Objective:To analyze and summarize the efficacy and safety of thalidomide in the treatment of refractory systemic juvenile idiopathic arthritis（sJIA）.Methods:The clinical data of ten patients with refractory sJIA admitted to Department of Nephrology and Immunology in Children's Hospital of Hebei Province from January 2015 to March 2022 were collected，and the clinical manifestations，efficacy and safety of thalidomide in the treatment of refractory sJIA were analyzed retrospectively. Systemic juvenile arthritis disease activity score（sJADAS）was used to evaluate the efficacy of the treatment. Statistical analysis was performed by repeated measurements using general linear models.Results:Among the 10 children（4 males and 6 females）with refractory sJIA，the average age of onset was（7.5±3.3）years. Seven patients were complicated with macrophage activation syndrome at an early stage of disease.The average course of disease was（4.4±1.7）years，and the longest course of disease was 8.3 years. Before the application of thalidomide，all the 10 children experienced relapses（ranging from 2 to 10 times）. The indices of 10 children treated with thalidomide at 6 months and 12 months were compared with those before treatment. Peripheral blood leukocytes［（10.19±3.67）×10 9/L，（8.53±2.83）×10 9/L vs.（16.11±7.81）×10 9/L， F=7.918，11.084， P=0.020，0.009］，C-reactive protein［19.13（0.38，35.21）mg/L，8.05（0.10，18.00）mg/L vs. 59.34（24.20，131.90）mg/L， F=7.030，12.731， P=0.026，0.006］，sJADAS scores［6.00（1.50，12.50）scores，3.00（0，12.50）scores vs. 20.00（11.50，28.00）scores， F=14.710，17.870， P=0.004，0.002］were decreased significantly. The doses of prednisone［0.13（0，0.45）mg/（kg·d），0.02（0，0.06）mg/（kg·d）vs. 0.42（0.16，1.47）mg/（kg·d）， F=5.890，7.623， P=0.041，0.022］were significantly decreased.All the differences were statistically significant. Prednisone was successfully discontinued in 7 cases. Tocilizumab was gradually withdrawn in 3 cases，and tocilizumab administration interval was prolonged in 1 case. None of the 10 children had serious adverse reactions. Conclusion:Thalidomide is clinically effective in the treatment of sJIA，and can reduce the required dose of prednisone and prolong the tocilizumab free remission.

Acquired cerebral amyloid angiopathy,as a cerebrovascular disease discovered in recent years,mainly spreads through iatrogenic factors.This paper focuses on acquired cerebral amyloid angiopathy and delves into the new applications and profound implications of the"toxins damaging brain collaterals"theory proposed by academician WANG Yongyan in stroke research.Starting from the perspective of"toxins damaging brain collaterals",it comprehensively analyzes the causes and pathological mechanisms of acquired cerebral amyloid angiopathy,emphasizing the importance of external toxins,latent toxins,and turbid toxins in understanding the pathogenesis of the disease.It reveals that"toxins damaging brain collaterals"is the core mechanism of acquired cerebral amyloid angiopathy.On this basis,it proposes a new connotation of"toxins damaging brain collaterals":first,"toxin"includes both internal and external toxins;second,the organic integration of"external wind theory"and"toxins damaging brain collaterals"guides the improvement of theoretical research,clinical treatment,and prevention strategies for stroke,and promotes a deeper understanding of the disease.This approach to understanding acquired cerebral amyloid angiopathy from the perspective of"toxins damaging brain collaterals"not only demonstrates the effective integration of traditional Chinese medicine theory and modern biomedical science,but also provides insights and references for the clinical diagnosis and treatment of the disease.

Objective:To understand the cognition of the family caregivers of terminal cancer patients on digital health intervention, to clarify their actual needs, and to analyze the obstacles to their acceptance of digital health intervention, so as to develop a digital health intervention plan for the family caregivers of terminal cancer patients.Methods:From February 2024 to March 2024, the family caregivers of 16 patients with terminal cancer in Hunan Cancer Hospital Pain Hospice Ward were selected by objective sampling, who met the inclusion and exclusion criteria were interviewed in a semi-structured manner about the cognition and needs of digital health intervention, and the interview contents were sorted and analyzed using the Colaizzi7 step method.Results:A total of 16 family caregivers of terminal cancer patients, 4 males and 12 females, aged 26-55 years, were interviewed. Four themes were distilled from the interview results: family caregivers of terminally cancer patients agree on the importance of digital health interventions; lack of awareness of digital health interventions; expectations of digital health interventions; and possible confounders affecting digital health interventions.Conclusions:The family caregivers of terminal cancer patients had insufficient awareness of digital health intervention, but all showed affirmation of the development of digital health intervention services. It is recommended to actively improve the basic conditions of digital health services, strengthen publicity, raise the level of awareness of the family caregivers, and positively overcome the relevant interfering factors, so as to gradually promote the development of digital health services.

Objective To construct a telemedicine-based home care system of palliative care for end-of-life cancer patients,and to evaluate its application effect.Methods The construction of the system was based on the literature research,interview results of the patients and their caregivers,and expert group meeting discussions.From May to August 2023,a total of 88 patients,who were about to be discharged from the palliative care ward of a cancer hospital in Hunan Province,were selected as the study subjects using a convenient sampling method,and then they were divided into an experimental group and a control group according to the order of admission.The experimental group was given home care services based on the system,while the control group was given routine discharge guidance and follow-up visits.The duration of the intervention was 8 weeks.The Chinese version of the Edmonton Symptom Assessment Scale and the Chinese version of the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 15 palliative were used to evaluate the symptom burden and quality of life between 2 groups before and after intervention;the Telemedicine Satisfaction Questionnaire was used to evaluate the care satisfaction of the experimental group.Results The system of patient side includes 8 main features,namely personal health record establishment,doctor-patient communication,palliative care knowledge library,medication assistance,smart reporting and monitoring,intelligent Q&A,intelligent follow-up,and intelligent forms.The healthcare professional side includes 7 main functions,namely workbench,case management,follow-up management,warning reminder,popularization push,statistical analysis and user management.A total of 67 patients completed the intervention,with 33 in the experimental group and 34 in the control group.The symptom burden of the experimental group was lower than that of the control group(P＜0.05);the quality of life in the experimental group was better than that in the control group(P＜0.05);the score of care satisfaction was(59.00±6.66),which indicated a high level of satisfaction.Conclusion The system can reduce the symptom burden and improve the quality of life of end-of-life cancer patients with good practicality

Objective To evaluate the effect of Internet-based psychosocial intervention for primary caregivers of patients with terminal stage cancer.Methods The relevant randomized controlled trials of Internet-based psychosocial intervention for primary caregivers of patients with terminal stage cancer were systematically searched from 10 databases,such as Chinese Biomedical Literature Database,CNKI,PubMed,Embase and CINAHL and etc.The search period was from the establishment to December 18,2023,and 2 investigators screened the literature according to inclusion and exclusion criteria and extracted data.Cochrane Manual 5.1.0 was used to assess the quality of the literature.Meta-analysis was performed using Revman 5.4 software.Results A total of 9 pieces of the literature including 592 primary caregivers were included.Meta-analysis showed that Internet-based psychosocial intervention reduced the level of anxiety and depression for primary caregivers of patients with terminal stage cancer[MD=-1.64,95％CI(-2.68,-0.59),P=0.002]and the burden of care[SMD=-0.39,95％CI(-0.60,-0.18),P＜0.001],and improved quality of life[SMD=0.25,95％CI(0.01,0.48),P=0.040];further research is needed to explore the effect of reducing the level of distress[MD=-0.88,95％CI(-1.91,0.16),P=0.100].Conclusion The Internet-based psychosocial intervention is effective in improving the anxiety,depression and the burden of care of the primary caregivers of patients with terminal stage cancer,and it can improve the quality of life;further research is needed to explore the level of distress reduction.

Objective:To compare the similarities and differences of macrophage activation syndrome（MAS）combined with systemic juvenile idiopathic arthritis（sJIA）versus with juvenile onset systemic lupus erythematosus（JSLE）.Methods:The clinical data of 48 children with MAS admitted to the Department of Nephrology and Immunology in Children's Hospital of Hebei Province from May 2015 to January 2023 were retrospectively analyzed. The patients were divided into sJIA-MAS and JSLE-MAS group，and the clinical manifestations，laboratory indicators and treatment of the two groups were compared.Results:Among the 48 children（14 males and 34 females）with MAS，the average age of onset was 9.5（3.0，11.8）years. There were 28 cases（11males and 17 females）of sJIA-MAS and 20 cases（3 males and 17 females）of JSLE- MAS. All the 48 children with MAS had fever and hyperferinemia，and the fever with sJIA-MAS was mostly continued fever or remittent fever. Respiratory tract infection was the most common trigger in sJIA-MAS［15 cases（53.6%）］，and disease activity was the most common trigger in JSLE-MAS［13 cases（65.0%）］.Additionally，viral infections（EB virus and cytomegalovirus）were also one of the triggers in MAS［sJIA：7 cases（25%），JSLE：4 cases（20%）］.Compared with JSLE-MAS，the number of days with fever［15.0（12.0，21.0）days vs. 6.0（4.0，9.5）days， Z=-3.812， P=0.001］and the length of hospital stay［29.0（26.3，39.8）days vs.26.0（19.3，30.8）days， Z=-1.958， P=0.049］were longer in sJIA. Compared with JSLE-MAS，ALT［（685.32±561.67）U/L vs.（139.61±124.44）U/L， t=4.973， P=0.001］，AST［784.00（235.25，1 251.25）U/L vs.189.50（53.25，374.08）U/L， Z=-3.283， P=0.001］，CRP［11.48（3.56，28.89）mg/L vs.1.91（0.53，8.98）mg/L， Z=-3.200， P=0.001］，ferritin［32 167.0（12 384.8，65 963.8）μg/L vs.2 003.5（922.5，11 430.0）μg/L， Z=-4.130， P=0.001］，ferritin max/ESR min［1 353.35（355.75，4 342.53）vs.91.92（34.94，291.53）， Z=-4.120， P=0.001］were higher in sJIA.The decrease of CRP was greater in sJIA［80.04（45.64，143.71）mg/L vs.10.20（6.27，25.64）mg/L， Z=-4.433， P=0.001］.Compared with sJIA-MAS，peripheral white blood cell counting［4.05（2.90，7.73）×10 9/L vs.1.56（1.15，3.47）×10 9/L， Z=-3.577， P=0.001］and platelet counting［（162.68±92.19）×10 9/L vs.（110.10±72.99）×10 9/L， t=2.118， P=0.040］were lower in JSLE-MAS. Kidney involvement was more common in JSLE-MAS［10 cases（50%）vs.0 cases（0%）， χ 2=17.684， P=0.001］.There was no significant difference in the incidence of sJIA-MAS and JSLE-MAS meeting the criteria of hemophagocytic lymphohistiocytosis［6 cases（21.4%）vs.5 cases（25.0%）， χ 2=0.084， P=0.772］. Conclusion:Compared with JSLE-MAS，sJIA-MAS is more dangerous and difficult to control，while JSLE-MAS involves more organs，among which the blood system and kidney are more common.

Objective This paper aims to investigate and analyze the baseline conformity rate of bacterial culture exami-nation records in a tertiary specialized hospital.By doing this,this paper seeks to understand the current situation of medical re-cord writing of bacterial culture examination and explore the improvement path and relevant measures for enhancing the conformity rate of bacterial culture examination records.Methods According to the requirements of"Quality Control Index of Medical Re-cord Management(2021 Edition)",a retrospective analysis was conducted on 6 317 medical records that underwent bacterial culture examination in 2022.Results The execution rate of medical orders was 100.00％,the completeness rate of the report sheet was 100.00％,the record rate of medical records was 81.05％,the analysis rate of results was 33.07％,and the conformi-ty rate of bacterial culture examination records was 33.07％.Conclusion It is imperative to enhance the conformity rate of bac-terial culture examination records and improve the quality of medical records in terms of content.

Objective:To analyze the differences in neuronal activation during fear memory extinction in various sub-regions of the hippocampus in post-traumatic stress disorder(PTSD)mice.Methods:Two immediate early gene-pro-tein labeling strategies were employed to label neurons associated with fear extinction in PTSD mice.In the first group,Arc protein in hippocampal neurons was labeled and observed through immunofluorescence staining in wild-type mice.In the second group,Fos-CreERT2;Ai9 transgenic mice were injected with tamoxifen 23 hours prior to inducing fear memory extinction,and the relevant neurons were labeled with fluorescent proteins for observation.The number of labeled hippocampal neurons and the dendritic branch structure were analyzed to compare the activation levels of hipp-ocampal neurons and the plasticity of neuronal dendrites.Results:The two groups of Arc and Fos positive neurons were mainly distributed in the dorsal hippocampus,in which Arc protein chromogenic was enriched in CA3 and DG subre-gions,while CA1 and CA2 subregions were scattered,while Fos-positive neurons were enriched in the DG subregion of hippocampus and scattered in CA1,CA2 and CA3 subregions.Compared to the control group,there was no significant difference in the number of neurons expressing Arc protein in each subregion of the hippocampus in the PTSD group.The number of Fos-positive neurons in CA1,CA3 and DG subregions in the hippocampus of the PTSD group was signifi-cantly increased(P＜0.01).The dendritic branches of neurons in the hippocampal region were observed and analyzed in Fos-CreERT2;Ai9 mice from both groups,but no significant changes were found.Conclusion:Abnormal activation of neurons occurs in different subregions of the hippocampus during fear extinction in PTSD mice,although there are no significant plasticity changes in the dendritic branches of the activated neurons.

Objective To analyze the current status of cognition and satisfaction of medical staff in public hospitals in Hangzhou towards medical prevention integration,in order to provide reference and reference for promoting the development of medical prevention integration.Methods A questionnaire survey was conducted on 421 medical staff from three public hospitals in Hangzhou using stratified random sampling.Results Only 29.5%of medical staff expressed a strong or relatively good under-standing of medical prevention integration work;The overall satisfaction score of medical staff with medical prevention integration is(3.54±0.74).The results of logistic regression analysis show that age,professional title,education level,and monthly in-come are important factors affecting the satisfaction of medical staff with medical prevention integration(P＜0.05).Conclusion The awareness and satisfaction of medical staff in public hospitals in Hangzhou regarding the integration of medical and preven-tive measures need to be strengthened.It is recommended to improve basic construction,optimize health policies,promote infor-mation integration,and strengthen work cohesion.

The data of a child with sphingosine phosphate lyase insufficiency syndrome (SPLIS) admitted to Children′s Hospital of Hebei Province on February 4, 2020 were retrospectively analyzed.The child had edema, complicated with ichthyosis, adrenal calcification, and hearing loss from the early infancy.Laboratory examination results suggested a low albumin level, hypercholesterolemia, a high proteinuria level, abnormal liver and renal functions, and hyponatremia.The child gave up treatment and died at home.Whole Exome Sequencing (WES) results showed two hete-rozygous mutations of SGPL1 gene (chr10: 72604336, c.134G>A, p.W45X; chr10: 72629563, c.719G>T, p.S240I). SPLIS is inherited in an autosomal recessive manner.It starts in infancy, and affects the kidney, skin, endocrine, nervous and immune systems.It is suggested that SPLIS patients should take genetic examination.Early diagnosis, appropriate intervention, and vitamin B 6 treatment may relieve some symptoms of SPLIS patients.Adeno-associated virus mediated SGPL1 gene replacement therapy can be a novel cure of SPLIS and is worthy of investigation.