Pulmonary lymphangioleiomyomatosis is a rare disease characterized by the abnormal proliferation of pulmonary lymphatic smooth muscle cells. It is common in women and often accompanied by recurrent pneumothorax, chylothorax and progressive dyspnea, imaging characterized by diffuse cystic lesions in both lungs. Pulmonary lymphangioleiomyomatosis progresses aggressively and has a very poor prognosis, with a lack of effective medical treatment options in the advanced stages. Lung transplantation is a safe and effective method for the treatment of advanced pulmonary lymphangioleiomyomatosis, which may significantly improve the survival rate and quality of life of patients. The median survival period after surgery can reach 12 years. This article reviews the pathogenesis, diagnosis, treatment of pulmonary lymphangioleiomyomatosis, and the current status and existing problems of lung transplantation in pulmonary lymphangioleiomyomatosis, aiming to provide a reference for the clinical treatment and subsequent research of pulmonary lymphangioleiomyomatosis.

Objective To address the challenges of poor performance and lack of interpretability in existing models,the SHAP algorithm is used to develop an interpretable machine learning model that offers a novel approach to wound age estimation,Methods Based on the previous discovery of the expression of 35 wound age healing-related genes in contused skeletal muscle,the woun age estimaton model was constructed using four algorithms,namly,Multilayer Perceptron(MLP),Random Forest(RF),LightGBM(LGBM),and Support Vector Machine(SVM).The SHAP(Shapley Additive Explanation)algorithm was used to rank the importance of genetic features,eliminate redundant attributes,and optimize the model for accurate wound age estimation.the genetic features of the optimal model were analyzed using SHAP's local interpretation capabilities.Results The best results were obtained using model of MLP(area under the curve(AUC)=0.99)The wound ages were classified into four categories:4～12 h,16～24 h,28～36 h,and 40～48 h,using only 15 gene features.According to SHAP analysis,Fam210a was identified as the most relevant gene.Local analysis revealed that high expression of Fam210a contributed to an increase in the predicted probability of 4 h～12 h,while high expression of Rae1 contributed to an increase in the predicted probability of 16 h～24 h.Additionally,low expression of Tbx18 contributed to an increase in the predicted probability of 28 h～36 h,whereas high expression of Tbx18 contributed to an increase in the predicted probability of 40 h～48 h.Conclusions The combined MLP and SHAP model can be used to predict wound age.Using the SHAP interpreter can better understand the degree of contribution of feature genes to the model prediction,and lay the foundation for further in-depth study of wound age estimation.

Objective To construct and validate a risk prediction model for delayed healing of venous leg ulcer(VLU),so as to provide a reference basis for early identification of people at high risk of delayed healing.Methods Using a convenience sampling method,331 VLU patients attending vascular surgery departments in 2 tertiary A hospitals in Sichuan Province from January 2018 to December 2022 were selected as a modeling group and an internal validation group,and 112 patients admitted to another tertiary A hospital were selected as an external validation group.Risk factors for delayed healing in VLU patients were screened using univariate analysis,LASSO regression,and multivariate logistic regression analysis,and a risk prediction model was constructed using R software,and the predictive effects of the models were examined using the area under the receiver operating characteristic curve,the Hosmer-Lemeshow test,decision curve,and the bootstrap resampling for internal validation and spatial external validation were performed,respectively.Results The predictors that ultimately entered the prediction model were diabetes(OR=4.752),deep vein thrombosis(OR=4.104),lipodermatosclerosis(OR=5.405),ulcer recurrence(OR=3.239),and ankle mobility(OR=5.520).The model had good discrimination(AUC:0.819 for internal validation and 0.858 for external validation),calibration(Hosmer-Lemeshow test:χ2=13.517,P=0.095 for internal validation and χ2=3.375,P=0.909 for external validation)and clinical validity.Conclusion The model constructed in this study has good differentiation and calibration,and it can effectively predict people at high risk of delayed healing of VLU,which facilitates targeted clinical interventions to improve ulcer outcomes and reduce the risk of delayed ulcer healing.

The purpose of this study is to construct a muscle-specific synthetic promoter library, screen out muscle-specific promoters with high activity, analyze the relationship between element composition and activity of highly active promoters, and provide a theoretical basis for artificial synthesis of promoters. In this study, 19 promoter fragments derived from muscle-specific elements, conserved elements, and viral regulatory sequences were selected and randomLy connected to construct a muscle-specific synthetic promoter library. The luciferase plasmids pCMV-Luc and pSPs-Luc were constructed and transfected into the myoblast cell line C2C12. The activities of the synthesized promoters were evaluated by the luciferase activity assay. Two non-muscle-derived cell lines HeLa and 3T3 were used to verify the muscle specificity of the highly active promoters. The sequences of promoters with high activity, good muscle specificity, and correct sequences were analyzed to explore the relationship between the element composition and activity of promoters. We successfully constructed a muscle-specific promoter library and screened out 321 effective synthetic promoter plasmids. Among them, the activity of SP-301 promoter was 5.63 times that of CMV. The 15 promoters with high activity were muscle-specific. In the promoters with high activity and correct sequences, there was a relationship between their element composition and activity. Muscle-specific elements accounted for a high proportion in the promoters, while they had weak correlations with the promoter activity, being tissue-specific determinants. Viral elements accounted for no less than 20% in highly active promoters, which may be the key elements for the promoter activity. The content of conserved elements was proportional to the promoter activity. This study lays a theoretical foundation for the synthesis of tissue-specific efficient promoters and provides a new idea for the construction and application of in-situ gene delivery systems.
Promoter Regions, Genetic ; Humans ; Animals ; Mice ; Gene Library ; Cell Line ; Transfection ; HeLa Cells ; Luciferases/metabolism* ; Muscle, Skeletal/metabolism* ; Plasmids/genetics* ; Myoblasts/cytology*

Objective:To investigate the effectiveness of nasal endoscopic anterior lacrimal recess approach combined with temporary fenestration of the nasal septum in resecting recurrent nasal inverted papilloma. Methods:Patients with recurrent nasal inverted papilloma who underwent reoperation in our hospital during the past 2 years were included . The nasal septum may hinder full access to and effective treatment of the lesions at the anterior and medial wall of the maxillary sinus by endoscope, aspirator and surgical instrument in the narrow aperture of the prelacrimal recess, although these lesions could be observed by 70° nasal endoscope. Results:The nasal septum is temporarily opened on the basis of the prelacrimal recess approach, and the nasal endoscope and instrument was introduced through trans-septal window, so as to provide a better view of the operative field and the angular range of the instrument's movement. Conclusion:The recurrent nasal inverted papilloma could be successfully managed by re-endoscopic anterior lacrimal recess approach combined with temporary fenestration of the nasal septum, and no recurrence was observed during the 2-year follow-up. This surgical approach is recommended for the inverted papilla which originates from the anterior medial wall of the maxillary sinus, as the tumor can be removed completely using this surgical approach.
Humans ; Papilloma, Inverted/pathology* ; Endoscopy ; Maxillary Sinus/pathology* ; Lacrimal Apparatus/surgery* ; Treatment Outcome ; Retrospective Studies

Objective:To investigate the correlation and predictive value of fibrinogen-to-albumin ratio (FAR) and clinical outcomes after intravenous thrombolysis in patients with acute ischemic stroke.Methods:Patients with acute ischemic stroke received intravenous thrombolysis treatment at Fuyang People's Hospital from November 2019 to August 2022 were retrospectively included. Their clinical, imaging, and laboratory data were collected. After 3 months of onset, a modified Rankin Scale was used for clinical outcome evaluation and a score >2 were defined as poor outcome. Multivariate logistic regression analysis was used to determine the correlation between FAR and poor outcome. Receiver operating characteristic (ROC) curve was used to evaluate the predictive value of FAR on adverse outcomes after intravenous thrombolysis in patients with acute ischemic stroke. Results:A total of 162 patients were included. There were 114 patients (70.4%) in the good outcome group and 48 (29.6%) in the poor outcome group. Univariate analysis showed that the baseline National Institutes of Health Stroke Scale (NIHSS) score, fasting blood glucose, fibrinogen, and FAR in the poor outcome group were significantly higher than those in the good outcome group, while the serum albumin was significantly lower than that of the good outcome group (all P<0.05). Multivariate logistic regression analysis showed that the baseline NIHSS score (odds ratio [ OR] 1.176, 95% confidence interval [ CI] 1.082-1.227; P<0.001), fasting blood glucose ( OR 1.206, 95% CI 1.018-1.430; P=0.030), and FAR ( OR 1.448, 95% CI 1.449-1.824; P=0.002) were the independent risk factors for poor outcome. The ROC curve analysis showed that the area under the curve of FAR for predicting poor outcomes was 0.706 (95% CI 0.616-0.796). When the FAR cutoff value was 8.06, the sensitivity and specificity were 66.7% and 78.2%, respectively. Conclusion:There is a significant correlation between FAR and outcomes after intravenous thrombolysis therapy in patients with acute ischemic stroke. Higher FAR has certain predictive value for poor outcomes of patients.