conducted， including 1 330 patients from 22 hospitals across 18 cities in China. All these patients met the diagnostic criteria for depressive episodes， recurrent depressive disorder from the Diagnostic and Statistical Manual of Mental Disorders， fifth edition （DSM-5）. Patients were divided into monotherapy group （n=969） and combination therapy group （n=361） according to the acute phase treatment protocol. At baseline， the end of the first half month as well as the 1^st， 2^nd， 3^rd， 6^th， 9^th and 12^th months of treatment， patients were assessed with Inventory of Depressive Symptomatology Self-report （IDS-SR30）， Quality of Life Enjoyment and Satisfaction Questionnaire-Short Form （Q-LES-Q-SF）， Concise Health Risk Tracking Scale （CHRT） and Siehan Disability Scale （SDS）. Frequency， Intensity， and Burden of Side Effects Rating （FIBSER） was adopted for assessment at each visit time point of treatment. Spearman correlation analysis was adopted to examine the correlation of quality of life with suicide risk， adverse reactions and impaired social functioning among patients. ResultsAt the end of three months of treatment， the Q-LES-Q-SF score of monotherapy group was higher than that of combination therapy group， and the difference was statistically significant （Z=2.008， P<0.05）. The time effects of both treatment protocols possessed statistical significance （F=111.393， P<0.01）. At the end of three months of treatment， the Q-LES-Q-SF score was negatively correlated with CHRT and SDS scores， respectively， in both monotherapy group and combination treatment group （r=-0.660， -0.712， -0.634， -0.718， P<0.01）. ConclusionBoth monotherapy and combination therapy can facilitate the improvement of the life quality of patients with acute depressive disorder， but monotherapy may achieve better than the combination therapy in this aspect. ［Funded by The National Key Research and Development Program of China "Research on the Prevention and Control of Major Chronic Non-communicable Diseases" （number， 2017YFC1311101）］

Abstract：Objective To evaluate the basic development status of public health emergencies of infectious diseases in Hainan Province from 2013 to 2022, analyze the distribution and characteristics of these emergencies, as to provide important scientific basis for the study and formulation of economic health development planning policies, for the health security policies and infectious disease prevention and control strategies in Hainan Province. Methods The relevant statistical data of public health emergencies involving infectious diseases reported in Hainan Province from 2013 to 2022 were collected, and the status quo of these emergencies was analyzed by using descriptive data statistical analysis method. The ARIMA model was used to predict the number of public health emergencies reported by infectious diseases in Hainan Province from 2023 to 2025. Results From 2013 to 2022, a total of 482 public health emergencies were reported in Hainan Province, of which 426 were infectious disease public health emergencies, accounting for 88.38%, with a total of 8 876 cases, an incidence rate of 0.61%, and eight deaths, a case fatality rate of 0.09%. The major public health emergencies of infectious diseases were unclassified events and general events, accounting for 69.25% and 29.58%, respectively. The main diseases were novel coronavirus infection, chickenpox and hand, foot and mouth disease, accounting for 45.07%, 15.49% and 11.97%, respectively; in terms of time distribution, the number of reported incidents showed an upward trend over time, with a decline in 2021, and mainly had three peak periods, April, August and December, with a total of 220 cases reported; in terms of regional distribution, the major cities and counties reported were Haikou, Wanning and Wenchang, accounting for 27.46%, 19.25% and 9.86%, respectively; in terms of location distribution, the events mainly occurred in schools, accounting for 42.02% of the total number of incidents. The ARIMA(1, 1, 0) model was used to predict infectious disease public health emergencies, and the results showed that the forecast state would be stable from 2023 to 2025, with no obvious upward trend, that is, the fluctuation range of public health emergencies related to infectious diseases in Hainan Province would be stable in the next three years. Conclusion The number of infectious disease public health emergencies reported in Hainan Province from 2013 to 2022 has increased year by year, with a decline in 2021. Although the forecast shows that public health emergencies such as infectious diseases are relatively stable with no obvious upward trend, the prevention and control of the epidemic should not be taken lightly. Hainan Province should continue to increase the investment in monitoring public health emergencies, improve the information system of public disease prevention and control, and carry out real-time monitoring of public health emergencies of infectious diseases.

Obsessive-compulsive disorder (OCD) mainly manifests as obsessions and compulsions with the characteristics of early age of onset, long illness duration, and low diagnosis and treatment rate.The development of OCD involves mutations in genes associated with the 5-hydroxytryptamine, glutamate, and dopamine systems, as well as changes in the brain structure and function.However, how the potentially pathogenic genes affect brain structure and function and lead to different clinical manifestations remains unknown.Imaging genetics has been used to study the etiological mechanisms of OCD by combining genetics and imaging to analyze the relationship between genetic variation, neurologic and clinical manifestations.As a complex polygenic disease, imaging genetics that combines polygenic association analysis with epigenetic inheritance may become a new trend and perspective to explore the mechanism of the environment-gene-brain-behavior model in OCD.Therefore, this paper reviewed the related imaging genetics studies of OCD, and provided a theoretical basis for the etiological mechanism and precise intervention for OCD.

Humans ; COVID-19 ; Quarantine ; Renal Dialysis ; SARS-CoV-2 ; Hospitals