[摘 要] 目的：探讨复发/转移性鼻咽癌（NPC）接受含PD-1单抗免疫治疗的临床特征和预后影响因素。方法：回顾性分析2019年3月至2024年7月期间南部战区总医院确诊的95例NPC患者的临床资料和外周血生化及免疫学指标。预后分析采用Kaplan-Meier曲线，组间比较使用Log-rank检验，采用Cox比例风险模型进行单因素和多因素分析。结果：95例患者中男性81例，女性14例，中位年龄49.72岁（16~74岁），Ⅳ期91例（95.79%），所有患者均采用免疫治疗，联合或不联合化疗方案治疗，中位无进展生存期（mPFS）为10.5个月，客观缓解率（ORR）70.53%，疾病控制率（DCR）89.47%，接受含铂治疗方案患者PFS相对更长，且差异有统计学意义。紫杉醇 + 顺铂 + 氟尿嘧啶（TPF）对比吉西他滨 + 顺铂（GP）和紫杉醇 + 顺铂（TP）显示出更长的PFS，但差异无统计学意义。不同PD-1单抗治疗组间的PFS未显示出有统计学意义的差异。单因素及多因素Cox回归分析结果显示，肿瘤复发状态、初始血浆EBV感染状态、治疗周期数、基线外周血SII是复发/转移性NPC患者接受PD-1抑制剂治疗疗效预测的独立相关因素（均P < 0.05），并且非复发患者、初始血浆EBV DNA阳性、接受 ≥ 4治疗周期、基线外周血SII < 772.81的患者接受PD-1抑制剂治疗预后相对更好。结论：在接受PD-1抑制剂治疗的复发/转移性NPC患者中，非复发患者、初始血浆EBV DNA阳性、≥ 4治疗周期且外周血SII < 772.81者PFS相对更长，可早期识别免疫治疗效果不佳患者并精准干预。

Stroke is the leading cause of death and disability among adults in China， and its common complications include digestive system abnormalities， cognitive impairment， depression， stroke-associated pneumonia， and hemiplegia. The combination of traditional Chinese and Western medicine has great potential in treating post-stroke complications. Xinglou Chengqitang （XLCQT） is a representative prescription of alleviating the disease in the upper part by treating the lower part. It has definite therapeutic effect and high safety. Clinically， XLCQT is often used to treat stroke and its complications. However， the quantity and quality of clinical trials of XLCQT in treating post-stroke complications need to be improved. Additionally， since the basic research is weak， the material basis and multi-target mechanism for the efficacy of this prescription are unknown. This article reviews XLCQT in terms of the pharmacodynamic basis， medicinal properties， safety evaluation， and progress in clinical research and mechanisms in treating post-stroke complications. This article summarizes 22 key active ingredients of XLCQT in treating acute stroke complicated with syndrome of phlegm heat and fu-organ excess. Among these key active ingredients， resveratrol， kaempferol， luteolin， chrysoeriol， apigenin， （+）-catechin， and adenosine have good pharmacokinetic properties and high bioavailability. The mechanisms of XLCQT in treating post-stroke complications are complex， including inflammatory response， brain-gut axis， hypothalamic-pituitary-adrenal （HPA） axis， intestinal flora， neurotrophic factors， autophagy， oxidative stress， and free radical damage. This review helps to deeply understand the pharmacodynamic basis and mechanisms of XLCQT in treating post-stroke complications and provides a theoretical basis for the clinical application of XLCQT against post-stroke complications and the development of drugs.

OBJECTIVE: To explore the clinical and genetic features of a child with Pontocerebellar hypoplasia type 2B (PCH2B) due to compound heterozygous variants of the TSEN2 gene. METHODS: A PCH2B patient presented at Department of Pediatric Neurology, Xiangya Hospital of Central South University in June 2023 was selected as the study subject. Clinical data of the patient were retrospectively analyzed. The patient and her parents were subjected to whole exome sequencing and bioinformatic analysis. Pathogenicity of the candidate variants were classified based on the guidelines from the American College of Medical Genetics and Genomics (ACMG). A literature review was also conducted by searching the China National Knowledge Infrastructure (CNKI), Wanfang Data, and PubMed databases from their establishment to May 2025 using keywords "TSEN2 gene" "PCH2B" and "Pontocerebellar Hypoplasia 2B" to summarize the clinical and genotypic features of patients with PCH2B due to variants of the TSEN2 gene. This study was approved by the Medical Ethics Committee of the Hospital (No.: #202310892). RESULTS: The patient, a 6-year-5-month-old girl, had exhibited severe global developmental delay, developmental regression, autism spectrum disorder, myoclonus of eyelids, feeding difficulty, irritability, progressive microcephaly, esotropia, and hypotonia. MRI showed reduced volume of bilateral cerebellar hemispheres and vermis. Genetic testing revealed that she has harbored compound heterozygous variants of the TSEN2 gene (NM_025265.4), namely c.1054A>T (p.Lys352*) and c.899G>T (p.Ser300Ile), which were inherited from her father and mother, respectively. Both variants were classified as likely pathogenic based on the ACMG guidelines and were previously unreported. Literature review has identified six PCH2B patients with missense, nonsense, frameshift, and splice site variants of the TSEN2 gene. Their main clinical manifestations included global developmental delay, progressive microcephaly, feeding difficulties, irritability, and vermis hypoplasia. Cranial MRI and genetic testing are crucial for definite diagnosis. CONCLUSION The c.1054A>T (p.Lys352*) and c.899G>T (p.Ser300Ile) compound heterozygous variants of the TSEN2 gene probably underlay the pathogenesis in this patient. Above findings has expanded the genotypic and phenotypic spectra of TSEN2-related PCH2B, and offered guidance for genetic counseling for this family.
Child ; Female ; Humans ; Cerebellar Diseases/genetics* ; Exome Sequencing ; Heterozygote ; Mutation

The quality and teaching level of clinical teaching staff are important factors affecting the training quality of newly recruited nurses, and strengthening the construction of the teaching staff team is the key to ensuring the training quality. This article reviews the relevant contents of the clinical teaching staff training project for newly recruited nurses at home and abroad, including training contents, training duration, training forms, achieved results, and deficiencies. Countermeasures are proposed for the existing problems, aiming to provide referential ideas for constructing a unified teaching staff training program for newly recruited nurses in China.

Objective To evaluate the effectiveness of a blended teaching model integrating the HBOPPPS framework(Hybrid Bridge-in,Objectives,Pre-assessment,Participatory learning,Post-assessment,Summary)and the"Cloud-based PAD Class"(Presentation-Assimilation-Discussion)in online clinical clerkship teaching for palliative oncology.Methods A self-con-trolled trial was conducted in October 2023,involving 41 undergraduate students majoring in Food Hygiene and Nutrition(Grade 2021)from Kunming Medical University.Phase 1 implemented conventional online teaching(control group),while Phase 2 adopted the"HBOPPPS+Cloud-based PAD Class"blended approach(experimental group).Teaching effectiveness was assessed via post-class examination scores,classroom participation rates,and satisfaction surveys.Results The experimental group dem-onstrated significantly higher outcomes than the control group:examination scores(86.34±4.19 vs.80.02±3.63,P＜0.05),classroom participation(51.2％vs.12.2％,P＜0.05),and teaching satisfaction(95.1％vs.80.5％,P＜0.05).Conclu-sionThe"HBOPPPS+Cloud-based PAD Class"blended teaching model effectively enhances online clinical clerkship outcomes in palliative oncology,significantly improving students' learning initiative,engagement,and classroom participation.

The incidence of digestive system diseases is high. So digestive system pathology is widely concerned. In the past 10 years, Chinese pathologists insist on hard work and have made significant progress. This article reviews the research and practice progress in digestive system tumors and non-tumors in the past 10 years, including the establishment of diagnostic criteria for gastrointestinal tract, hepatobiliary and pancreatic diseases, the establishment and classification of new diseases with special morphology, the classification and standard update of gastrointestinal and pancreatic neuroendocrine tumors, the progress of molecular pathology, and non-tumor lesions of the digestive system. The future development trend of digestive system pathology is also prospected.

Objective:To explore the practical experience in perineal protection and assessment by midwives in general hospitals, so as to provide reference for further reducing the episiotomy rate for non-medical indications.Methods:From December 2021 to January 2022, applying a phenomenological research methodology, 14 midwives from four general hospitals in Anhui Province were selected through purposive sampling combined with maximum difference sampling for semi-structured interviews. Colaizzi 7-step analysis method was used to summarize, analyze and refine themes.Results:Three themes and 10 sub-themes were extracted, namely the motivation for perineal protection (policy orientation, reduction of childbirth injuries, maternal needs), decision-making factors for perineal protection (maternal and infant safety, maternal perineal condition, work experience, work environment, maternal cooperation), and techniques for perineal protection (patience, controlling the speed of fetal head delivery) .Conclusions:Midwives' perineal protection and assessment practices are influenced by multiple factors. Hospital administrators are advised to enhance midwife training, establish system processes to reduce perineal injuries, unify perineal assessment standards, and rationally control episiotomy rates while fully prioritizing the maternal and infant safety.

Sj?gren's syndrome(SS)is one of the common rheumatic diseases in clinical practice.Modern medicine commonly uses drugs such as artificial tears,saliva,glucocorticoids,immunosuppressants,and biologics to control the condition.Clinical practice has shown that in addition to modern medical basic treatment,the use of traditional Chinese medicine(TCM)can help improve the clinical efficacy of SS.According to the symptoms and signs of Sj?gren's syndrome in TCM,it is classified as"dryness and obstruction",and the core pathogenesis of the disease is spleen deficiency and deficiency of body fluids.Subsequently,toxic and pathogenic factors gather,leading to the decline of internal organs.The initial causes are spleen damage,unstable barrier,and invasion of pathogenic factors.The core link is spleen dysfunction,insufficient body fluid,and dryness arising from it.Spleen deficiency generates evil,obstruction of qi,and lack of body fluids are the root causes of illness.The main treatment method is the"spleen strengthening method",which treats spleen deficiency,dampness and stagnation,and the body fluid is not distributed.The treatment focuses on strengthening the spleen and qi,supplementing the lungs and generating fluids.Spleen deficiency leads to loss of vitality,blood stasis obstructs blood vessels,and the treatment is to strengthen the spleen,soothe the liver,remove blood stasis,and unblock the orifices.The spleen yang is not vigorous,and qi transformation is impaired.The treatment is to invigorate the spleen and warm the stomach,promote yang circulation,and promote diuresis.

Objective To investigate the efficacy of nimodipine combined with donepezil in the treatment of CSVD patients with mild cognitive impairment.Methods A total of 130 CSVD pa-tients admitted to our department from October 2020 to October 2023 were recruited and ran-domly divided into monotherapy group(nimodipine,65 cases)and combined group(nimodipine plus donepezil,65 cases).The clinical efficacy after 3 months of treatment was recorded.The blood-brain barrier function and brain function indicators[serum neuron-specific enolase(NSE),vascular endothelial growth factor(VEGF),central nervous system specific protein(S100β)]were compared between the two groups.Results The clinical efficacy was significantly higher in the combined group than the monotherapy group(92.3％vs 78.5％,P＜0.05).The cerebrospinal fluid albumin and blood-brain barrier index were obviously increased in the two groups after treatment(P＜0.05),and the two indicators were notably lower in the combined group than the monothera-py group(P＜0.01).After treatment,the level of VEGF was remarkably elevated while those of NSE and S100β were marvelously reduced in the two groups when compared with the levels be-fore treatment(P＜0.05).The combined group obtained significantly higher VEGF level but low-er NSE and S100β levels than the monotherapy group after treatment(P＜0.01).Conclusion For CSVD patients with mild cognitive impairment,the combination of nimodipine and donepezil can effectively improve the cognitive impairment,and promote the brain function recovery.

Neuronal ceroid lipofuscinoses (NCLs) are a group of monogenic lysosomal storage diseases characterized by progressive cognitive and motor deterioration, visual impairment, epileptic seizures, and early death. The therapeutic landscape for NCLs encompasses a range of approaches, including enzyme replacement therapy, gene therapy, stem cell therapy, immunotherapy and small molecule pharmacotherapy. A recombinant human tripeptidyl peptidase 1 is the only approved enzyme replacement therapy for neuronal ceroid lipofuscinosis type 2 disease administered via intracerebroventricular infusion. Other potential treatments for the NCLs are at preclinical stages and under clinical trials. This review provides an updated progress in pre-clinical and clinical study of potential therapeutics for the NCLs.