Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder primarily caused by pathogenic variants in the TSC1 or TSC2 genes. It is characterized by multisystem hamartomatous lesions and neuropsychiatric manifestations. Here we report a young male patient with TSC involving multiple organs, caused by a heterozygous frameshift mutation in TSC2 (c.2071delC, p.Arg691Alafs^*7). The patient initially presented with classic facial angiofibromas and hypomelanotic macules, and subsequently developed psychiatric and behavioral disturbances with auditory hallucinations. Neuroimaging revealed an intracranial mass lesion, which was confirmed as a subependymal giant cell astrocytoma on postoperative histopathology following surgery performed at an outside institution. After admission, the patient underwent a comprehensive diagnostic workup, and multidisciplinary treatment evaluation revealed extensive multisystem involve-ment, including the nervous system, skin, kidneys, lungs, heart, eyes, pancreas, liver and bones. Combined with relevant literature, this article discusses the molecular mechanisms, clinical phenotypes, and comprehensive management of TSC, in order to provide a reference for the standardized and individualized management of this disease.

The Pharmacovigilance Guidelines for Clinical Application of Traditional Chinese Medicine Injections （hereinafter referred to as the Guidelines） were released by the China Association of Chinese Medicine， with the standard number T/CACM 1563.4—2024. It is the first specialized guideline in China on the approach to pharmacovigilance activities for the clinical application of traditional Chinese medicine injections （TCMIs）. The Guidelines were jointly developed by the Institute of Basic Research in Clinical Medicine， China Academy of Chinese Medical Sciences， along with 30 experts in TCM pharmacovigilance， clinical practice （TCM， as well as integrated traditional Chinese and Western medicine），and evidence-based medicine from across the country. This publication filled the gap in standard documents in this field， both domestically and internationally. The Guidelines were formulated according to GB/T1.1—2020 Directives for standardization—Part 1： Rules for the structure and drafting of standardizing documents， the WHO Handbook for Guideline Development，and other methodological norms. Based on international norms，national laws and regulations，and scientific research results in the field of pharmacovigilance， methods adopted included expert interviews，literature research，nominal group technique， and Delphi method. Then， key points for pharmacovigilance for TCM injections were summarized and clarified in the four critical sections of "monitoring"，"identification"，"assessment"，and "control". The development process of the Guidelines included project initiation， international registration， expert interviews， literature search， and evaluation. Based on the research results of these steps，a draft was formed and revised through multiple rounds of in-group expert discussion and peer evaluations by 56 external experts. After revisions by the working group based on the feedback， the final version was formed. The Guidelines came into effect on January 8，2024，providing suggestions and reference norms for pharmacovigilance in the clinical application of TCMIs. To further promote the application and popularization of the Guidelines and help pharmacovigilance personnel better understand the development process，this study elucidates the background，methodological framework，and key development steps of the Guidelines.

The Pharmacovigilance Guidelines for Clinical Application of Traditional Chinese Medicine Injections （hereinafter referred to as the Guidelines） were released by the China Association of Chinese Medicine， with the standard number T/CACM 1563.4—2024. It is the first specialized guideline in China on the approach to pharmacovigilance activities for the clinical application of traditional Chinese medicine injections （TCMIs）. The Guidelines were jointly developed by the Institute of Basic Research in Clinical Medicine， China Academy of Chinese Medical Sciences， along with 30 experts in TCM pharmacovigilance， clinical practice （TCM， as well as integrated traditional Chinese and Western medicine），and evidence-based medicine from across the country. This publication filled the gap in standard documents in this field， both domestically and internationally. The Guidelines were formulated according to GB/T1.1—2020 Directives for standardization—Part 1： Rules for the structure and drafting of standardizing documents， the WHO Handbook for Guideline Development，and other methodological norms. Based on international norms，national laws and regulations，and scientific research results in the field of pharmacovigilance， methods adopted included expert interviews，literature research，nominal group technique， and Delphi method. Then， key points for pharmacovigilance for TCM injections were summarized and clarified in the four critical sections of "monitoring"，"identification"，"assessment"，and "control". The development process of the Guidelines included project initiation， international registration， expert interviews， literature search， and evaluation. Based on the research results of these steps，a draft was formed and revised through multiple rounds of in-group expert discussion and peer evaluations by 56 external experts. After revisions by the working group based on the feedback， the final version was formed. The Guidelines came into effect on January 8，2024，providing suggestions and reference norms for pharmacovigilance in the clinical application of TCMIs. To further promote the application and popularization of the Guidelines and help pharmacovigilance personnel better understand the development process，this study elucidates the background，methodological framework，and key development steps of the Guidelines.

Objective To analyze the effect of releasing the lower pulmonary ligament on right residual lung expansion after right upper lobe resection under different body mass index (BMI) levels. Methods The clinical data of patients who underwent thoracoscopic right upper lobe resection in the First Affiliated Hospital with Nanjing Medical University from 2021 to 2022 were retrospectively analyzed. Patients were divided into a group A (17 kg/m2＜BMI≤23 kg/m2), a group B (23 kg/m2＜BMI≤29 kg/m2) and a group C (BMI＞29 kg/m2) according to BMI. The presence of residual cavity was judged by chest X-ray at 7-10 days after operation, the degree of compensation change of the right main bronchus angle was measured, and the changes in lung volume were determined by CT three-dimensional reconstruction. Results A total of 157 patients who underwent thoracoscopic right upper lobe resection were included, including 71 males and 86 females, with an average age of (59.7±11.2) years. There were 50 patients in the group A, 75 patients in the group B, and 32 patients in the group C. In the group A, compared with those without releasing the lower pulmonary ligament, patients with releasing had a lower incidence of postoperative residual cavity (P=0.016), greater changes in bronchus angle (P<0.001), and smaller changes in lung volume (P<0.001). In the group B and C, there was no significant effect of releasing the lower pulmonary ligament on postoperative residual cavity, bronchus angle, and lung volume changes (P>0.05). Conclusion For patients with thin and long body shape and low BMI, releasing the lower pulmonary ligament is helpful to promote the expansion of the residual lung after right upper lobe resection and reduce the occurrence of postoperative residual cavity in patients.

ObjectiveTo investigate the effects of Zuoguiwan on osteoclast activation induced by breast cancer and its mechanism. MethodsTo simulate breast cancer-induced osteoclastic bone metastasis， RAW264.7 cells were cultured in conditioned medium containing 50% supernatant of MDA-MB-231 breast cancer cells. The dosages of Zuoguiwan used in the experiment were sera containing 5% and 10% Zuoguiwan. Tartrate-resistant acid phosphatase （TRAP） staining was used to detect osteoclast activation. Enzyme-linked immunosorbent assay （ELISA） was used to measure Cathepsin K secretion from RAW264.7 cells. Real-time quantitative polymerase chain reaction （PCR） was used to detect the mRNA expression levels of osteocalcin （OCN） and bone sialoprotein （BSP）. Immunoprecipitation was employed to detect the interaction between Runt-related transcription factor 2 （Runx2） and core binding factor β subunit （CBF-β）. Western blot was used to assess the protein expression of Runx2， phosphorylated Runx2 （p-Runx2）， extracellular signal-regulated kinases 1/2 （ERK1/2）， p-ERK1/2， p38 mitogen-activated protein kinase （MAPK）， p-p38 MAPK， and CBF-β. ResultsCompared with the blank group， the MDA-MB-231 cell supernatant group showed a significant increase in TRAP-positive cell counts and Cathepsin K secretion. Meanwhile， the expression levels of p-Runx2， Runx2-CBF-β interaction， BSP and OCN mRNA， p-p38 MAPK， and p-ERK1/2 proteins were significantly decreased （P<0.01）. Compared with the MDA-MB-231 cell supernatant group， Zuoguiwan-containing sera significantly reduced TRAP-positive cell counts and Cathepsin K secretion （P<0.01）， significantly increased p-Runx2， BSP and OCN mRNA expression， as well as p-p38 MAPK and p-ERK1/2 protein levels， and promoted the interaction between Runx2 and CBF-β （P<0.01）. No significant change in Runx2 expression was observed. Compared to the blank group， the BVD-523 group showed significantly lower expression of p-p38 MAPK and p-ERK1/2 proteins （P<0.01）. Compared with the BVD-523 group， both low and high concentration Zuoguiwan-containing sera groups showed significantly higher p-p38 MAPK expression （P<0.01）， and the high concentration Zuoguiwan group also exhibited a significant increase in p-ERK1/2 expression （P<0.01）， while no statistical difference was found in the low-dose group. ConclusionZuoguiwan inhibits osteoclast activation by inducing phosphorylation of the key transcriptional regulator Runx2 in intra-osteoclast bone formation， and this process is closely associated with the activation of the p38 MAPK/ERK signaling pathway.

AIM: To prepare a novel functionalized upconversion nanomaterial UCNPs-PEG-Tf through an improved method and observe its specific imaging phenomenon to choroidal melanoma cells(OCM-1).METHODS: NaYF4:Yb/Er(Up-conversion nanoparticles, UCNPs)was Prepared and plasma was treated to carboxylate the surface; then amino polyethylene glycol and transferrin(Tf)were respectively loaded to prepare UCNPs-PEG-Tf. Characterized them accordingly, its biocompatibility was tested accordingly, and the specific fluorescence phenomenon of OCM-1 was detected by fluorescence spectrophotometer and inverted fluorescence microscopy.RESULTS: Characterization confirmed successful synthesis of UCNPs, UCNPs-PEG, and further loading of Tf to form UCNPs-PEG-Tf. UCNPs-PEG-Tf showed excellent biocompatibility and emitted significant green fluorescence. Under the same conditions, fluorescence intensity measurement and observations from the inverted fluorescence microscope both demonstrated its significant specificity in imaging to OCM-1 cells.CONCLUSION: The synthesized novel functionalized UCNPs-PEG-Tf nanocomposite showed good biocompatibility and achieve specific imaging to OCM-1 cells.

Objective:To explore a simple emergency management method for elderly patients with sigmoid volvulus.Methods:The clinical data of 22 elderly patients (>70 years) with sigmoid volvulus from January 2020 to March 2024 in the Affiliated Hospital of Xuzhou Medical University were retrospectively analyzed. All patients were treated with a self-made simple closed-loop enema decompression kit. The abdominal circumference, white blood cell count and C-reactive protein (CRP) before treatment and 12 h after tube placement were measured. The patients were followed for 3 months, and the recurrence was recorded. The key indexes on recurrence, including age, American Society of Anesthesiologists (ASA) classification and procedure time, were compared. Pearson correlation analysis was used to examine the relationship between age, procedure time, pain level and gas/stool output within 30 min after tube placement.Results:All 22 patients successfully underwent transanal tube decompression. The procedure time ranged from 1 to 15 min. The gas and stool output within 30 min after tube placement was 600 to 2 100 ml, The rectal tube was retained for 2 to 6 d. Compared with before treatment, the abdominal circumference, white blood cell count and CRP 12 h after tube placement were significantly lower: (85.9 ± 9.6) cm vs. (94.5 ± 10.2) cm, (9.2 ± 2.1) ×10 9/L vs. (11.4 ± 2.5) ×10 9/L and (27.8 ± 22.6) mg/L vs. (46.2 ± 38.9) mg/L, and there were statistical differences ( P<0.01). Four patients underwent elective surgery, while 18 were discharged smoothly after tube removal. No death occurred within 1 month after treatment. Five patients experienced recurrence 3 months after treatment, all were successfully retreated using the same method and discharged. There were no statistical differences in recurrence rates between aged ≥80 years and aged < 80 years patients, ASA class ≥ Ⅳ and ASA class Ⅲ patients, or procedure times ≤5 min and procedure times >5 min patients ( P>0.05). The gas/stool output within 30 min after tube placement was positively correlated with pain level (moderate/severe vs. mild) before tube placement ( r = 215.50, P = 0.015), but showed no significant correlation with age or procedure time ( P>0.05). Conclusions:The self-made simple closed-loop enema decompression kit provides a straightforward, economical and minimally invasive emergency treatment method for elderly patients with sigmoid volvulus. For patients at very high surgical risk, this kit can achieve decompression and volvulus reposition, even in cases of recurrence.

Objective:To investigate the clinical value of right-sided overlap and single-flap valvuloplasty (ROSF) in Da Vinci robotic proximal gastrectomy.Methods:The retrospective and descriptive study was conducted. The clinicopathological data of 12 patients who underwent ROSF in Da Vinci robotic proximal gastrectomy at The Affiliated Hospital of Xuzhou Medical University from September 2023 to May 2024 were collected. There were 7 males and 5 females, aged 62(range, 35?75)years. Observation indicators: (1) surgical conditions; (2) postoperative pathological results; (3) follow-up. Measurement data with normal distribution were represented as Mean± SD, and mea-surement data with skewed distribution were represented as M(range). Count data were expressed as absolute numbers. Results:(1) Surgical conditions. All patients successfully completed the surgery, without conversion to laparotomy. The operation time of 12 patients was (236±24)minutes. The time of single-layer fabrication and anastomosis was (105±28)minutes. The volume of intra-operative blood loss was (36±19)mL. Time to postoperative first fluid food intake was (3.5±1.1)days. The amylase levels in the abdominal drainage fluid on postoperative days 1, 2, and 3 were (321±121)U/L, (225±97)U/L, and (85±22)U/L, respectively. Time to postoperative drainage tube removal was (5.3±1.5)days. Duration of postoperative hospital stay was (7.6±1.1)days. All 12 patients had no anasto-motic related complications such as anastomotic leakage, anastomotic bleeding, anastomotic stenosis, and had no functional complications such as gastric emptying disorders or gastroesophageal reflux after surgery. (2) Postoperative pathological results. The distance of the proximal tumor margin of the 12 patients was (1.8±1.1)cm. The distance of distal margin was (5.7±2.1)cm. Number of lymph node dissected was 31.0(range, 22.0?45.0). Number of positive lymph node dissected was 3.4±2.4. Number of lymph node dissected from the pancreatic superior margin was 14.7±4.3. Results of postoperative pathological examination in the 12 patients showed 8 cases of Ⅰ stage, 3 cases of Ⅱ stage, 1 case of Ⅲ stage of the TNM staging. (3) Follow-up. All 12 patients were followed up for 6 (range, 3?24)months. During the follow-up period, all 12 patients had no local tumor recurrence or distant metastasis. All 12 patients had no complications such as anastomotic leakage, anastomotic bleeding, or anastomotic stenosis, and did not experience symptoms of gastroesophageal reflux such as heartburn or vomiting.Conclusion:The ROSF in Da Vinic robotic proximal gastrectomy is safe and feasible.

Objective To explore the mediating effect of negative emotion and rumination on the quality of discharge guidance and discharge readiness of patients after coronary artery bypass grafting.Methods A total of 288 patients who underwent coronary artery bypass surgery in the Second Affiliated Hospital of Nanchang University from February 2022 to February 2024 were selected as study objects.General data questionnaire,self-rating anxiety scale(SAS),self-rating depression scale(SDS),Chinese version of event related rumination inventory(CERRI),quality of discharge teaching scale(QDTS),readiness for hospital discharge scale(RHDS)were used to investigate,and Amos 24.0 structural equation model was used to analyze the chain mediation effect.Results A total of 288 questionnaires were sent out,and 280 valid questionnaires were recovered,with an effective recovery rate of 97.22％.The patients'SAS scores were(57.83±5.32)points,SDS scores were(63.31±4.97)points,CERRI total scores were(31.03±4.76)points,QDTS total scores were(116.78±9.22)points,and RHDS total scores were(79.30±7.21)points.The RHDS scores of patients who lived alone after discharge,paid at their own expense and had grade Ⅲ heart function were significantly lower than those of patients with nursing care,medical insurance payment and grade Ⅰ heart function after discharge(P<0.05).Anxiety was positively correlated with depression,intrusive rumination,but negatively correlated with purposive rumination,quality of discharge guidance and discharge readiness.Depression was positively correlated with intrusive rumination,but negatively correlated with purposive rumination,quality of discharge guidance and discharge readiness.Invasive rumination was negatively correlated with purposive rumination,quality of discharge guidance and discharge readiness.Purposive rumination was positively correlated with the quality of discharge guidance and discharge readiness.The quality of discharge guidance was positively correlated with discharge readiness(P<0.05).The chain-mediated model was well fitted,and the results showed that the quality of patients'discharge guidance could affect their discharge readiness,while negative emotions and rumination pathways also affected their discharge readiness(P<0.05).Conclusion The quality of discharge guidance can directly or indirectly affect the discharge readiness of patients after coronary artery bypass grafting through negative emotions and rumination,and there is a chain mediation effect.

The main pathological features of Parkinson disease(PD)include accumulation of α-synuclein and loss of dopaminergic neurons.PET imaging can be used to diagnose and monitor PD and explore its neural mechanisms through specific targeted imaging agents.The research progresses of PD related PET imaging agents were reviewed in this article.