Gorham-Stout disease(GSD) is a rare osteolytic disorder characterized by spontaneous and progressive osteolysis, along with abnormal angiogenesis and lymphangiogenesis, with no new bone formation. We present a case of a 15-year-old female admitted due to " recurrent right leg pain for 5 years, 11 months after undergoing right femoral fracture surgery". Through comprehensive integration of the patient's clinical phenotype, laboratory tests, imaging findings, pathological examinations, and molecular biological test results, GSD was considered highly likely. A multidisciplinary treatment approach was conducted, including a combination of zoledronic acid and sirolimus to inhibit osteolysis, along with rehabilitation training and orthopedic intervention, providing a personalized and comprehensive treatment strategy.

Objective:To explore the clinical characteristics, diagnosis and treatment strategies, and prognosis of pulmonary embolism (PE) complicating childhood rheumatic diseases.Methods:A retrospective case series study was performed on the demographic data, laboratory indicators, imaging features, treatment regimens, and follow-up data of 8 children with rheumatic diseases complicated by PE who were admitted to the Department of Rheumatology and Immunology, Capital Center for Children′s Health, Capital Medical University from January 2014 to October 2023.Results:Among the 8 children, there were 4 boys and 4 girls, with an age of 12.0 (7.5, 13.0) years. Among the primary diseases, there were 3 cases of systemic lupus erythematosus, 2 cases of Beh?et′s disease, 2 cases of Takayasu arteritis, and 1 case of antiphospholipid syndrome. All children developed PE during the active phase of the primary disease. PE was detected at the onset of the primary disease in 3 cases, and the median time from the diagnosis of the primary disease to the development of PE was 10.0 (6.0, 25.0) months in the remaining 5 cases. Fever was present in all 8 children, 4 cases were accompanied by chest tightness, dyspnea, etc., and 2 cases only presented with fever. Laboratory examinations revealed the following results: erythrocyte sedimentation rate was 42.0 (17.0, 78.0) mm/1 h, high-sensitivity C-reactive protein was 12.7 (2.6, 78.7) mg/L, white blood cell count was 9.6 (7.2, 18.7)×10 9/L; D-dimer was 2.3 (0.9, 6.2) mg/L; and hemoglobin was (109±16) g/L.Imaging examinations revealed that 5 cases had involvement of the bilateral lower pulmonary arteries, 5 cases had peripheral embolism, and 3 cases had central PE. Complications included 3 cases of deep vein thrombosis, 2 cases of intracranial venous sinus thrombosis, and 1 case of mild pulmonary hypertension.In terms of treatment, 7 cases received anticoagulation with heparin followed by warfarin. Immunomodulation was mainly based on glucocorticoids combined with immunosuppressants, and 4 cases were combined with biological agents. The follow-up time of 4.17 (1.75, 7.17) years, the time for complete absorption of PE was 10.5 (6.0, 18.0) months; all 8 children had no target events, with no recurrence or chronic thromboembolic pulmonary hypertension, and the pulmonary artery remodeling was good. Conclusions:PE complicating childhood rheumatic diseases is closely related to the activity of the primary disease. The clinical manifestations are insidious, with fever as the main symptom. Imaging examination is the key to diagnosis.Early adoption of heparin followed by warfarin anticoagulation and glucocorticoids combined with immunosuppressants and (or) biological agents to control the primary disease can achieve a favorable prognosis.

Objective:To explore the clinical characteristics, diagnosis and treatment strategies, and prognosis of pulmonary embolism (PE) complicating childhood rheumatic diseases.Methods:A retrospective case series study was performed on the demographic data, laboratory indicators, imaging features, treatment regimens, and follow-up data of 8 children with rheumatic diseases complicated by PE who were admitted to the Department of Rheumatology and Immunology, Capital Center for Children′s Health, Capital Medical University from January 2014 to October 2023.Results:Among the 8 children, there were 4 boys and 4 girls, with an age of 12.0 (7.5, 13.0) years. Among the primary diseases, there were 3 cases of systemic lupus erythematosus, 2 cases of Beh?et′s disease, 2 cases of Takayasu arteritis, and 1 case of antiphospholipid syndrome. All children developed PE during the active phase of the primary disease. PE was detected at the onset of the primary disease in 3 cases, and the median time from the diagnosis of the primary disease to the development of PE was 10.0 (6.0, 25.0) months in the remaining 5 cases. Fever was present in all 8 children, 4 cases were accompanied by chest tightness, dyspnea, etc., and 2 cases only presented with fever. Laboratory examinations revealed the following results: erythrocyte sedimentation rate was 42.0 (17.0, 78.0) mm/1 h, high-sensitivity C-reactive protein was 12.7 (2.6, 78.7) mg/L, white blood cell count was 9.6 (7.2, 18.7)×10 9/L; D-dimer was 2.3 (0.9, 6.2) mg/L; and hemoglobin was (109±16) g/L.Imaging examinations revealed that 5 cases had involvement of the bilateral lower pulmonary arteries, 5 cases had peripheral embolism, and 3 cases had central PE. Complications included 3 cases of deep vein thrombosis, 2 cases of intracranial venous sinus thrombosis, and 1 case of mild pulmonary hypertension.In terms of treatment, 7 cases received anticoagulation with heparin followed by warfarin. Immunomodulation was mainly based on glucocorticoids combined with immunosuppressants, and 4 cases were combined with biological agents. The follow-up time of 4.17 (1.75, 7.17) years, the time for complete absorption of PE was 10.5 (6.0, 18.0) months; all 8 children had no target events, with no recurrence or chronic thromboembolic pulmonary hypertension, and the pulmonary artery remodeling was good. Conclusions:PE complicating childhood rheumatic diseases is closely related to the activity of the primary disease. The clinical manifestations are insidious, with fever as the main symptom. Imaging examination is the key to diagnosis.Early adoption of heparin followed by warfarin anticoagulation and glucocorticoids combined with immunosuppressants and (or) biological agents to control the primary disease can achieve a favorable prognosis.

Objectives: . This study aimed to investigate the regulatory functions of gamma-aminobutyric acid (GABA)ergic neural circuits from the inferior colliculus (IC) to the medial geniculate body (MGB) in salicylate-induced tinnitus. Methods: . Mice were treated with salicylate to induce tinnitus, and tinnitus-like behaviors were evaluated via gap prepulse inhibition of acoustic startle. Using combined viral tracing methodologies, we identified and mapped the pathways and connections from the IC to the MGB. Furthermore, we employed Gq-coupled human M3 designer receptors exclusively activated by designer drugs (DREADDs) and Gi-coupled human M4 DREADDs to achieve targeted excitation or suppression of GABAergic neurons in the IC and MGB. Following the administration of clozapine N-oxide, which binds to these receptors, we modulated these neural circuits to assess their impact on tinnitus severity in a mouse model. Results: . Our findings demonstrated that mice exposed to salicylate exhibited tinnitus-like behaviors. GABAergic neurons projecting retrogradely from the MGB to the IC were primarily concentrated in the external nucleus of the IC. After clozapine N-oxide administration, chemogenetic activation of IC-MGB GABAergic neurons aggravated salicylate-induced tinnitus. Additionally, activation of GABAergic neurons between the IC and MGB induced the perception of tinnitus even without salicylate. However, chemogenetic inhibition of the IC-MGB GABAergic circuit did not reverse salicylate-induced tinnitus. Conclusion . These findings suggest that activation of the IC-MGB GABAergic neural circuit may contribute to tinnitus generation through a mechanism distinct from that of salicylate-induced tinnitus. This study provides novel insights into the mechanisms underlying tinnitus.

ObjectiveThis study aims to develop and validate a novel multimodal predictive model， termed criss-cross network（CCNet）-directed graph neural network（DGNN）（CGN）， for accurate assessment of pulmonary nodule progression in high-risk individuals for lung cancer， by integrating longitudinal chest computed tomography（CT） imaging with both traditional Chinese and western clinical evaluation data. MethodsA cohort of 4 432 patients with pulmonary nodules was retrospectively analyzed. A twin CCNet was employed to extract spatiotemporal representations from paired sequential CT scans. Structured clinical assessment and imaging-derived features were encoded via a multilayer perceptron， and a similarity-based alignment strategy was adopted to harmonize multimodal imaging features across temporal dimensions. Subsequently， a DGNN was constructed to integrate heterogeneous features， where nodes represented modality-specific embeddings and edges denoted inter-modal information flow. Finally， model optimization was performed using a joint loss function combining cross-entropy and cosine similarity loss， facilitating robust classification of nodule progression status. ResultsThe proposed CGN model demonstrated superior predictive performance on the held-out test set， achieving an area under the receiver operating characteristic curve（AUC） of 0.830， accuracy of 0.843， sensitivity of 0.657， specificity of 0.712， Cohen's Kappa of 0.417， and F₁ score of 0.544. Compared with unimodal baselines， the CGN model yielded a 36%-48% relative improvement in AUC. Ablation studies revealed a 2%-22% increase in AUC when compared to simplified architectures lacking key components， substantiating the efficacy of the proposed multimodal fusion strategy and modular design. Incorporation of traditional Chinese medicine （TCM）-specific symptomatology led to an additional 5% improvement in AUC， underscoring the complementary value of integrating TCM and western clinical data. Through gradient-weighted activation mapping visualization analysis， it was found that the model's attention predominantly focused on nodule regions and effectively captured dynamic associations between clinical data and imaging-derived features. ConclusionThe CGN model， by synergistically combining cross-attention encoding with directed graph-based feature integration， enables effective alignment and fusion of heterogeneous multimodal data. The incorporation of both TCM and western clinical information facilitates complementary feature enrichment， thereby enhancing predictive accuracy for pulmonary nodule progression. This approach holds significant potential for supporting intelligent risk stratification and personalized surveillance strategies in lung cancer prevention.

Objective This study aims to investigate the current status and potential profiles of emotional inhibition in stoma patients with colorectal cancer,to analyze the differences and influencing factors,and thereby provide a basis for the development of intervention strategies.Methods A convenience sample of 348 colorectal cancer patients with ostomies was recruited from colorectal surgery departments and ostomy outpatient clinics of a tertiary A hospital in Anhui Province.Data were collected using the General Information Questionnaire,Emotional Inhibition Scale,Chinese Perceived Stress Scale,10 item Conner Davidson Resilience Scale,and Family Care Index Questionnaire.Latent profile analysis was performed using Mplus 8.3,while univariate analysis and multivariate logistic regression analysis were conducted using SPSS 26.0.Results Valid questionnaires were collected from 336 participants(response rate 96.6％).Colorectal cancer patients with ostomies demonstrated a mean emotional inhibition score of 30.84±10.49.Latent profile analysis identified 3 distinct emotional inhibition patterns:high inhibition-suppressive type(31.25％),moderate inhibition-adaptive type(39.88％),and low inhibition-excessive type(28.87％).Gender,residential location,household income per capita,ostomy self-care proficiency,perceived stress levels,psychological resilience scores and family functioning status emerged as significant determinants of emotional inhibition heterogeneity among colorectal cancer patients with ostomies across distinct latent profiles(P＜0.05).Conclusion Colorectal cancer patients with ostomies exhibit significant heterogeneity in emotional inhibition profiles.Clinicians should conduct early identification of these latent classes through standardized assessments and develop tailored interventions to improve emotional inhibition outcomes in this clinical population.

Objective To explore and analyze the current situation of consumer health activation index and its influencing factors in hospitalized elderly patients with multiple chronic conditions,and to provide reference basis for clinical intervention.Methods Convenience sampling method was used to select 332 hospitalized elderly patients with multiple chronic conditions from a tertiary hospital in Hefei City from July to December 2024 as the study subjects,and general information questionnaire,Consumer Health Activation Index,Health Literacy Management Scale,Scale of the Elderly Self-neglcet(Rural),and Perceived Social Support Scale were used to investigate the current situation of consumer health activation index of hospitalized elderly patients with multiple chronic conditions.Univariate analysis and multiple linear regression analysis were employed to explore the influencing factors of consumer health activation index in patients with multiple chronic conditions.Results A total of 332 questionnaires were distributed,and 325 valid questionnaires were retrieved.The score of the consumer health activation index of hospitalized elderly patients with multiple chronic conditions was(61.68±15.12).The results of multiple linear regression showed that literacy,average monthly household income,number of chronic diseases,medication frequency,self-care ability,health literacy management,perceived social support and self-neglect were the influencing factors for the positivity of hospitalized elderly patients with multiple chronic conditions(P＜0.05),which could explain 59.9％of the variance of the consumer health activation index of hospitalized elderly patients with multiple chronic conditions.Conclusion The consumer health activation index of hospitalized elderly patients with multiple chronic conditions is at a low level.In the future,precise and systematic intervention measures for enhancing positive health status can be formulated by taking into account relevant influencing factors,in order to promote the transformation of patients from passive health to active health,and thereby facilitating positive aging.

Objective To explore the impact and threshold effect of productive engagement on the elderly patients with chronic diseases with demoralization syndrome,and to provide theoretical basis for further improving the elderly patients with chronic diseases with demoralization syndrome.Methods A total of 350 elderly patients with chronic diseases from July to December 2024 in a tertiary hospital in Hefei were selected as the research subjects by convenience sampling method,and the general information questionnaire,Productive Engagement Scale and Demoralization Scale-Ⅱ were used for investigation.Using multiple stratified regression analysis to explore the impact of participation in productive activities on dementia syndrome in elderly patients with chronic diseases,and conducting threshold effect analysis.Results 341 valid questionnaires were collected.The score of demoralization syndrome in elderly patients with chronic diseases was 14.00(11.00,17.00),and the score of productive engagement was 37.00(29.50,46.00).Stratified regression analysis showed that productive engagement could independently explain 8.7％of the variance(P＜0.05).Curve fitting and threshold effect analysis showed that the degree of demoralization syndrome was not affected when the score of productive engagement of elderly patients with chronic diseases was ≤27 points(P＞0.05),and it was decreased with the increasing of productive engagement score＞27 points(P＜0.001).Conclusion The demoralization syndrome of elderly patients with chronic diseases were in the middle level,and productive engagement is negatively correlated with demoralization syndrome.Healthcare professionals can improve the level of demoralization syndrome by encouraging elderly patients with chronic diseases to actively participate in productive engagement activities.

Aim To explore the in vitro anti-human triple-negative breast cancer(TNBC)effect and mech-anism of Austocystin R.Methods MTT assay was used to evaluate the anti-tumor potential of Austocystin R for various human tumor cells and normal cells.Flow cytometry was employed to evaluate the influence on cell cycle progression.mRFP-GFP-LC3 adenovirus transfection was used to evaluate the autophagic flux process.Western blot assay was used to verify the effect of Austocystin R on the expression of related pro-teins.Results The results showed that Austocystin R significantly inhibited the proliferation of multiple tumor cells in a dose-dependent manner,especially for the MDA-MB-231 cells with an IC50 of 1.45μmol·L-1.In addition,Austocystin R increased the protein expression of PTEN,p53,p-p53,p27,p21,and down-regulated the expression of p-PI3K,p-AKT and p-mTOR.Austocystin R can significantly increase the proportion of S-phase MDA-MB-231 cells,inhibit the expression of Cyclin D1,CDK4,CDK6,Rb,Cyclin B1 and CDK1,and promote the expression of Cyclin E1 and CDK2.Austocystin R can promote the autophagic flux process of MDA-MB-231 cells,promote the expres-sion of LC3 Ⅰ/Ⅱ,p-Beclin-1,p-ULK1,HMGB-1 and Atg 14 proteins,and inhibit the expression of Beclin-1,ULK1,p62,ATG 3,ATG 4B,ATG 5,ATG 7,ATG 12,ATG 13 and ATG 16L1 proteins.Conclusion Austo-cystin R can exhibit its anti-TNBC activity by inhibi-ting the PI3K/AKT/mTOR signaling pathway,blocking the cell cycle at the S phase and inducing autophagic cell death.

Purpose To explore the correlation between spectral CT-derived quantitative parameters and different programmed death-ligand 1(PD-L1)expression status in gastric adenocarcinoma,in order to provide additional imaging-based references for immunotherapy regimen selection in patients with this disease.Materials and Methods A total of 45 patients with gastric adenocarcinoma,pathologically confirmed by biopsy,who underwent PD-L1 testing prior to treatment were retrospectively enrolled from Lanzhou University Second Hospital between January 2021 and September 2023.All patients underwent baseline spectral CT scans.Based on PD-L1 expression status,patients were categorized into the higher combined positive score(CPS)group(CPS≥5)and the lower CPS group(CPS<5).From the single-energy images of arterial and venous phases,the CT values(CT40 keV,CT70 keV),effective atomic number,and iodine concentration of the lesions were measured.The standardized iodine concentration and slope of the spectral curve were calculated.Differences in spectral CT parameters between the two groups were compared.Receiver operating characteristic curves were plotted for parameters with statistically significant differences,and the area under the curve was calculated to evaluate the efficacy of each single parameter and combined parameters in distinguishing PD-L1 expression status.Results In the arterial phase,the CT40 keV,CT70 keV,iodine concentration,standardized iodine concentration,effective atomic number,and slope of the spectral curve of lesions in the higher expression group were all significantly higher than those of lesions in the lower expression group(t/Z=-3.115 to-2.725,all P<0.05),whereas no significant differences were observed in all parameters in the venous phase(all P>0.05).For the standardized iodine concentration in the enhanced arterial phase,the area under the curve for distinguishing PD-L1 expression status was 0.755(95%CI 0.612 to 0.898),with a sensitivity of 81.8%and a specificity of 65.2%.No significant differences were found between it and other single spectral CT parameters in the arterial phase(all P>0.05),and its diagnostic efficacy was similar to that of the combined parameters[the area under the curve was 0.755(95%CI 0.613 to 0.897),sensitivity was 90.9%,specificity was 52.2%].Conclusion Quantitative parameters of spectral CT in the arterial phase can predict the PD-L1 expression of gastric adenocarcinoma,which may provide a reference for the screening and immunotherapy evaluation of patients with gastric adenocarcinoma.