OBJECTIVES: To study the clinical characteristics of pediatric Behçet syndrome (BS). METHODS: A retrospective review was conducted on the medical records of children hospitalized in the Department of Pediatrics at the Second Hospital of Hebei Medical University between December 2014 and December 2024 who met diagnostic criteria for BS. RESULTS: Among 45 children with BS, 26 (58%) were male. Oral aphthous ulcers were the most common manifestation (43/45, 96%), followed by genital ulcers (23/45, 51%) and gastrointestinal involvement (18/45, 40%). Genital ulcers were more frequent in girls, whereas ocular involvement was more common in boys (P<0.05). The pathergy test was positive in 10 (22%), and HLA-B51 was positive in 13 (29%). Fecal calprotectin (FC) was elevated in 16 (36%); gastrointestinal involvement was more frequent in children with elevated FC than in those with normal FC (P<0.05). According to the respective criteria, 17 (38%) patients met the International Study Group criteria (1990), 33 (73%) met the International Criteria for Behçet Disease (2014), and 13 (29%) met the Pediatric Behçet Disease criteria (2015). CONCLUSIONS Pediatric BS shows marked clinical heterogeneity. HLA-B51 is associated with disease susceptibility.
Humans ; Behcet Syndrome/genetics* ; Male ; Female ; Child ; Retrospective Studies ; Adolescent ; Child, Preschool ; Leukocyte L1 Antigen Complex/analysis* ; HLA-B51 Antigen

Kv1.3,a voltage-gated potassium channel,is highly expressed in T lymphocytes,the nervous system,and vascular smooth muscle cells.It plays a critical role in membrane excitability and electrical signal transduction,serving as an important target for studying T-cell function and providing a promising direction for developing therapeutics against autoimmune and inflammatory diseases.Therefore,the de-velopment of specific inhibitors of Kv1.3 channel has emerged as a novel therapeutic strategy for these disorders.In this study,we isolated and purified a novel Kv1.3-inhibitory peptide toxin,LmKTx13,from the venom of the scorpion Lychas mucronatus using reversed-phase high-performance liquid chroma-tography(RP-HPLC).LmKTx13 consists of 38 amino acid residues,including six cysteines that form three disulfide bonds.Whole-cell patch-clamp recordings revealed that LmKTx13 potently inhibited Kv1.3 with an IC50 of 7.92±3.0 nmol/L.Selectivity analysis showed that 2 μmol/L LmKTx13 also in-hibited Kv1.2 and Kv1.7,but exhibited no significant effects on other potassium channel subtypes or voltage-gated sodium channels.Further investigation into the mechanism demonstrated that LmKTx13 acts as a pore-blocking inhibitor of Kv1.3.By analyzing the effects of LmKTx13 on Kv1.3 channel gating ki-netics and performing sequence alignment of the pore regions of Kv1.3 and Kv1.5,we constructed site-directed mutants and identified the pore region of Kv1.3 as the critical binding site for LmKTx13.Key residues involved in the interaction included T425,G427,and H451.In summary,we discovered a no-vel pore-blocking Kv1.3 inhibitor,LmKTx13,from L.mucronatus venom,which exhibits high affinity and selectivity for Kv1.3.These findings highlight its potential as a potential lead molecule for developing Kv1.3-targeted therapeutics.

Aim To investigate the protective effects and potential mechanisms of Radix Angelica sinensis and Radix Hedysari ultrafiltrate(RAS-RH)on X-ray-induced cellular damage in Raw264.7 macrophages.Methods An integrated approach combining network pharmacology,molecular docking,and bioinformatics a-nalysis was employed to predict therapeutic targets and signaling pathways of RAS-RH in coronary heart dis-ease(CHD).Subsequent in vitro validation was per-formed using an X-ray(6 Gy)-induced macrophage in-jury model with four experimental groups:control,radi-ation-only model,and three RAS-RH-treated groups at varying concentrations.Cell viability was assessed by CCK-8 assay,apoptosis by flow cytometry with Annexin V-FITC/PI staining,mitochondrial membrane potential by JC-1 fluorescence,and inflammatory cytokine levels(IL-1 β,IL-6,IL-18,TNF-α)by ELISA.Molecular mechanisms were investigated through Western blot and qRT-PCR analyses of TLR4/NLRP3/Caspase-1 sig-naling pathway components and Bcl-2 family proteins.Results Network pharmacology revealed RAS-RH's multi-target action on apoptosis and inflammation-relat-ed pathways,particularly NF-κB and Bcl-2 signaling.Molecular docking identified strong binding affinities between RAS-RH components and TLR4/NLRP3 pro-teins.In vitro studies demonstrated that RAS-RH treat-ment significantly improved cell viability(P＜0.01),reduced apoptosis(P＜0.01),restored mitochondrial membrane potential(P＜0.05),and attenuated radia-tion-induced ultrastructural damage including mem-brane disruption and cytoplasmic vacuolization.ELISA showed marked suppression of pro-inflammatory cyto-kines(P＜0.01).Transmission electron microscopy(TEM)analysis revealed that RSA-RH ameliorated pyroptosis-associated ultrastructural alterations,inclu-ding plasma membrane disruption and cytoplasmic vac-uolization.Protein and gene expression analyses con-firmed downregulation of TLR4/NLRP3/Caspase-1 pathway and modulation of Bcl-2/Bax ratio.Conclu-sion RAS-RH exerts radioprotective effects through dual regulation of pyroptosis and apoptosis pathways,suggesting its potential as an adjuvant therapy for radia-tion-induced cardiovascular complications in CHD pa-tients.

Objective To study the regulatory effect of Qufeng Xiaodian Formula on serum differential metabolites of allergic purpura,and provide scientific basis for the diagnosis and treatment of allergic purpura in traditional Chinese medicine.Methods Sixty rats were randomly divided into a blank control group(referred to as the blank group),a model group,a compound glycyrrhizin group,a low-dose Qu Feng Xiao Dian Fang group,a medium dose Qu Feng Xiao Dian Fang group,and a high-dose Qu Feng Xiao Dian Fang group according to a random number table method,with 10 rats in each group.The model group was constructed by combining dried ginger,pepper,and long pepper with ovalbumin to create an allergic purpura rat model.After successful modeling,each treatment group was intervened with corresponding drugs for 4 weeks.After 4 weeks,serum was collected and non targeted metabolomics screening of serum differential metabolites was performed using ultra-high performance liquid chromatography quadrupole time-of-flight tandem mass spectrometer(UPLC-QTOF/MS).Subsequently,data extraction and multivariate statistical analysis will be conducted to identify potential metabolic pathways.Results Compared with the control group,there were 91 possible differential metabolites in the serum of the model group rats,corresponding to 20 metabolic pathways;Compared with the model group,there were a total of 43 possible differential metabolites in the serum of rats in the wind dispelling and disease eliminating group,corresponding to 15 metabolic pathways.Among them,there are a total of 12 metabolic pathways.Inflammatory metabolites such as arachidonic acid and ceramide can damage vascular endothelium.Ten biomarkers,including arachidonic acid and ceramide,were significantly abnormal in the serum of the model group rats compared to the normal group.The Qufeng Xiaodian formula can significantly reverse these metabolites and significantly enrich them in arachidonic acid metabolism and sphingolipid metabolism pathways.Conclusion Qufeng Xiaodian Formula has a certain regulatory effect on metabolites such as arachidonic acid and ceramide that affect vascular endothelial injury.

Kv1.3,a voltage-gated potassium channel,is highly expressed in T lymphocytes,the nervous system,and vascular smooth muscle cells.It plays a critical role in membrane excitability and electrical signal transduction,serving as an important target for studying T-cell function and providing a promising direction for developing therapeutics against autoimmune and inflammatory diseases.Therefore,the de-velopment of specific inhibitors of Kv1.3 channel has emerged as a novel therapeutic strategy for these disorders.In this study,we isolated and purified a novel Kv1.3-inhibitory peptide toxin,LmKTx13,from the venom of the scorpion Lychas mucronatus using reversed-phase high-performance liquid chroma-tography(RP-HPLC).LmKTx13 consists of 38 amino acid residues,including six cysteines that form three disulfide bonds.Whole-cell patch-clamp recordings revealed that LmKTx13 potently inhibited Kv1.3 with an IC50 of 7.92±3.0 nmol/L.Selectivity analysis showed that 2 μmol/L LmKTx13 also in-hibited Kv1.2 and Kv1.7,but exhibited no significant effects on other potassium channel subtypes or voltage-gated sodium channels.Further investigation into the mechanism demonstrated that LmKTx13 acts as a pore-blocking inhibitor of Kv1.3.By analyzing the effects of LmKTx13 on Kv1.3 channel gating ki-netics and performing sequence alignment of the pore regions of Kv1.3 and Kv1.5,we constructed site-directed mutants and identified the pore region of Kv1.3 as the critical binding site for LmKTx13.Key residues involved in the interaction included T425,G427,and H451.In summary,we discovered a no-vel pore-blocking Kv1.3 inhibitor,LmKTx13,from L.mucronatus venom,which exhibits high affinity and selectivity for Kv1.3.These findings highlight its potential as a potential lead molecule for developing Kv1.3-targeted therapeutics.

Objective To study the regulatory effect of Qufeng Xiaodian Formula on serum differential metabolites of allergic purpura,and provide scientific basis for the diagnosis and treatment of allergic purpura in traditional Chinese medicine.Methods Sixty rats were randomly divided into a blank control group(referred to as the blank group),a model group,a compound glycyrrhizin group,a low-dose Qu Feng Xiao Dian Fang group,a medium dose Qu Feng Xiao Dian Fang group,and a high-dose Qu Feng Xiao Dian Fang group according to a random number table method,with 10 rats in each group.The model group was constructed by combining dried ginger,pepper,and long pepper with ovalbumin to create an allergic purpura rat model.After successful modeling,each treatment group was intervened with corresponding drugs for 4 weeks.After 4 weeks,serum was collected and non targeted metabolomics screening of serum differential metabolites was performed using ultra-high performance liquid chromatography quadrupole time-of-flight tandem mass spectrometer(UPLC-QTOF/MS).Subsequently,data extraction and multivariate statistical analysis will be conducted to identify potential metabolic pathways.Results Compared with the control group,there were 91 possible differential metabolites in the serum of the model group rats,corresponding to 20 metabolic pathways;Compared with the model group,there were a total of 43 possible differential metabolites in the serum of rats in the wind dispelling and disease eliminating group,corresponding to 15 metabolic pathways.Among them,there are a total of 12 metabolic pathways.Inflammatory metabolites such as arachidonic acid and ceramide can damage vascular endothelium.Ten biomarkers,including arachidonic acid and ceramide,were significantly abnormal in the serum of the model group rats compared to the normal group.The Qufeng Xiaodian formula can significantly reverse these metabolites and significantly enrich them in arachidonic acid metabolism and sphingolipid metabolism pathways.Conclusion Qufeng Xiaodian Formula has a certain regulatory effect on metabolites such as arachidonic acid and ceramide that affect vascular endothelial injury.

Aim To investigate the protective effects and potential mechanisms of Radix Angelica sinensis and Radix Hedysari ultrafiltrate(RAS-RH)on X-ray-induced cellular damage in Raw264.7 macrophages.Methods An integrated approach combining network pharmacology,molecular docking,and bioinformatics a-nalysis was employed to predict therapeutic targets and signaling pathways of RAS-RH in coronary heart dis-ease(CHD).Subsequent in vitro validation was per-formed using an X-ray(6 Gy)-induced macrophage in-jury model with four experimental groups:control,radi-ation-only model,and three RAS-RH-treated groups at varying concentrations.Cell viability was assessed by CCK-8 assay,apoptosis by flow cytometry with Annexin V-FITC/PI staining,mitochondrial membrane potential by JC-1 fluorescence,and inflammatory cytokine levels(IL-1 β,IL-6,IL-18,TNF-α)by ELISA.Molecular mechanisms were investigated through Western blot and qRT-PCR analyses of TLR4/NLRP3/Caspase-1 sig-naling pathway components and Bcl-2 family proteins.Results Network pharmacology revealed RAS-RH's multi-target action on apoptosis and inflammation-relat-ed pathways,particularly NF-κB and Bcl-2 signaling.Molecular docking identified strong binding affinities between RAS-RH components and TLR4/NLRP3 pro-teins.In vitro studies demonstrated that RAS-RH treat-ment significantly improved cell viability(P＜0.01),reduced apoptosis(P＜0.01),restored mitochondrial membrane potential(P＜0.05),and attenuated radia-tion-induced ultrastructural damage including mem-brane disruption and cytoplasmic vacuolization.ELISA showed marked suppression of pro-inflammatory cyto-kines(P＜0.01).Transmission electron microscopy(TEM)analysis revealed that RSA-RH ameliorated pyroptosis-associated ultrastructural alterations,inclu-ding plasma membrane disruption and cytoplasmic vac-uolization.Protein and gene expression analyses con-firmed downregulation of TLR4/NLRP3/Caspase-1 pathway and modulation of Bcl-2/Bax ratio.Conclu-sion RAS-RH exerts radioprotective effects through dual regulation of pyroptosis and apoptosis pathways,suggesting its potential as an adjuvant therapy for radia-tion-induced cardiovascular complications in CHD pa-tients.

Objective To investigate the incidence rate,clinical characteristics,and prognosis of neonatal stroke in Shenzhen,China.Methods Led by Shenzhen Children's Hospital,the Shenzhen Neonatal Data Collaboration Network organized 21 institutions to collect 36 cases of neonatal stroke from January 2020 to December 2022.The incidence,clinical characteristics,treatment,and prognosis of neonatal stroke in Shenzhen were analyzed.Results The incidence rate of neonatal stroke in 21 hospitals from 2020 to 2022 was 1/15 137,1/6 060,and 1/7 704,respectively.Ischemic stroke accounted for 75％(27/36);boys accounted for 64％(23/36).Among the 36 neonates,31(86％)had disease onset within 3 days after birth,and 19(53％)had convulsion as the initial presentation.Cerebral MRI showed that 22 neonates(61％)had left cerebral infarction and 13(36％)had basal ganglia infarction.Magnetic resonance angiography was performed for 12 neonates,among whom 9(75％)had involvement of the middle cerebral artery.Electroencephalography was performed for 29 neonates,with sharp waves in 21 neonates(72％)and seizures in 10 neonates(34％).Symptomatic/supportive treatment varied across different hospitals.Neonatal Behavioral Neurological Assessment was performed for 12 neonates(33％,12/36),with a mean score of(32±4)points.The prognosis of 27 neonates was followed up to around 12 months of age,with 44％(12/27)of the neonates having a good prognosis.Conclusions Ischemic stroke is the main type of neonatal stroke,often with convulsions as the initial presentation,involvement of the middle cerebral artery,sharp waves on electroencephalography,and a relatively low neurodevelopment score.Symptomatic/supportive treatment is the main treatment method,and some neonates tend to have a poor prognosis.

Patients with severe trauma require an extremely timely treatment and transfusion plays an irreplaceable role in the emergency treatment of such patients. An increasing number of evidence-based medicinal evidences and clinical practices suggest that patients with severe traumatic bleeding benefit from early transfusion of low-titer group O whole blood or hemostatic resuscitation with red blood cells, plasma and platelet of a balanced ratio. However, the current domestic mode of blood supply cannot fully meet the requirements of timely and effective blood transfusion for emergency treatment of patients with severe trauma in clinical practice. In order to solve the key problems in blood supply and blood transfusion strategies for emergency treatment of severe trauma, Branch of Clinical Transfusion Medicine of Chinese Medical Association, Group for Trauma Emergency Care and Multiple Injuries of Trauma Branch of Chinese Medical Association, Young Scholar Group of Disaster Medicine Branch of Chinese Medical Association organized domestic experts of blood transfusion medicine and trauma treatment to jointly formulate Chinese expert consensus on blood support mode and blood transfusion strategies for emergency treatment of severe trauma patients ( version 2024). Based on the evidence-based medical evidence and Delphi method of expert consultation and voting, 10 recommendations were put forward from two aspects of blood support mode and transfusion strategies, aiming to provide a reference for transfusion resuscitation in the emergency treatment of severe trauma and further improve the success rate of treatment of patients with severe trauma.

Objective To understand the disease experiences of women with endometriosis(EMs),so as to provide a basis for improving the diagnosis,treatment,nursing and support of this population.Methods The databases of PubMed,Web of Science,Scopus,Embase,Cochrane Library,VIP,Wanfang Data,CNKI,CBM were retrieved on qualitative research about the disease experiences of endometriosis patients from inception to Jun 2023.The quality of the literature was evaluated by Joanna Briggs Institute(JBI)Critical Appraisal Tool for qualitative studies.Results A total of 17 studies were included,51 clear research findings were extracted,which were summarized into 10 new categories and 3 integrated results:(1)Cyclical episodes of the disease not only bring physical and psychological distress,but also lead to decreasing the patient's sense of female identity,destroying social and intimating relationships;(2)The doctor and patient interaction is influenced by imbalance of cognitive,the process of diagnosis and treatment is full of challenges,and patients have a demand for professional information and social support;(3)Growing up in pain,patients actively self-adjust and positively cope with the disease.Conclusion EMs affects patients'quality of life physiologically and psychologically,with prevalent issues of delayed diagnosis and repeated treatments.The professional information supported by health professionals needs to be improved.Healthcare providers should pay more attention to patients'physical and emotional experiences in their clinical work,improve their informal support,participate in long-term management,and improve patients'ability to manage their diseases.