Neurofibromatosis type 1 (NF1) presents with a diverse range of symptoms that can affect the skin, bones, eyes, central nervous system, and other organs. This article reports the diagnosis and treatment process of a patient with NF1 complicated by bilateral severe-to-profound sensorineural hearing loss. Genetic testing revealed a heterozygous variant of NF1 NM_ 000267.3: c.4054_ 4058del(p.Ser1352LeufsTer20, supporting the diagnosis of NF1. After thorough evaluation, a right cochlear implantation was performed, yielding satisfactory postoperative results. This case suggests that NF1 patients may exhibit phenotypic heterogeneity and atypicality, providing a reference for clinicians in the diagnosis and treatment of such patients.

Objective To explore the changes in serum energy metabolites in patients with peripheral T-cell lymphoma,and investigate serum biomarkers for monitoring peripheral T-cell lymphoma from the perspective of energy metabolism.Methods Multiple/selected reaction monitoring(MRM/SRM)was used to detect the energy-related metabolites in the sera of 16 patients with newly diagnosed peripheral T-cell lymphoma admitted in the Hematology Medical Center of the Second Affiliated Hospital of Army Medical University from November 2020 to December 2021,as well as 10 recruited healthy volunteers.The corresponding clinical data including medical history,laboratory results and image data were collected and retrospectively analyzed.Results Significant differences were seen in the contents and expression profiles of serum energy metabolism-related products between the patients and the healthy volunteers.The patients had significantly reduced serum contents of cyclic AMP,succinate,citrate and cis-aconitate(P＜0.05),and elevated D-glucose 6-phosphate content(P＜0.05).The serum contents of citrate and succinate were negatively correlated with the risk stratification(low-,moderate-and high-risk)and clinical stage of the disease(P＜0.05).Meanwhile,there was a negative correlation between the contents of L-malic acid and citrate and the mid-term efficacy evaluation results,such as complete/partial response(CR/PR)or stable disease(SD)(P＜0.05).For patients with extranodal NK/T cell lymphoma(n=10),there were also significant reductions in the contents of cyclic AMP,succinate,citrate,isocitrate and cis-aconitate in the sera of patients compared with healthy volunteers(P＜0.05),and the contents of citrate and succinate were negatively correlated with the clinical stage(P＜0.05)and were rather correlated with mid-term efficacy evaluation results(CR/PR or SD)(P＜0.05).For patients with angioimmunoblastic T-cell lymphoma(n=6),the serum contents of cyclic AMP,citrate and succinate were significantly lower,while the content of D-glucose 6-phosphate was higher when compared with the healthy volunteers(P＜0.05),and the content of succinate was negatively correlated with both clinical stage and risk grade of the patients(P＜0.05).Conclusion There are 5 serum differential metabolites identified between patients with peripheral T-cell lymphoma and healthy controls,and succinate and citrate are expected to be serum biomarkers of peripheral T-cell lymphoma.

Guided by the theory of "kidney generates marrow"， the study elaborates the viewpoint that the route of Yin Heel Channel （阴跷脉） is consistent with the "kidney-marrow-brain" axis from the perspective of the circulation of the meridians and the relationship between the zang-fu organs. Accordingly， it is believed that disease of Yin Heel Channel and dysfunction of the "kidney-marrow-brain" axis are the core pathogenesis of children enuresis， and it is elaborated from the following three major aspects， firstly， insufficient kidney essence， dysfunction of the "kidney-marrow-brain" axis， secondly， disease of Yin Heel Channel and deficiency and cold in lower jiao， and thirdly， disease of Yin Heel Channel and loss of nourishment of Chong Vessel. It is proposed to use the mode of "firstly needle， secondly moxibustion， and lastly consolidation" to treat children enuresis. Needle is to adjust yin and yang， warm yang and tonify kidney， and wake up the brain and open the orifices. The acupoints in Yin Heel Channel such as Zhaohai （KI 6）， Jiaoxin （KI 8） and confluence points of the eight extraordinary vessels such as Waiguan （TE 5）， Zulinqi （GB 41） are used， together with Baihui （GV 20）， Yintang （EX-HN 3）， Guanyuan （CV 4）， Qixue （KI 13）， Dazhong （KI 4）. Moxibustion is to reinforce healthy qi and warm yang， bank up the root and consolidate the original qi by moxibustion at Shenque （CV 8）， Mingmen （GV 4）， and Xuanshu （GV 5）. Consolidation is to use acupoints application to consolidate the therapeutic effect， and Guanyuan （CV 4） & Pangguangshu （BL 28）， Qihai （CV 6） & Zhishi （BL 52）， and Shenque （CV 8） & Ciliao （BL 32） are commonly used as the three groups of acupoints to warm the kidney and stop collapse， regulate and tonify the qi and blood.

Objective To investigate the effect of dexmedetomidine(Dex)on the rat model of perioperative stroke and its mechanism.Methods One hundred male rats were randomly divided into sham group,middle cerebral artery occlusion(MCAO)group,low dose Dex[Dex-L,0.5 μg/(kg·h)]group,medium dose Dex[Dex-M,2 μg/(kg·h)]group,high dose Dex[Dex-H,10 μg/(kg·h)]group,20 rats in each group.A rat model of perioperative stroke was established by middle cerebral artery occlusion.Dex was injected intravenously at different doses during ischemia.After 24 h,the neurological function of the rats was evaluated.Then,the rats were sacrificed and the peripheral blood and whole brain tissue were collected,ischemic core area tissue was separated from some brain tissues and the cerebral infarction area was observed by TTC staining.The inflammatory cytokine contents in serum and ischemic core area were measured by ELISA.In addition,the expressions of formyl peptide receptor 1(FPR1),transmembrane protein 119(TMEM119),CD31 and VE-cadherin proteins were assayed by immunofluorescence,and the expressions of FPR1,nuclear factor-κB(NF-κB)and NLRP3 proteins by Western blotting in the ischemic core area.Results Compared with sham group,the proportion of cerebral infarction area and neurological scores in the MCAO group were significantly increased,and the contents of interleukin(IL)-1β,IL-6 and tumor necrosis factor(TNF)-α in the serum and the ischemic core area were significantly increased,the expressions of FPR1,TMEM119,p-NF-κB,NLRP3,CD31 and VE-cadherin in the ischemic core were significantly increased(P<0.001),and there was obvious co-expression of FPR1 and TMEM119.Compared with MCAO group,the proportion of cerebral infarction area and neurological scores in the Dex-M and Dex-H groups were significantly decreased,and the contents of IL-1β,IL-6 and TNF-α in serum and brain ischemic core area were significantly decreased,the expressions of FPR1,TMEM119,p-NF-κB,NLRP3,CD31 and VE-cadherin in the ischemic core were significantly decreased(P<0.001).Conclusions Dex can significantly alleviate perioperative stroke injury.The mechanism may be due to inhibiting the expression of FPR1 protein,activation of microglia cells and cerebral collateral circulation angiogenesis.

Objective To discuss the clinical application of coronary CT angiography(CCTA)-derived fractional flow reserve(CT-FFR)in evaluating the risk stratification of the coronary artery stenosis and atherosclerotic plaque quantitative parameters.Methods A total of 122 patients,who received CCTA examination at the Xuzhou Municipal Central Hospital of China,were enrolled in this study.The patients were divided into non-ischemia group(CT-FFR＞0.8,n=66)and ischemia group(CT-FFR0.8,n=56).The characteristics of atherosclerotic plaque were compared between the two groups.Logistic regression analysis was used to analyze the correlation between plaque characteristics and ischemic lesions.Results There were 218 vessels having a CT-FFR＞0.8 and 174 vessels having a CT-FFR ≤0.8.Statistically significant differences in the total plaque volume,calcified plaque volume,plaque length,and stenosis ratio＞50％existed between the two groups(all P＜0.05).Logistic regression analysis indicated that the total plaque volume,calcified plaque volume,plaque length,and stenosis ratio＞50％were the risk factors for myocardial ischemia.Conclusion CT-FFR can be used for the risk stratification of coronary stenosis and atherosclerotic plaque characteristics,which can evaluate the local myocardial blood supply condition from the anatomical stenosis and functional level so as to optimize the diagnosis and treatment measures.

Objective This study aimed to investigate the potential relationship between urinary metals copper (Cu), arsenic (As), strontium (Sr), barium (Ba), iron (Fe), lead (Pb) and manganese (Mn) and grip strength. Methods We used linear regression models, quantile g-computation and Bayesian kernel machine regression (BKMR) to assess the relationship between metals and grip strength.Results In the multimetal linear regression, Cu (β=-2.119), As (β=-1.318), Sr (β=-2.480), Ba (β=0.781), Fe (β= 1.130) and Mn (β=-0.404) were significantly correlated with grip strength (P < 0.05). The results of the quantile g-computation showed that the risk of occurrence of grip strength reduction was -1.007 (95％ confidence interval:-1.362, -0.652; P < 0.001) when each quartile of the mixture of the seven metals was increased. Bayesian kernel function regression model analysis showed that mixtures of the seven metals had a negative overall effect on grip strength, with Cu, As and Sr being negatively associated with grip strength levels. In the total population, potential interactions were observed between As and Mn and between Cu and Mn (Pinteractions of 0.003 and 0.018, respectively).Conclusion In summary, this study suggests that combined exposure to metal mixtures is negatively associated with grip strength. Cu, Sr and As were negatively correlated with grip strength levels, and there were potential interactions between As and Mn and between Cu and Mn.

Objective To investigate the neuroprotective effect of 7,8-dihydroxyflavone(7,8-DHF)on cerebral hemorrhage in mice by regulating the tyrosine kinase receptor B(TrkB)/brain derived neurotrophic factor(BDNF)signal pathway.Methods C57BL/6 mice were injected with bacterial collagenase V Ⅱ to establish cerebral hemorrhage model.The mice were randomly grouped into model group,control group(5 mg·kg-1 7,8-DHF),experimental group(1 mg·kg-1 K252a),and combined group(5 mg·kg-1 7,8-DHF+1 mg·kg-1 K252a),mice injected with normal saline were used as sham-operation group,with 10 mice in each group.After the treatment,the mice were scored for neurological function by Garcia method,brain water contents of the brain tissue were detected by the dry and wet weight method,the blood brain barrier permeability was examined using the Evans blue method,the neuronal apoptosis was detected by TUNEL method,and the protein expression levels of TrkB,phosphorylated TrkB(p-TrkB)and BDNF were detected by Western blot.Results The neurological function scores of control,experimental,combined,model and sham-operation groups were(15.47±1.55),(7.23±0.73),(10.55±1.06),(10.45±1.05)and(16.12±1.62)points;the brain water contents were(62.88±2.19)％,(83.77±3.11)％,(72.71±2.59)％,(72.88±2.61)％and(59.64±2.06)％;the Evans blue contents were(3.26±0.36),(16.23±1.63),(8.78±0.88),(9.47±0.95)and(1.02±0.11)μg·g-1;neuronal apoptosis rates were(9.82±0.99)％,(39.88±3.99)％,(22.15±2.24)％,(25.71±2.58)％and(6.46±0.65)％;p-TrkB/TrkB ratios were 1.01±0.11,0.21±0.03,0.48±0.05,0.49±0.05 and 1.03±0.11;the protein expression levels of BDNF were 1.15±0.12,0.18±0.02,0.46±0.05,0.42±0.05 and 1.18±0.12,respectively.The above indexes of sham-operation,control and experimental groups were compared with those of model group,and the above indexes of control and experimental groups were compared with those of combined group,the differences were statistically significant(all P＜0.05).Conclusion 7,8-DHF has neuroprotective effect on mice with intracerebral hemorrhage,and its mechanism may be related to the activation of TrkB/BDNF signal pathway.

Inflammasome is a kind of intracellular polyprotein complex,which is an important component of the complex system of local inflammatory microenvironment after human tissue damage.When the inflammasome is activated,it induces the activation of cysteine aspartate proteinase 1(caspase-1),mediates the maturation and secretion of proinflammatory cytokines,such as interleukin(IL)-1 β and IL-18,and induces cell death,which plays an important role in regulating the host immune response to pathogen infection and tissue repair of cell damage.Nod-like receptor protein 3(NLRP3)inflammatory body,which is composed of NLRP3,pro-cysteine aspartic acid specific protease-1(pro-caspase-1)and apoptosis-related spot-like protein(ASC),is the most deeply and widely studied type of inflammatory body,which plays an important role in the regulation of inflammation.When NLRP3 inflammatory bodies are activated,inflammatory mediators are produced and released,which participate in the occurrence and development of a variety of inflammatory diseases.Some studies have shown that traditional Chinese medicine can improve the pathological state of a variety of diseases by inhibiting NLRP3 inflammatory bodies,and play a role in the prevention and treatment of a variety of inflammatory diseases,including cardiovascular diseases,joint inflammation,diabetes and so on.This paper systematically combs the mechanism of NLRP3 inflammatory bodies,and summarizes the latest research reports on the effects of traditional Chinese medicine compound prescription,traditional Chinese medicine monomers and traditional Chinese medicine extracts on NLRP3 inflammatory bodies in the treatment of inflammatory diseases,in order to provide new ideas for the further study of the pathogenesis and drug treatment of many inflammatory diseases.

Following the principles of evidence-based medicine,in accordance with the structure and drafting rules of standardized documents,based on literature research,according to the characteristics of chronic cough in children and issues that need to form a consensus,the TCM Guidelines for Diagnosis and Treatment of Chronic Cough in Children was formulated based on the Delphi method,expert discussion meetings,and public solicitation of opinions.The guideline includes scope of application,terms and definitions,eti-ology and diagnosis,auxiliary examination,treatment,prevention and care.The aim is to clarify the optimal treatment plan of Chinese medicine in the diagnosis and treatment of this disease,and to provide guidance for improving the clinical diagnosis and treatment of chronic cough in children with Chinese medicine.

BACKGROUND:Previous studies have shown that knee joint moment changes in patients with knee osteoarthritis,but there are few reports on the correlation of moment changes with knee extensor muscle strength and gait spatiotemporal parameters. OBJECTIVE:To explore the correlation of knee extensor muscle strength and gait spatiotemporal parameters with peak knee flexion moment and knee adduction moment in female patients with knee osteoarthritis. METHODS:Twenty knee osteoarthritis female patients with single knee disease hospitalized in Guizhou Hospital,Beijing Jishuitan Hospital from February to August 2022 were selected as the knee osteoarthritis group,and an additional 20 healthy females without musculoskeletal disease were selected as the control group.The knee extensor force at 60(°)/s was measured with the Biodex isokinetic instrument.The gait spatiotemporal parameters and peak knee flexion moment and knee adduction moment were collected with the Italian BTS infrared motion capture system and force measuring platform.Pearson correlation analysis was used to explore the correlation of muscle strength and gait spatiotemporal parameters with peak knee adduction moment and knee flexion moment,and the variables significantly related to knee joint moment were further included in the multiple stepwise regression analysis. RESULTS AND CONCLUSION:(1)Compared with the control group,the knee osteoarthritis group had significantly lower knee extensor force,step speed,step frequency,step length,step width,peak knee adduction moment and knee flexion moment at 60(°)/s(P<0.05).(2)Pearson correlation analysis showed that the 60(°)/s centripetal extensor force,step speed,step frequency and step length were positively correlated with the peak knee flexion moment,and negatively correlated with the peak knee adduction moment,with a statistically significant difference(P<0.05).(3)The results of multiple stepwise regression showed that step speed and 60(°)/s knee extensor force were the strongest predictors of peak knee flexion moment,and the total R2 value of the two factors was 0.426,indicating that 42.6%of the total variance of this parameter could be explained.Step length and 60(°)/s centripetal extensor force were the strongest predictors of peak knee adduction moment.The total R2 value of the two factors was 0.602,indicating that 60.2%of the total variance of this parameter could be explained.(4)It is concluded that knee extensor strength,step speed and step length are the main variables affecting peak knee adduction moment and knee flexion moment.Therefore,these variables can be used for clinical gait monitoring and guidance to change knee joint load during knee osteoarthritis rehabilitation.