血友病是一种由于X染色体上凝血因子基因突变所致的遗传性出血性疾病,目前主要的治疗方法是凝血因子替代疗法。但长期频繁的注射用药往往导致患者依从性差,容易产生抑制性抗体,从而影响治疗效果。虽然现在延长半衰期的新型凝血因子药物、人源化双特异性抗体以及抗组织因子途径抑制剂单克隆抗体等用于疾病治疗,在给药方式和作用持续时间上已有很大进步,但它们仍无法治愈血友病。因此,以疾病根治为重要目标的基因治疗被设计出来,近年来受到了广泛的关注。该文介绍了血友病基因治疗的原理、基因治疗载体的选择、基因治疗预处理方案,总结了现阶段基因治疗临床应用的安全性和有效性;最后讨论基因治疗目前存在的问题以及未来发展方向。

Hemophilia is a genetic bleeding disorder resulting from mutations in coagulation factor genes on the X chromosome．The mainstay of current treatment is coagulation factor replacement therapy．However,frequent and long-term injections often lead to poor patient compliance,easy inhibitor development,and compromised therapeutic efficacy．Despite advancements in delivery methods and prolonged action of novel agents such as extended half-life coagulation factor concentrates,humanized bispecific antibodies,and anti-tissue factor pathway inhibitor monoclonal antibodies,these approaches still fall short of curing hemophilia．Consequently,gene therapy,aiming for disease eradication,has garnered significant attention in recent years．This review delves into the principles of gene therapy,the selection of gene therapy vectors,and gene therapy preconditioning regimens．It summarizes the safety and efficacy of gene therapy in current clinical applications and discusses challenges and future directions in this field．

目的 探讨间歇经口至食管管饲法与自创吞咽功能训练联合应用于脑卒中吞咽障碍患者的效果。方法 选取河南大学淮河医院2022年1月—2023年12月收治的50例脑卒中吞咽障碍患者,应用随机数字表法将其分为两组,每组各25例。对照组采取持续留置鼻饲的同时应用自创吞咽功能训练进行训练,观察组采取间歇经口至食管管饲的同时应用自创吞咽功能训练进行训练。对比两组吞咽障碍改善情况、干预前后营养状况指标及生活质量变化,比较两组并发症发生率。结果 观察组总有效率高于对照组（P<0.05）。干预后两组血清总蛋白、白蛋白和血红蛋白水平和体质量指数均升高,观察组高于对照组（P<0.05）。干预后两组生活质量综合评定量表评分升高,观察组高于对照组（P<0.05）。观察组并发症发生率低于对照组（P<0.05）。结论 对脑卒中吞咽功能障碍患者采取间歇经口至食管管饲法与自创吞咽功能训练,能够更有效改善患者吞咽功能,提升其生活质量,且可降低留置饲管营养支持过程中的并发症发生率。

Objective To explore the application effect of intermittent oral to esophageal feeding combined with self created swallowing function training exercises in stroke patients with swallowing disorders．Methods Fifty stroke patients with swallowing disorders admitted to our hospital from January 2022 to December 2023 were selected and divided into two groups by random number table method,both with 25 cases．Control group patients received continuous nasogastric feeding while using solf created swallowing function training exercises for training,while observation group patients received intermittent oral to oesophageal tube feeding and using solf created swallowing function training exercises for training．The improvement of swallowing disorders,changes in nutritional status indicators and quality of life before and after the intervention were compared,and also the frequency of complications．Results The observation group was generally more effective than the control group（P<0.05）．After intervention,the serum total protein,albumin hemoglobin,and BMI levels of both groups of patients increased,with the observation group being higher than the control group（P<0.05）．After the intervention,GQOLI-74 score increased in both groups,and the observation group was higher than the control group（P<0.05）．The complication rate was lower in the observation group（P<0.05）．Conclusions The intervention method of intermittent oral to esophageal tube feeding combined with self created swallowing function training exercises can further improve the swallowing function of stroke patients with swallowing dysfunction,improve their quality of life,and reduce the occurrence of complications during the nutritional support process of feeding tubes．

目的 观察营养指导联合预防护理在小儿癫痫意外预防中作用。方法 抽取焦作市妇幼保健院2021年1月—2023年1月收治的116例小儿癫痫患儿,按照护理方法分为两组,每组各58例。对照组行常规护理,联合组患儿在常规护理基础上给予营养指导联合预防护理干预,对比两组家属满意度、患儿依从率、蒙特利尔认知评估量表（MoCA）评分。结果 经过护理干预,联合组家属满意率98.28%,依从率96.55%,比对照组87.93%、82.76%高（P<0.05）;住院至出院居家三个月内,相较于对照组,联合组患儿外伤发生次数、发病持续时间、癫痫发作次数较少（P<0.05）;护理后,联合组MoCA评分高于对照组（P<0.05）。结论 在小儿癫痫意外预防中联合应用营养指导和预防护理可改善患儿遵医行为,优化小儿癫痫意外预防效果,减少患儿外伤、癫痫发生次数,缩短患儿发病持续时间,提升患儿生活质量和家属满意度。

Objective To observe the effect of nutritional guidance combined with preventive nursing on the prevention of epilepsy accidents in children．Methods A total of 116 cases of pediatric epilepsy admitted in Jiaozuo Maternal and Child Health Care Hospital from January 2021 to January 2023 were selected．They were divided into two groups according to the nursing method,with 58 cases in each group．The control group was given usual care,while the combined group was given nutritional guidance combined preventive nursing intervention．The satisfaction,the compliance rate and MoCA score were compared between two groups．Results After nursing intervention,the satisfaction rate of the combined group was 98.28%,and the compliance rate of the combined group was 96.55%,which were higher than 87.93% and 82.76% in the control group（P<0.05）．Within three months from hospitalization to discharge,compared with the control group,number of injuries,duration and frequency of seizures were less in the combined group（P<0.05）．After nursing,the MoCA score of the combined group was higher than that of the control group（P<0.05）．Conclusions The combined application of nutritional guidance and preventive nursing in the prevention of pediatric epileptic accidents can improve the compliance behavior of children,optimize the prevention effect of pediatric epileptic accidents,reduce the incidence of trauma and epilepsy in children,shorten the duration of illness,and improve the quality of life of children and family satisfaction．

目的 调查与探讨高龄髋部骨折患者术后谵妄（POD）的发生因素,并提出相关处理对策。方法 选取2019年8月—2022年12月择在南阳市中医院独山院区进行手术治疗的高龄髋部骨折患者82例为研究对象,所有患者在术前1 d进行机械痛阈评定,在术后7 d判定患者的POD发生情况,进行POD与术前痛阈水平的相关性分析,并提出相关的处理对策。结果 术后7 d,82例患者中发生POD 12例（谵妄组）,占比14.6%,未发生POD 70例（非谵妄组）,占比85.37%。谵妄组的性别、体质指数、骨折类型、骨折至手术时间与非谵妄组对比差异无统计学意义（P>0.05）,谵妄组的年龄、术前血红蛋白水平、术前白蛋白水平与非谵妄组对比差异有统计学意义（P<0.05）。谵妄组的术前1 d的痛阈水平低于非谵妄组（P<0.05）。Spearman分析显示,POD与术前痛阈、年龄、术前血红蛋白、术前白蛋白均存在相关性（P<0.05）。Logistic回归分析显示,术前痛阈、年龄、术前血红蛋白、术前白蛋白等都为导致POD发生的影响因素（P<0.05）,要积极加强预防性护理干预。结论 高龄髋部骨折患者POD的发生率较高,患者的术前痛阈、年龄、术前血红蛋白、术前白蛋白等均为导致POD发生的影响因素,要积极加强预防性护理干预。

Objective To investigate and explore the factors leading to postoperative delirium（POD）in elderly patients with hip fractures,and to propose relevant handling measures．Methods From August 2019 to December 2022,82 cases of elderly patients with hip fractures who underwent surgical treatment in Nanyang Hospital of Traditional Chinese Medicine Dushan District were selected as the research subjects．All patients underwent mechanical pain threshold assessment 1 day before surgery,and their postoperative delirium were determined 7 days after surgery,followed by correlation analysis,and relevant handling measures were proposed．Results Seven days after surgery,there were 12 patients（delirium group）of POD,accounted for 14.6%,and 70 patients（non delirium group）without POD,accounted or 85.37%．There was no significant difference in genders,body mass index,fracture types and fracture to surgery time compared between the delirium group and the non delirium group（P>0.05）． However,there were significant differences in ages,preoperative hemoglobin levels and preoperative albumin levels compared between the delirium group and the non delirium group（P<0.05）．The pain threshold level of the delirium group on the first day before surgery was significantly lower than that of the non delirium group（P<0.05）．Spearman analysis showed that POD was associated with preoperative pain threshold,ages,preoperative hemoglobin and preoperative albumin levels（P<0.05）．Logistic regression analysis showed that the preoperative pain threshold level,ages,preoperative hemoglobin and preoperative albumin levels were all independent risk factors for the development of POD（P<0.05）,preventive nursing intervention should be actively strengthened．Conclusions The incidence of POD is high in elderly patients with hip fractures．Preoperative pain threshold level,age,preoperative hemoglobin and preoperative albumin levels are all factors that contribute to the occurrence of POD．It is necessary to actively strengthen preventive nursing interventions．

目的 分析恩格列净对2型心肾综合征（CRS）患者的治疗效果。方法 研究于2021年10月—2023年10月进行,随机抽取88例2型CRS患者,经计算机程序随机分成对照组（44例,给予常规治疗）、实验组（44例,在常规治疗的同时加用恩格列净）,通过对比两组患者心、肾功能指标来评估疗效,并记录两组患者治疗过程中发生的不良反应。结果 治疗后,实验组LAD、LVED低于对照组,而LVEF高于对照组;实验组血清肌酐、尿酸等肾功能指标水平均低于对照组（均P<0.05）。两组不良反应发生率比较差异无统计学意义（P>0.05）。结论 对于2型CRS患者,恩格列净可促进其心、肾功能的改善,且无过多不良反应,安全性理想,治疗效果可观,值得推广。

Objective To analyze the therapeutic effect of empagliflozin on patients with type 2 cardiorenal syndrome（CRS）．Methods The study was conducted from October 2021 to October 2023,and 88 patients with type 2 CRS were randomly selected and divided into control group（44 cases,receiving conventional treatment）and experimental group（44 cases,receiving conventional treatment combined with englaglitzin）by computer program．The efficacy was evaluated by comparing cardiac and renal function indexes and adverse reactions．Results After treatment,the LAD and LVED of the experimental group were lower than those of the control group,while LVEF was higher than that of the control group．The levels of serum creatinine,uric acid and other renal function indexes in the experimental group were lower than those in the control group after treatment（P<0.05）．There was no significant difference in the incidence of adverse reactions between the two groups（P>0.05）．Conclusions For patients with type 2 CRS,empagliflozin can promote the improvement of cardiac and renal function without many side effects,with ideal safety and considerable therapeutic effect,which is worthy of promotion．

目的 探讨产科监护室妊娠期糖尿病产妇泌乳启动延迟及影响因素,以期作为预防泌乳启动延迟的参考依据。方法 选择2021年1月—2023年5月期间医院产科监护室接收的妊娠期糖尿病产妇80例为研究对象,采用单因素和多因素分析产妇的年龄、体质指数、产次、定期复查血糖、妊娠期高血压、分娩方式、开奶时间、新生儿体质量、产后焦虑、产后抑郁、产后疲乏、吸吮次数等对泌乳启动延迟的影响。结果 妊娠期糖尿病产妇泌乳启动延迟33例,发生率41.25%;泌乳启动延迟产妇的年龄≥35岁者占51.52%、未定期复查血糖者占39.39%、合并妊娠期高血压者占54.55%、开奶时间≥6 h者占57.58%、产后焦虑者占30.0%、产后抑郁者占36.36%、产后疲乏者占36.36%、吸吮次数<6次者占60.61%,与非泌乳启动延迟产妇比较差异均有统计学意义（P<0.05）。两组孕前体质指数、产次、分娩方式、新生儿出生体质量比较差异无统计学意义（P>0.05）。年龄≥35岁、未定期复查血糖、合并妊娠期高血压、开奶时间≥6 h、产后焦虑、产后抑郁、产后疲乏、吸吮次数<6次为妊娠期糖尿病产妇泌乳启动延迟的危险因素（P<0.05）。结论 产科监护室妊娠期糖尿病产妇泌乳启动延迟发生率较高,主要受到年龄、未定期复查血糖、合并妊娠期高血压、开奶时间、产后心理状态、吸吮次数等因素影响,应重视健康教育和早期辅助干预,降低泌乳启动延迟发生率。

Objective To explore the delayed lactation initiation and its influencing factors of pregnant women with diabetes in the obstetric care unit,so as to provide a reference for preventing delayed lactation initiation．Methods A total of 80 pregnant women with diabetes who were received by the hospital obstetric care unit from January 2021 to May 2023 were selected as the research objects．The age,body mass index,parity,regular blood glucose recheck,pregnancy hypertension,delivery mode,starting time,neonatal weight,postpartum anxiety,postpartum depression,postpartum fatigue,sucking times and other factors that led to the delay of lactation initiation were analyzed by single factor and multi factor analysis．Results The onset of lactation was delayed in 33 pregnant women with diabetes,with an incidence of 41.25%．The age of postpartum women with delayed lactation initiation over 35 years old accounted for 51.52%,blood sugar was not regularly rechecked accounted for 39.39%,pregnancy induced hypertension accounted for 54.55%,lactation time over 6 hours accounted for 57.58%,postpartum anxiety accounted for 30.0%,postpartum depression accounted for 36.36%,postpartum fatigue accounted for 36.36%,and sucking frequency <6 times accounted for 60.61%,which were higher than that of non-delayed lactation initiation women（P<0.05）．There was no statistically significant difference between the two groups in terms of pre pre-pregnancy body mass index,parity,delivery method and newborn birth weight（P>0.05）．The risk factors of delayed lactation initiation in pregnant women with diabetes were age ≥ 35 years,no regular blood glucose review,hypertension during pregnancy,≥ 6 h of first milk expression time,postpartum anxiety,postpartum depression,postpartum fatigue and sucking times<6（P<0.05）．Conclusions The incidence of delayed lactation initiation in pregnant women with diabetes in the obstetric care unit is high,which is mainly affected by age,pregnancy induced hypertension,time of starting breast feeding,postpartum psychological state and sucking times．Health education and early auxiliary intervention should be emphasized to reduce the incidence of delayed lactation initiation．

本文探讨临床药师对口服靶向药物的非小细胞肺癌患者开展药学服务的要点,以案例为依据,通过查阅药品说明书、指南及文献等,分析药学服务的内容和方向。临床药师在安全性评估、剂量调整、个体化治疗方案选择、用药教育和健康宣教等方面为患者和临床医生提供专业、全面的药学服务。临床药师通过全程参与患者的治疗过程,指导患者正确用药、优化治疗方案,利用专业优势解决临床实际问题,提升药学服务质量的同时体现了药师的职业价值。

To explore the key points of pharmaceutical care for non-small cell lung cancer patients with oral targeted drugs．Based on clinical cases,the content and direction of pharmaceutical care were analyzed with drug instructions,guidelines and literature．Clinical pharmacists provided professional and comprehensive pharmaceutical services for patients and clinicians in safety assessment,dose adjustment,individualized treatment plan selection,medication education and health education．Clinical pharmacists participate in the whole treatment process,guide patients to use drugs correctly,optimize treatment plans,use professional advantages to solve clinical practical problems,improve the quality of pharmaceutical care and reflect the professional value of pharmacists．

目的 探讨综合呼吸康复训练对老年慢性阻塞性肺疾病急性期（AECOPD）患者的临床症状、生活质量、活动能力、肺功能、肺康复依从性的影响。方法 纳入70 例AECOPD 患者,随机数字分为对照组（n=35）和干预组（n=35）,对照组在常规治疗基础上予缩唇腹式呼吸功能锻炼,干预组在常规治疗基础上进行综合呼吸康复训练,干预前后采用慢性阻塞性肺疾病评估测试（CAT）调查问卷、改良版英国医学研究委员会呼吸问卷（mMRC）、肺功能第一秒用力呼气容积（FEV1）预计值进行评估。结果 对照组呼吸康复训练前后CAT评分、mMRC量表呼吸困难评级比较差异均有统计学意义（t=16.781,t=8.103,均P<0.001）;干预组呼吸康复训练前后CAT评分、mMRC呼吸困难评级比较差异也均有统计学意义（t=24.035,t=15.938,均P<0.001）;干预组患者的CAT评分、mMRC量表呼吸困难评级较对照组下降明显。结论 综合呼吸康复训练能有效改善 AECOPD 患者临床症状、增强患者活动能力、提高生活质量、提高肺康复依从性。

Objective To investigate the effects of comprehensive respiratory rehabilitation training on clinical symptoms,quality of life,activity ability,lung function and compliance of elderly patients with acute exacerbation of obstructive pulmonary disease（AECOPD）．Methods Seventy patients with AECOPD were included and randomly divided into control group（n=35）and intervention group（n=35）．The control group was given routine treatment and respiratory muscle function exercise．The intervention group was given routine treatment and comprehensive respiratory rehabilitation training．COPD Assessment Test（CAT）,modified Medical Research Council Scale（mMRC）and FEV1 predicted value of lung function were used to evaluate before and after intervention．Results In the control group,differences of CAT score and mMRC score were significant（t=16.781,t=8.103,P<0.001）before and after respiratory rehabilitation training．In the intervention group,the CAT score before and after respiratory rehabilitation training showed a 0.01 level of significance（t=24.035,P<0.001）,and the mMRC score before and after training showed a 0.01 level of significance（t=15.938,P<0.001）．There were significant differences between control group and intervention group（P<0.01）．Compared with the control group,CAT score and mMRC score were significantly lower in the intervention group．Conclusions Comprehensive respiratory rehabilitation training can effectively improve the clinical symptoms of AECOPD patients,enhance the activity ability of patients,improve the quality of life,and improve the compliance of patients to perform pulmonary rehabilitation．

肝癌患者因食欲降低、进食减少导致不同程度的营养不良,引发厌食症,甚至恶病质,严重影响患者生存质量。食欲评估是降低营养风险的基础,医护人员可通过食欲症状的评估调整患者饮食及营养指导措施,以改善患者营养状态,提高生存质量。该文对食欲的评估工具、方法及干预在肝癌营养不良患者中的应用现状进行阐述,为肝癌患者的食欲干预提供科学依据。

Hepatocellular carcinoma patients suffer from different degrees of malnutrition due to decreased appetite and eating,which triggers anorexia and even cachexia,seriously affecting the quality of patient survival．Appetite assessment is the basis for reducing nutritional risk,and healthcare professionals can adjust patients’ diet and nutritional guidance measures through the assessment of appetite symptoms in order to improve patients’ nutritional status and survival quality．In this paper,the current status of the assessment tools,methods and interventions of appetite in malnutrition patients with hepatocellular carcinoma are described to provide a scientific basis for appetite interventions．

价值医疗下公立医院需转变运营管理模式,建设专门运营管理部门对满足外部政策要求和改善内部运营管理十分必要。通过梳理公立医院开展运营管理的主要任务,确定公立医院专门运营管理部门的职责,设计与承担职责相适应的组织架构,保证专门运营管理部门的职能充分发挥。

Public hospitals need to change the operation management mode under value-based healthcare．It is necessary to build a special operation management department to meet the requirements of external policies and improve internal operation management．By sorting out the operation and management tasks of public hospitals,the responsibilities of the operation management department are determined,and the organizational structure suitable for the responsibilities is designed,so that the construction of specialized operation management department can be effectively implemented．