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Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease caused by abnormal expression of glycosylphosphatidylinositol (GPI) on the cell membrane due to mutations in the phosphatidylinositol glycan class A(PIGA) gene. It is commonly characterized by intravascular hemolysis, repeated thrombosis, and bone marrow failure, as well as multiple systemic involvement symptoms such as renal dysfunction, pulmonary hypertension, swallowing difficulties, chest pain, abdominal pain, and erectile dysfunction. Due to the rarity of PNH and its strong heterogeneity in clinical manifestations, multidisciplinary collaboration is often required for diagnosis and treatment. Peking Union Medical College Hospital, relying on the rare disease diagnosis and treatment platform, has invited multidisciplinary clinical experts to form a unified opinion on the diagnosis and treatment of PNH, and formulated the Expert Consensus of Multidisciplinary Diagnosis and Treatment for Paroxysmal Nocturnal Hemoglobinuria (2024), with the hope of promoting the standardization of PNH diagnosis and treatment.

Resident training is a necessary path to cultivate excellent clinical doctors. Based on the Consensus on Core Competency Framework for Residency Education Among China Consortium of Elite Teaching Hospitals for Residency Education, Department of Neurology from Peking Union Medical College Hospital has established a training program for neurology residents with a graded evaluation system and a progressive clinical responsibility concept guided by core competency. Overcoming the teaching difficulties of neurology and considering the clinical characteristics of neurology, we have established a series of replicable and promotable neurology residency training curriculum through inheritance and innovation, which provides reference for the continuous improvement of China's neurology residency training program.

目的 在神经科住院医师中开展困难谈话技能培训课程，探究困难谈话技能培训的可行性，并初步探索本课程的效果。 方法 在北京协和医院神经科住院医师中开展2次困难谈话技能培训课程，每月1次，每次2 h。课程主题均为与神经科临床实践相关的具体临床情景，包括：①告知坏消息；②与“困难”患者或家属沟通；③讨论不施行心肺复苏等抢救措施；④与家属讨论重症患者的临终关怀。采用情景模拟教学方法，邀请标准化病人和住院医师在教师指导下完成案例情景模拟，并进行医患沟通伦理知识、技能策略的学习。采用匿名在线调查评估住院医师对本课程的满意度，课程前后采用困难谈话技能自我评估问卷分析住院医师11个维度技能的改善情况。课程结束1年后使用医患沟通技能评价量表（set the stage，elicit information，give information，understand the patient’s perspective，and end the encounter；SEGUE）对部分住院医师的沟通能力进行考核。 结果 共有14位住院医师参加了困难谈话技能培训课程，12位（85.7%）完成了相关调查评估问卷。完成问卷的住院医师中9位（75.0%）为女性，10位（83.3%）为低年级住院医师（第一年或第二年住院医师）。总体而言，住院医师对本课程反响积极，所有完成问卷的住院医师均对课程表示满意；参与的住院医师在困难谈话技能自我评估问卷涉及的绝大多数维度上均有不同程度改善；4位住院医师完成SEGUE评估，3位获得A级评分。 结论 在神经科住院医师中使用基于真实病例的特定临床情景模拟来开展困难谈话技能培训课程是可行的，住院医师对课程满意度高且课程对其有积极影响，住院医师的困难谈话技能在培训后得到一定提升。 Abstract: Objective A preliminary training course on difficult conversation skills was developed for neurology residents to explore the feasibility and effects of difficult conversation skills training. Methods Two difficult conversation skills training courses focusing on specific clinical scenarios of particular relevance to neurologic practice were conducted among the neurology residents in Peking Union Medical College Hospital, once a month for 2 hours each time. The topics were: giving bad news, communicating with “difficult” patients or family members, discussing not implementing cardiopulmonary resuscitation and other rescue measures, and discussing the hospice care of patients in severe or critical condition with their families. Standardized patients and residents were invited to complete simulation-based teaching and the learning of doctor-patient communication ethics knowledge, skill, and strategies under the guidance of teachers. An anonymous online survey was used to assess residents’ satisfaction with the course. The self-assessment questionnaire of difficult conversation skills was used to analyze the improvement of residents’ skills in 11 dimensions before and after the course. Set the stage, elicit information, give information, understand the patient’s perspective, and end the encounter (SEGUE) was used to assess the communication ability of partial residents after one year of the course. Results A total of 14 residents participated in the course, and 12 (85.7%) completed the questionnaire, of which 9 (75.0%) were female, and 10 (83.3%) were junior residents, including first- and second-year residents. Overall, the residents reacted positively to the training course and gained improvements in most aspects addressed in the questionnaire to different extents. All residents who completed the questionnaire were satisfied with the course, and 3 of the 4 residents who completed the SEGUE evaluation got a grade of A. Conclusions Difficult conversation skills training using specific clinical scenarios stimulation based on real neurologic cases was well accepted by neurology residents. The residents had a high degree of satisfaction with the course and the course had a positive influence on the participants. Their ability of difficult conversations has been improved to different degrees after the training.

This paper conducts a detailed study on the thermal transport and thermoelectric properties of Li 2 Rb(Cs)Bi and analyzes the optical phonon frequency shift caused by considering anharmonicity. We mainly focus on studying the microscopic mechanism of the difference in lattice thermal conductivity (κ L) of the two materials. By calculating the group velocity, scattering rate, scattering phase space and scattering sub-process, it is concluded that κ L is mainly dominated by the acoustic branch. Due to its small group velocity and large scattering rate, Li 2 CsBi has a low κ L , which is 0.60 W m − 1 K − 1 at 300 K. Research results show that n-type Li 2 CsBi has a higher ZT value of about 2.1 at T = 900 K, while p-type Li 2 RbBi has a higher ZT value of about 1.5 at the same temperature. These results provide an important theoretical basis for the application of Li 2 Rb(Cs)Bi in the field of thermoelectric conversion. [ABSTRACT FROM AUTHOR]

Abstract Chronic non-communicable diseases (CNCDs) pose a significant public health challenge. Addressing this issue, there has been a notable breakthrough in the prevention and mitigation of NCDs through the use of antioxidants and anti-inflammatory agents. In this study, we aim to explore the effectiveness of Eupatorium adenophora Spreng leaves (EASL) as an antioxidant and anti-inflammatory agent, and its potential applications. To construct a cellular model of oxidative damage and inflammation, Caco-2 cells were treated with tert-butyl hydroperoxide (t-BHP). The biocompatibility of EASL-AE with Caco-2 cells was assessed using the MTT assay, while compatibility was further verified by measuring LDH release and the protective effect against oxidative damage was also assessed using the MTT assay. Additionally, we measured intracellular oxidative stress indicators such as ROS and 8-OHdG, as well as inflammatory pathway signalling protein NFκB and inflammatory factors TNF-α and IL-1β using ELISA, to evaluate the antioxidant and anti-inflammatory capacity of EASL-AE. The scavenging capacity of EASL-AE against free radicals was determined through the DPPH Assay and ABTS Assay. Furthermore, we measured the total phenolic, total flavonoid, and total polysaccharide contents using common chemical methods. The chemical composition of EASL-AE was analyzed using the LC–MS/MS technique. Our findings demonstrate that EASL-AE is biocompatible with Caco-2 cells and non-toxic at experimental levels. Moreover, EASL-AE exhibits a significant protective effect on Caco-2 cells subjected to oxidative damage. The antioxidant effect of EASL-AE involves the scavenging of intracellular ROS, while its anti-inflammatory effect is achieved by down-regulation of the NFκB pathway. Which in turn reduces the release of inflammatory factors TNF-α and IL-1β. Through LC–MS/MS analysis, we identified 222 compounds in EASL-AE, among which gentianic acid, procaine and L-tyrosine were the compounds with high antioxidant capacity and may be the effective constituent for EASL-AE with antioxidant activity. These results suggest that EASL-AE is a natural and high-quality antioxidant and anti-inflammatory biomaterial that warrants further investigation. It holds great potential for applications in healthcare and other related fields.

This article reports a case of acute myelomonocytic leukemia (AML-M4, CBFB gene positive) with lower limb microthrombus formation after haploidentical hematopoietic stem cell transplantation, and summarizes the experience of diagnosis and treatment. The patient received achieved complete remission (CR) after routine chemotherapy, but relapsed 2 years later and underwent a haploidentical hematopoietic stem cell transplantation. During routine treatment after transplantation, the patient’s coagulation related indicators, including D-dimer (D-D), antithrombin Ⅲ (AT-Ⅲ), von Willebrand factor (vWF), fibrinogen degradation products (FDP) and fibrin monomer (FM), were dynamically observed. The patient experienced jaundice and elevated transaminases around 100 days after transplantation, and underwent plasma exchange treatment three times through the right lower limb femoral vein catheterization. After removing the venous catheter for one week, there was left ankle concavea pitting edema of the left ankle. Vascular ultrasound examination indicated the formation of microthrombus in the superficial venous plexus of the gastrocnemius muscle in the left lower limb. D-D, vWF, FDP, FM and other indicators were increased, while AT-Ⅲ levels was decreased. After 2 weeks of oral administration of 5 mg/d berritol for antithrombotic treatment, the above coagulation indicators gradually returned to normal, and the left lower limb intermuscular venous plexus thrombosis and limb swelling disappeared. Therefore, dynamic monitoring of coagulation indicators, such as D-D, AT-Ⅲ, vWF, FDP, and FM may serve as diagnostic indicators for predict transplant related microthrombus and hypercoagulable status, providing an experimental basis for effective anticoagulant treatment in clinical practice.