This article reports a rare case of autoinflammatory disease presenting initially with skin induration and swelling after trauma as the initial manifestation, followed by progressive limb weakness. The patient was a middle-aged female who developed skin induration and swelling after trauma, which gradually progressed to limb weakness, dysarthria and bilateral facial paralysis, accompanied by livedo reticularis of the lower extremities, diffuse skin induration of the limbs, and beaded subcutaneous nodules in the right upper limb. The patient had a susceptibility to infection since childhood and a history of chronic livedo reticularis. Skin pathological examination revealed panniculitis. A comprehensive etiological screening for special infections and autoimmune diseases was completed with an unremarkable results, and whole-exome sequencing showed no abnormal findings. Following a multidisciplinary discussion combined with RNA sequencing results, the patient was diagnosed with an autoinflammatory disease, with a suspected type Ⅰ interferonopathy. Treatment with tofacitinib resulted in gradual improvement of clinical symptoms. This case highlights the importance of detailed medical history collection, systematic physical examination and multidisciplinary collaborative diagnosis and treatment, and underscores the pivotal role of molecular diagnosis in the confirmation of rare diseases. It can provide a reference for the clinical diagnosis and management of similar rare cases.

This paper summarizes professor ZHU Shengliang's academic idea and clinical experience in treating gastroesophageal reflux disease （GERD） with qi constraint and phlegm obstruction syndrome. It is believed that the core pathogenesis of GERD is the dysfunction of the liver， gallbladder， spleen and stomach， with binding of phlegm and stasis. The basic pathogenesis is the liver and gallbladder failing to dredge and disperse， the stomach failing to harmonize and descend， and the stomach qi counterflowing upward. In clinical practice， the treatment principle of regulating qi as the priority， resolving phlegm as the key， and activating blood as the assistant is applied， with harmonizing the stomach and directing counterflow downward throughout the whole process. A self-made Shugan Hewei Huatan Formula （疏肝和胃化痰方） is employed with the function of soothing the liver and rectifying qi， opening constraint and dissipating knot， dissolving phlegm and dispelling stasis， harmonizing stomach and directing counterflow downward， with an emphasis on flexible modification according to the syndrome as well as careful protection of stomach qi.

ObjectiveTo investigate the mechanism by which the Zishen Huoxue prescription （ZSHXP） ameliorates cognitive dysfunction in rats with vascular dementia （VD） induced by the bilateral common carotid artery ligation （2-VO model rats） through regulating the glucose-regulated protein 78 （GRP78）/protein kinase R-like endoplasmic reticulum kinase （PERK）/activating transcription factor 4 （ATF4） signaling pathway. MethodsA VD rat model was established via the 2-VO method. A total of 72 male Sprague-Dawley （SD） rats were randomly divided into six groups： Sham group， Model group， donepezil hydrochloride group （0.45 mg·kg^-1）， and ZSHXP groups at low （8.90 g·kg^-1）， medium （17.80 g·kg^-1）， and high （35.60 g·kg^-1） doses，with 12 rats in each group. The Morris Water Maze test was utilized to assess spatial learning and memory abilities of rats， and the Novel Object Recognition test was used to evaluate cognitive performance. Hematoxylin-eosin （HE） and Nissl staining were applied to observe the histological and morphological changes in hippocampal tissues. Transmission electron microscopy （TEM） was used to observe the morphological changes of endoplasmic reticulum in rat hippocampal neurons. Immunofluorescence staining was adopted to detect the colocalization of neuronal nuclei antigen （NeuN） with GRP78 and βⅢ Tubulin with gasdermin D （GSDMD） in hippocampal neurons. Western blot was used to detect the expression levels of endoplasmic reticulum stress （ERS）-related proteins including GRP78， PERK， ATF4， phosphorylated protein kinase R-like endoplasmic reticulum kinase （p-PERK）， C/EBP homologous protein （CHOP）， NOD-like receptor protein 3 （NLRP3）， Caspase-1 and GSDMD. ResultsCompared with the sham operation group， the model group showed a significantly prolonged escape latency （P<0.01）， a significant decrease in the number of platform crossings and the residence time in the target quadrant （P<0.01）， and a markedly reduced recognition index （P<0.01）. Histological observations revealed that the hippocampal neurons in the model group were disorderly arranged with reduced quantity， deformed and shrunken cell bodies， and pyknotic and hyperchromatic nuclei. The number of Nissl bodies decreased significantly. The number of endoplasmic reticula reduced obviously， accompanied by abnormal dilation and swelling， and the loss of normal folding structure. The fluorescence colocalization of NeuN with GRP78 and βⅢ Tubulin with GSDMD in the hippocampus was significantly increased in the model group. The protein expression levels of GRP78， p-PERK/PERK， ATF4， CHOP， NLRP3， GSDMD and Caspase-1 in the model group were significantly elevated （P<0.01）. Compared with the model group， the donepezil hydrochloride group and the ZSHXP medium- and high-dose groups had a significantly shortened escape latency （P<0.01） and an increased number of platform crossings （P<0.05， P<0.01）. The residence time in the target quadrant was increased in the donepezil hydrochloride group and all ZSHXP groups （P<0.05， P<0.01）， with a significantly improved recognition index （P<0.01）. In the donepezil hydrochloride group and all ZSHXP groups， the number of hippocampal neurons increased with a more compact arrangement and reduced nuclear hyperchromasia. The number of Nissl bodies increased with morphological structures tending to be normal. In the ZSHXP high-dose group， the number of endoplasmic reticula increased and the folding structure was restored. The fluorescence colocalization of NeuN with GRP78 and βⅢ Tubulin with GSDMD in the hippocampus was significantly weakened in the treatment groups. In the donepezil hydrochloride group， the protein expressions of GRP78， ATF4 and CHOP were increased （P<0.01）， while the expression of p-PERK/PERK was decreased （P<0.05）. In the ZSHXP low-dose group， the expressions of GRP78， p-PERK/PERK and CHOP were elevated （P<0.05， P<0.01）. The ZSHXP medium- and high-dose groups showed a significant decrease in the protein expressions of p-PERK/PERK， ATF4 and CHOP （P<0.01）， and the high-dose group had a markedly reduced GRP78 protein expression （P<0.01）. In the donepezil hydrochloride group， the Caspase-1 protein expression was increased （P<0.01） and the NLRP3 protein expression was decreased （P<0.01）. In the ZSHXP low-dose group， the GSDMD expression was elevated （P<0.01） while the NLRP3 protein expression was reduced （P<0.01）. After treatment with medium and high doses of ZSHXP， the protein expression levels of NLRP3， GSDMD and Caspase-1 were significantly decreased （P<0.01）. ConclusionThe ameliorative effect of ZSHXP on cognitive function in 2-VO model rats may be associated with its regulation of the GRP78/PERK/ATF4 signaling pathway， which ameliorates ERS and inhibits neuronal pyroptosis.

ObjectiveTo investigate the mechanism by which the Zishen Huoxue prescription （ZSHXP） ameliorates cognitive dysfunction in rats with vascular dementia （VD） induced by the bilateral common carotid artery ligation （2-VO model rats） through regulating the glucose-regulated protein 78 （GRP78）/protein kinase R-like endoplasmic reticulum kinase （PERK）/activating transcription factor 4 （ATF4） signaling pathway. MethodsA VD rat model was established via the 2-VO method. A total of 72 male Sprague-Dawley （SD） rats were randomly divided into six groups： Sham group， Model group， donepezil hydrochloride group （0.45 mg·kg^-1）， and ZSHXP groups at low （8.90 g·kg^-1）， medium （17.80 g·kg^-1）， and high （35.60 g·kg^-1） doses，with 12 rats in each group. The Morris Water Maze test was utilized to assess spatial learning and memory abilities of rats， and the Novel Object Recognition test was used to evaluate cognitive performance. Hematoxylin-eosin （HE） and Nissl staining were applied to observe the histological and morphological changes in hippocampal tissues. Transmission electron microscopy （TEM） was used to observe the morphological changes of endoplasmic reticulum in rat hippocampal neurons. Immunofluorescence staining was adopted to detect the colocalization of neuronal nuclei antigen （NeuN） with GRP78 and βⅢ Tubulin with gasdermin D （GSDMD） in hippocampal neurons. Western blot was used to detect the expression levels of endoplasmic reticulum stress （ERS）-related proteins including GRP78， PERK， ATF4， phosphorylated protein kinase R-like endoplasmic reticulum kinase （p-PERK）， C/EBP homologous protein （CHOP）， NOD-like receptor protein 3 （NLRP3）， Caspase-1 and GSDMD. ResultsCompared with the sham operation group， the model group showed a significantly prolonged escape latency （P<0.01）， a significant decrease in the number of platform crossings and the residence time in the target quadrant （P<0.01）， and a markedly reduced recognition index （P<0.01）. Histological observations revealed that the hippocampal neurons in the model group were disorderly arranged with reduced quantity， deformed and shrunken cell bodies， and pyknotic and hyperchromatic nuclei. The number of Nissl bodies decreased significantly. The number of endoplasmic reticula reduced obviously， accompanied by abnormal dilation and swelling， and the loss of normal folding structure. The fluorescence colocalization of NeuN with GRP78 and βⅢ Tubulin with GSDMD in the hippocampus was significantly increased in the model group. The protein expression levels of GRP78， p-PERK/PERK， ATF4， CHOP， NLRP3， GSDMD and Caspase-1 in the model group were significantly elevated （P<0.01）. Compared with the model group， the donepezil hydrochloride group and the ZSHXP medium- and high-dose groups had a significantly shortened escape latency （P<0.01） and an increased number of platform crossings （P<0.05， P<0.01）. The residence time in the target quadrant was increased in the donepezil hydrochloride group and all ZSHXP groups （P<0.05， P<0.01）， with a significantly improved recognition index （P<0.01）. In the donepezil hydrochloride group and all ZSHXP groups， the number of hippocampal neurons increased with a more compact arrangement and reduced nuclear hyperchromasia. The number of Nissl bodies increased with morphological structures tending to be normal. In the ZSHXP high-dose group， the number of endoplasmic reticula increased and the folding structure was restored. The fluorescence colocalization of NeuN with GRP78 and βⅢ Tubulin with GSDMD in the hippocampus was significantly weakened in the treatment groups. In the donepezil hydrochloride group， the protein expressions of GRP78， ATF4 and CHOP were increased （P<0.01）， while the expression of p-PERK/PERK was decreased （P<0.05）. In the ZSHXP low-dose group， the expressions of GRP78， p-PERK/PERK and CHOP were elevated （P<0.05， P<0.01）. The ZSHXP medium- and high-dose groups showed a significant decrease in the protein expressions of p-PERK/PERK， ATF4 and CHOP （P<0.01）， and the high-dose group had a markedly reduced GRP78 protein expression （P<0.01）. In the donepezil hydrochloride group， the Caspase-1 protein expression was increased （P<0.01） and the NLRP3 protein expression was decreased （P<0.01）. In the ZSHXP low-dose group， the GSDMD expression was elevated （P<0.01） while the NLRP3 protein expression was reduced （P<0.01）. After treatment with medium and high doses of ZSHXP， the protein expression levels of NLRP3， GSDMD and Caspase-1 were significantly decreased （P<0.01）. ConclusionThe ameliorative effect of ZSHXP on cognitive function in 2-VO model rats may be associated with its regulation of the GRP78/PERK/ATF4 signaling pathway， which ameliorates ERS and inhibits neuronal pyroptosis.

Objective To investigate the influencing factors of stroke patients with hypertension and their compliance with antihypertensive drugs, and to provide targeted intervention measures for stroke prevention in hypertensive patients.  Methods Using the method of multi-stage cluster sampling, a total of 59,434 permanent residents aged 40 and above were selected from 48 monitoring sites in 9 cities of Hebei Province from December 2019 to December 2020. Unconditional logistic regression analysis was used to explore the influencing factors of stroke complicated with hypertension and the compliance of patients with antihypertensive drugs.  Results Among the 59 434 subjects, the prevalence rate of stroke was 4.33% and the prevalence rate of stroke complicated with hypertension was 82.47%. The results of univariate analysis showed that the proportion of women, rural areas, dyslipidemia, diabetes, obesity, and people with family history of stroke was higher in stroke patients with hypertension, and the difference was statistically significant (P<0.05). Multivariate logistic regression analysis showed that rural areas, dyslipidemia, diabetes, obesity, and family history of stroke significantly increased the risk of stroke, and the OR (95%CI) values were 1.29 (1.03-1.62), 1.39 (1.12-1.72), 1.58 (1.25-1.99), 1.61 (1.22-2.12) and 1.60 (1.26-2.04), respectively. Among stroke patients with hypertension, 92.71% of patients took antihypertensive drugs. It was found that women's compliance with antihypertensive drugs was good, with an OR (95%CI) value of 1.46 (1.01-2.09).  Conclusion The prevalence rate of stroke complicated with hypertension is high in people aged 40 and above in Hebei Province. Hypertensive people should lower blood lipids, control blood glucose, and lose weight as soon as possible to prevent the occurrence of stroke.

Objective To explore the value of spectral CT multi-parameter imaging for diagnosing bronchial anthracofibrosis(BAF)complicated with active pulmonary tuberculosis.Methods Totally 77 patients with BAF complicated with atelectasis were retrospectively enrolled.According to undergoing anti-tuberculosis treatment or not during hospitalization,26 patients complicated with active pulmonarg tuberculosis were divided into group A(n=26),while 51 cases without active pulmonarg tuberculosis were divided into group B.Signs indicating active pulmonary tuberculosis(i.e.tree-in-bud sign or centrilobular nodules)on chest spectral CT images were analyzed.Non-enhanced CT(NECT)values,single-energy CT values at 40 keV(CT40 kev)and 70 keV(CT70 kev),as well as effective atomic number(Zeff),iodine concentration(IC),calcium concentration(CC)and hydroxyapatite concentration(HAP)in the arterial phase and venous phase of enhancement at the site of bronchial obstruction and in subcarinal lymph nodes were measured.the slope of spectral line(λ40-70keV)was calculated and compared between groups.Receiver operating characteristic(ROC)curve was plotted,and the area under the curve(AUC)was used to evaluate the efficacy of the above parameter alone and their combinations for diagnosing BAF complicated with active pulmonarg tuberculosis.Results The displaying rate of active pulmonary tuberculosis CT signs in group A was higher than that in group B(P=0.005).NECT values,enhanced venous phase CT40kev,λ40-70 kev,as well as Zeff,IC,CC and HAP at the site of bronchial obstruction in group A were all lower than those in group B(all P＜0.05).The AUC of active pulmonary tuberculosis CT signs for assessing BAF complicated with active pulmonary tuberculosis was 0.659,of the combination of CT quantitative parameters at the site of bronchial obstruction was 0.769,while of the combination of CT signs and CT quantitative parameters was 0.825,higher than of active pulmonary tuberculosis CT signs alone(P＜0.05).Conclusion Spectral CT multi-parameter imaging could be used to effectively diagnose BAF complicated with active pulmonary tuberculosis.

Objective:To investigate the current situation and influencing factors of geriatric nursing competence of nursing students in the later stage of practice, and to provide reference for formulating the reform strategy of geriatric nursing teaching, carrying out nursing education intervention and improving students′ geriatric nursing competence.Methods:From December 2023 to February 2024, 361 students from 6 colleges and universities in Inner Mongolia Autonomous Region were investigated by using the questionnaire of geriatric nursing competence by convenient sampling method.Results:Therefore, 361 nursing students in the late stage of clinical practice who meta the inclusion and exclusion criteria were included, 65 males and 296 females with (21.20 ± 2.02) years. The total score of geriatric nursing competence was (164.47 ± 32.25). Learning motivation ( β=11.09, P<0.05), whether to participate in activities to serve the elderly ( β=10.03, P<0.05), and training level ( β=-4.42, P<0.05) were the influencing factors affecting the elderly care ability of nursing students in the later stage of practice, which explained 12.5% of total variance. Conclusions:The geriatric nursing competence of nursing students in the later stage of practice is generally at a medium level, and the ability of each dimension is not balanced. Nursing educators should enrich the content of geriatric nursing courses, pay attention to the practical activities of the elderly, stimulate students′ learning motivation, and promote the construction of training programs for the geriatric nursing competence of nursing students at different training levels, so as to improve students' geriatric nursing competence.

Objective:To summarize the domestic and international research progress on adverse reactions to contrast-enhanced CT examinations, and forecast research themes, future hotspots, and trends.Methods:Using CiteSpace bibliometric methodology, retrieve Chinese and English literature on adverse reactions in patients undergoing contrast-enhanced CT examinations from China National Knowledge Infrastructure(CNKI), Wanfang Data Knowledge Service Platform, VIP Chinese Science and Technology Periodical Database, and Web of Science. The search period covers database inception to December 31, 2023. After deduplication, CiteSpace and other software are employed to conduct statistics and analyses on publication volume, publishing countries, institutions, and keywords for both Chinese and English literature separately.Results:A total of 3 749 articles were included, comprising 3 044 Chinese-language articles and 705 English-language publications. Visual analysis revealed that the annual number of publications on CT contrast-enhanced examination-related adverse reactions exhibited fluctuating growth from 1994 to 2023. The United States emerged as the leading contributor in this research domain, while China demonstrated rapid growth in recent years. Network analysis of research institutions revealed insufficient collaboration and communication among domestic institutions. Thematic clustering identified 22 clusters and 25 emerging keywords in Chinese literature, compared to 20 clusters and 7 burst keywords in English publications. Domestic research priorities focused predominantly on adverse reactions to contrast agents, nursing interventions, and nursing prevention strategies. International research trends emphasized safety, risk factor identification for adverse reactions, contrast-induced acute kidney injury (CI-AKI) prevention, and the prevention of adverse reactions using contrast-agent nanomaterials.Conclusions:Both domestic and international research in this field exhibits distinct emphases but universally underscores the importance of nursing. It is imperative to strengthen multidisciplinary collaboration among domestic institutions in the future. By focusing on research hotspots and cutting-edge frontiers, expanding the depth and breadth of China's research in this domain, and continuously optimizing nursing strategies, we can effectively enhance nursing quality for CT contrast-enhanced examination patients, optimize patient experience, and contribute to the high-quality development of nursing practice in this field.

Objective:To summarize the clinical and genetic characteristics of 23 children with pathogenic ACAN gene variants, enhance the understanding of this disorder, and explore possible regulatory mechanisms. Methods:A retrospective case series summary.The clinical characteristics and genetic analysis results of 23 children with ACAN gene variants treated in the Department of Endocrinology, Genetics and Metabolism, Children′s Hospital of Soochow University from January 2016 and September 2024 were analyzed retrospectively.Transcriptome sequencing was performed on peripheral blood samples from 3 of affected children and 3 age-matched healthy children as controls.Differentially expressed genes (DEGs) in the peripheral blood transcriptome profiles were identified.Gene ontology (GO) and Kyoto encyclopedia of genes and genomes (KEGG) pathway enrichment analyses were conducted to explore the potential signaling pathways involved. Results:Among the 23 cases, there were 13 males and 10 females, aged from 2 years and 8 months to 12 years old, with 11 cases presenting advanced bone age.Thirteen cases were treated with growth hormone (GH), including 6 cases who received concomitant gonadotropin-releasing hormone analogue therapy.The treatment duration ranged from 3 to 70 months, resulting in varying degrees of height improvement in all treated patients.Transcriptomic analysis identified 811 DEGs, with 516 up-regulated and 295 down-regulated.GO and KEGG enrichment analyses revealed that the heterozygous ACAN variants were significantly associated with FcγR-mediated phagocytosis, nuclear factor-κB signaling pathway, the intestinal immune network for IgA production, rheumatoid arthritis, and systemic lupus erythematosus signaling pathways. Conclusions:The predominant clinical manifestations of patients with ACAN gene variants are short stature and advanced bone age.Although GH provocation tests may indicate normal GH levels, GH therapy can be effective in improving height.Immune-related factors may play a role in the pathogenesis of this disorder.

Objective:To explore the effect of loneliness on self-perceived aging among elderly patients after gastric cancer surgery, and to examine the mediating role of emotion regulation strategies, in order to provide a basis for clinical interventions.Methods:A convenience sample of 220 elderly postoperative gastric cancer patients hospitalized in Shanxi Provincial Cancer Hospital between August and November 2024 was selected. Data were collected using a general demographic questionnaire, the Emotion Regulation Questionnaire (ERQ), the Cancer Loneliness Scale (CLS), and the Brief Aging Perceptions Questionnaire (B-APQ). A structural equation model was constructed using AMOS 26.0, and the significance of the mediating effect was tested using the bootstrap method with 5 000 resamples.Results:A total of 220 questionnaires were distributed in this study, and 203 valid questionnaires were recovered, with a response rate of 92.27%. The total B-APQ score was (47.45±11.43). For the ERQ, the score for cognitive reappraisal was (22.80±8.17), and for expressive suppression was (18.37±5.10). The CLS score was (18.19±6.11). Mediation analysis showed that loneliness had a direct effect of 0.353 on self-perceived aging. Emotion regulation strategy—cognitive reappraisal—partially mediated the relationship between loneliness and self-perceived aging, with a mediation effect value of 0.178, accounting for 33.52% of the total effect (0.178/0.531) .Conclusions:Clinical practitioners should implement interventions to reduce loneliness in elderly patients after gastric cancer surgery and guide them in adopting cognitive reappraisal as an emotion regulation strategy. These approaches may help promote positive aging in this patient population.