MDS

MDS
  • 文章类型: Journal Article
    健康的饮食可以防止超重和高血压。我们调查了儿童早期健康饮食与体重指数(BMI)和血压(BP)的关系。在GECKO出生队列中,高度,体重,在5岁和10岁时测量血压。3年时使用三个饮食评分进行饮食评估:停止高血压的饮食方法(DASH),地中海饮食评分(MDS),和生命线饮食评分(LLDS)。线性和逻辑回归模型评估了饮食评分与BMI和BP的关联。包括1077名儿童,10.8%的人在5年时超重或肥胖。这个数字在10年内为16.5%。此外,34.5%的人在5年时血压升高。这个数字在10年内为23.9%。更高的DASH,MDS,LLDS,这表明饮食更健康,均与10岁时较低的BMIz评分相关.较高的DASH与10年时较低的超重风险有关。在5年或10年时,饮食评分均与BP或BP升高无关。此外,在超重的子集中,饮食与血压无关.儿童早期的健康饮食与儿童超重较少有关,但在10岁时血压没有降低。
    A healthy diet prevents overweight problems and hypertension. We investigated the associations of a healthy diet with the body mass index (BMI) and blood pressure (BP) in early childhood. In the GECKO birth cohort, height, weight, and BP were measured at 5 and 10 years of age. Diet was evaluated at 3 years using three diet scores: the Dietary Approaches to Stop Hypertension (DASH), the Mediterranean Diet Score (MDS), and the Lifelines Diet Score (LLDS). Linear and logistic regression models assessed the associations of diet scores with the BMI and BP. Of the 1077 children included, 10.8% were overweight or obese at 5 years. That number was 16.5% at 10 years. In addition, 34.5% had elevated BP at 5 years. That number was 23.9% at 10 years. Higher DASH, MDS, and LLDS, which indicate healthier diets, were all associated with lower BMI z-scores at 10 years of age. Higher DASH is related to lower overweight risk at 10 years. None of the diet scores were associated with BP or elevated BP at either 5 or 10 years. Also, in an overweight subset, diet was not related to BP. A healthy diet in early childhood is related to children being less overweight but not having lower BP at 10 years of age.
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  • 文章类型: Journal Article
    复发和方案相关的毒性仍然是实现长期生存的主要挑战。特别是在接受异基因造血干细胞移植(allo-HSCT)的高危骨髓增生异常综合征(MDS)或急性髓细胞白血病(AML)的老年患者中.以前的研究已经证明了基于曲硫丹的调理的可行性,注意到接受HLA匹配的allo-HSCT的患者的植入稳定和低的非复发死亡率(NRM)。然而,在HLA-单倍体相合移植(HaploT)设置中基于曲硫丹的预处理数据有限.我们回顾性比较了移植后环磷酰胺(PTCy)在HaploT之前使用氟达拉滨-环磷酰胺(FC)-美法仑(110mg/m2)和FC-曲硫丹(30g/m2)对≥50岁的高危MDS/AML患者的预处理,从2009-2021年在我们机构移植(n=80)。在通过配对分析平衡患者特征后,我们确定了21对匹配的。两组之间的两年OS和LFS相似(OS66%和LFS66%,p=0.8和p=0.57)。然而,与FC-曲硫丹相比,FC-美法仑的复发概率显着降低(0%与24%,p=0.006),由更高的NRM平衡(33%与10%,p=0.05)。植入时间和急性和慢性移植物抗宿主病(GvHD)的发生率没有显着差异。总之,在年龄≥50岁的MDS/AML患者中,使用FC-曲硫丹联合PTCy的HaploT似乎安全有效,特别是在疾病晚期。我们证实了有利的髓外毒性,允许潜在的剂量增强以增强抗白血病活性。
    Relapse and regimen-related toxicities remain major challenges in achieving long-term survival, particularly among older patients with high-risk myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML) undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). Previous studies have demonstrated the feasibility of treosulfan-based conditioning, noting stable engraftment and low non-relapse mortality (NRM) in patients undergoing HLA-matched allo-HSCT. However, data on treosulfan-based conditioning in the HLA-haploidentical transplantation (HaploT) setting are limited. We retrospectively compared conditioning with fludarabine-cyclophosphamide (FC)-melphalan (110 mg/m2) and FC-treosulfan (30 g/m2) prior to HaploT using post-transplantation cyclophosphamide (PTCy) in patients with high-risk MDS/AML patients ≥ 50 years, transplanted from 2009-2021 at our institution (n = 80). After balancing patient characteristics by a matched-pair analysis, we identified twenty-one matched pairs. Two-year OS and LFS were similar among the groups (OS 66% and LFS 66%, p = 0.8 and p = 0.57). However, FC-melphalan was associated with a significantly lower probability of relapse compared to FC-treosulfan (0% vs. 24%, p = 0.006), counterbalanced by a higher NRM (33% vs. 10%, p = 0.05). Time to engraftment and incidences of acute and chronic graft-versus-host disease (GvHD) did not differ significantly. In conclusion, HaploT using FC-treosulfan in combination with PTCy in patients aged ≥50 years with MDS/AML appears safe and effective, particularly in advanced disease stages. We confirm the favorable extramedullary toxicity profile, allowing for potential dose intensification to enhance antileukemic activity.
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  • 文章类型: Journal Article
    国家灾害管理局(BadanNasionalPenanggulanganBencana)通过收集,将印度尼西亚的灾害管理作为一个卫生集群来处理,存储,并在灾害期间从各种来源报告幸存者状况及其健康状况的信息。在纸上收集数据并转移到MicrosoftExcel电子表格。这些活动具有挑战性,因为没有数据收集标准。世界卫生组织(WHO)以最小数据集(MDS)的形式为紧急医疗队(EMT)引入了灾难期间的健康数据收集标准。同时,印度尼西亚卫生部启动了SATUSEHAT平台,以基于快速医疗互操作性资源(FHIR)整合印度尼西亚的所有电子病历。
    本研究旨在实施WHOEMTMDS,以使用FHIR为SATUSEHAT平台创建灾难简介。
    我们从2个EMTMDS病历-WHO和东南亚国家联盟(ASEAN)版本-以及每日报告表中提取了变量。然后,我们执行了映射过程,将这些变量与FHIR资源进行匹配,并分析了变量与基础资源之间的差距。接下来,我们进行了分析,以查看所选资源中是否有任何更改,并使用Forge应用程序创建了扩展以填补空白。随后,该配置文件是使用开源FHIR服务器实现的。
    从WHOEMTMDS中提取的变量总数,东盟EMTMDS,每日报告表格为30、32和46,与FHIR资源匹配的变量百分比为100%(30/30),97%(31/32),85%(39/46),分别。从FHIRID核心的40个可用资源中,我们为WHOEMTMDS使用了10、14和9,东盟EMTMDS,和每日报告表格,分别。根据差距分析,我们在每日报告表格中发现了4个不在资源范围内的变量.因此,我们创建了扩展来解决这个差距。
    我们成功创建了一个灾难配置文件,该配置文件可用作SATUSEHAT平台的灾难案例。此配置文件可以在灾难期间标准化健康数据收集。
    UNASSIGNED: The National Disaster Management Agency (Badan Nasional Penanggulangan Bencana) handles disaster management in Indonesia as a health cluster by collecting, storing, and reporting information on the state of survivors and their health from various sources during disasters. Data were collected on paper and transferred to Microsoft Excel spreadsheets. These activities are challenging because there are no standards for data collection. The World Health Organization (WHO) introduced a standard for health data collection during disasters for emergency medical teams (EMTs) in the form of a minimum dataset (MDS). Meanwhile, the Ministry of Health of Indonesia launched the SATUSEHAT platform to integrate all electronic medical records in Indonesia based on Fast Healthcare Interoperability Resources (FHIR).
    UNASSIGNED: This study aims to implement the WHO EMT MDS to create a disaster profile for the SATUSEHAT platform using FHIR.
    UNASSIGNED: We extracted variables from 2 EMT MDS medical records-the WHO and Association of Southeast Asian Nations (ASEAN) versions-and the daily reporting form. We then performed a mapping process to match these variables with the FHIR resources and analyzed the gaps between the variables and base resources. Next, we conducted profiling to see if there were any changes in the selected resources and created extensions to fill the gap using the Forge application. Subsequently, the profile was implemented using an open-source FHIR server.
    UNASSIGNED: The total numbers of variables extracted from the WHO EMT MDS, ASEAN EMT MDS, and daily reporting forms were 30, 32, and 46, with the percentage of variables matching FHIR resources being 100% (30/30), 97% (31/32), and 85% (39/46), respectively. From the 40 resources available in the FHIR ID core, we used 10, 14, and 9 for the WHO EMT MDS, ASEAN EMT MDS, and daily reporting form, respectively. Based on the gap analysis, we found 4 variables in the daily reporting form that were not covered by the resources. Thus, we created extensions to address this gap.
    UNASSIGNED: We successfully created a disaster profile that can be used as a disaster case for the SATUSEHAT platform. This profile may standardize health data collection during disasters.
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  • 文章类型: Journal Article
    骨髓增生异常综合征(MDS)是一种罕见的早期血癌,通常会导致白血病和其他致命并发症。MDS的典型诊断包括混合血液检查,骨髓活检,和遗传分析。流式细胞术通常用于分析这些类型的样品,然而,在几个领域似乎仍然有进步的空间,如检测限,周转时间,和成本。本文探讨了微流式细胞术技术的最新进展,以及如何将其用于补充诊断血癌的常规方法。如MDS和白血病,通过流式细胞术。微流式细胞术,最近对经过充分研究的传统流式细胞术技术进行了调整,与微流体集成,在诊断血液相关疾病如MDS时,在解决流式细胞术面临的许多缺点方面表现出巨大的潜力。这个平台带来的好处,如便携性,处理速度,和运营成本,举例说明探索微流式细胞术作为MDS和其他形式的血癌的即时(POC)诊断设备的重要性。
    Myelodysplastic syndromes (MDS) are a rare form of early-stage blood cancer that typically leads to leukemia and other deadly complications. The typical diagnosis for MDS involves a mixture of blood tests, a bone marrow biopsy, and genetic analysis. Flow cytometry has commonly been used to analyze these types of samples, yet there still seems to be room for advancement in several areas, such as the limit of detection, turnaround time, and cost. This paper explores recent advancements in microflow cytometry technology and how it may be used to supplement conventional methods of diagnosing blood cancers, such as MDS and leukemia, through flow cytometry. Microflow cytometry, a more recent adaptation of the well-researched and conventional flow cytometry techniques, integrated with microfluidics, demonstrates significant potential in addressing many of the shortcomings flow cytometry faces when diagnosing a blood-related disease such as MDS. The benefits that this platform brings, such as portability, processing speed, and operating cost, exemplify the importance of exploring microflow cytometry as a point-of-care (POC) diagnostic device for MDS and other forms of blood cancer.
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  • 文章类型: Case Reports
    监测剂量系统(MDS)是一种有效的、可靠的和批准的设备在药房的药物再处理。这些系统意味着对适当的药物使用以及初级卫生保健与药剂师之间的合作进行审查。该案例研究描述了2021年和2022年因腰痛而进行手术干预的女性患者。患者描述了与不当药物使用相关的不受控制的慢性疼痛和混乱。在定期配药期间,检测到这些药物相关问题(MRP),并将患者转诊至MDS服务.实施后,消除了患者的困惑,实现了疼痛管理,提高她的生活质量。作为结论,药房提供的不同健康服务可以提高患者的生活质量,治疗依从性和MRP检测。
    Monitored Dosage Systems (MDS) are an efficient, reliable and approved device for drug reconditioning in pharmacy. These systems imply a review on proper drug use and the collaboration between primary health care and pharmacists. The case study describes a female patient with a surgical intervention due to lumbosciatica in 2021 and 2022. Patient describes uncontrolled chronic pain and confusion related to improper drug use. During regular dispensing of her medication, these medicine-related problems (MRP) were detected and the patient was referred to the MDS service. After its implementation, the patient\'s confusion was eliminated and pain management was achieved, increasing her quality of life. As a conclusion, the different health services provided by the pharmacy can improve a patient\'s quality of life, treatment adherence and MRP detection.
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  • 文章类型: Journal Article
    使用下一代测序(NGS)的全面基因组分析的出现揭示了丰富的潜在可操作的遗传畸变,这些畸变塑造了我们对癌症生物学格局的理解。异柠檬酸脱氢酶(IDH)是存在于细胞质(IDH1)和线粒体(IDH2和IDH3)中的酶。在线粒体中,它催化异柠檬酸酯的不可逆氧化脱羧,产生α-酮戊二酸和烟酰胺腺嘌呤二核苷酸磷酸(NADPH)以及二氧化碳(CO2)的生产。在细胞质中,IDH催化异柠檬酸脱羧为α-酮戊二酸以及α-酮戊二酸反向还原羧化为异柠檬酸。三羧酸循环中的这些限速步骤,以及细胞质对氧化应激的反应,在基因调控中起关键作用,细胞分化,和组织稳态。编码IDH1和IDH2的基因突变,不太常见,IDH3已在多种癌症中发现,最常见的是神经胶质瘤,急性髓系白血病(AML),软骨肉瘤,和肝内胆管癌。在本文中,我们打算阐明IDH异构体突变背后的理论病理生理学,它在癌症表现中的含义,并讨论有关新型IDH抑制剂的使用及其在治疗中的作用的一些可用临床数据。
    The advent of comprehensive genomic profiling using next-generation sequencing (NGS) has unveiled an abundance of potentially actionable genetic aberrations that have shaped our understanding of the cancer biology landscape. Isocitrate dehydrogenase (IDH) is an enzyme present in the cytosol (IDH1) and mitochondria (IDH2 and IDH3). In the mitochondrion, it catalyzes the irreversible oxidative decarboxylation of isocitrate, yielding the production of α-ketoglutarate and nicotinamide adenine dinucleotide phosphate (NADPH) as well as carbon dioxide (CO2). In the cytosol, IDH catalyzes the decarboxylation of isocitrate to α-ketoglutarate as well as the reverse reductive carboxylation of α-ketoglutarate to isocitrate. These rate-limiting steps in the tricarboxylic acid cycle, as well as the cytoplasmic response to oxidative stress, play key roles in gene regulation, cell differentiation, and tissue homeostasis. Mutations in the genes encoding IDH1 and IDH2 and, less commonly, IDH3 have been found in a variety of cancers, most commonly glioma, acute myeloid leukemia (AML), chondrosarcoma, and intrahepatic cholangiocarcinoma. In this paper, we intend to elucidate the theorized pathophysiology behind IDH isomer mutation, its implication in cancer manifestation, and discuss some of the available clinical data regarding the use of novel IDH inhibitors and their role in therapy.
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  • 文章类型: Journal Article
    GATA2缺乏症是儿童和青少年儿童骨髓增生异常综合征(MDS)最常见的遗传易感性之一。广泛的疾病包括,其中,血液学,免疫和肺部表现,以及偶尔明显的器官异常。由于进展风险升高,几乎所有GATA2相关MDS患者最终都在其生命中的某个阶段接受了造血干细胞移植(HSCT).然而,最佳时机,方法,甚至在某些情况下HSCT的适应症仍存在争议,需要进一步研究。在这篇文章中,我们报告了5例GATA2缺乏的血液学和免疫学表现不同的患者,从无染色体畸变的儿童免疫缺陷和难治性血细胞减少到复发性MDS相关急性髓细胞性白血病.我们讨论采用的策略,包括监视的强度,HSCT的指示和定时,基于形态学,临床和分子标志物,以及患者的个人需求。我们得出的结论是,更好地表征自然疾病的病程,为了实现旨在改善这些患者护理的个性化方法,需要更好地理解躯体畸变的预后意义,并全面评估患者的观点和偏好。
    GATA2 deficiency is one of the most common genetic predispositions to pediatric myelodysplastic syndrome (MDS) in children and adolescents. The wide spectrum of disease comprises, among others, hematological, immunological and pulmonary manifestations, as well as occasionally distinct organ anomalies. Due to the elevated risk of progression, nearly all individuals with GATA2-related MDS eventually undergo a hematopoietic stem cell transplantation (HSCT) at some point in their lives. Nevertheless, the optimal timing, method, and even the indication for HSCT in certain cases are still matter of debate and warrant further research. In this article, we report five patients with different hematological and immunological manifestations of GATA2 deficiency ranging from immunodeficiency and refractory cytopenia of childhood without chromosomal aberrations to relapsed MDS-related acute myeloid leukemia. We discuss the adopted strategies, including intensity of surveillance, indication and timing of HSCT, based on morphological, clinical and molecular markers, as well as individual patient needs. We conclude that a better characterization of the natural disease course, a better understanding of the prognostic significance of somatic aberrations and a thorough evaluation of patients´ perspectives and preferences are required to achieve a personalized approach aimed at improving the care of these patients.
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  • 文章类型: Journal Article
    土壤侵蚀对土壤质量的影响仍未得到系统的理解。因此,这项研究的目的是量化土壤侵蚀对土壤质量的影响及其随农田坡度形态的变化,基于放射性核素137Cs的中国东北,无人机推导出高分辨率数字高程模型,和土壤取样。137Cs方法产生的平均土壤侵蚀率为-275tkm-2yr-1,范围为-1870至1557tkm-2yr-1。从总数据集(SQI_TDS)得出的土壤质量指标可以很好地解释为从最小数据集(SQI_MDS)得出的指标,决定系数R2为0.874。SOM,沙子,与其他土壤指标相比,MDS中的阳离子交换量起着更重要的作用。土壤质量受土壤侵蚀影响显著,Adj.SQI_TDS和SQI_MDS的R2为0.29和0.33,分别。土壤侵蚀和土壤质量的空间变化均受斜坡地形的影响。必须根据东北地区的地形和侵蚀特征来控制土壤侵蚀。
    The impact of soil erosion on soil quality is still not systematically understood. The purpose of this study was thus to quantify the impact of soil erosion on soil quality and its change with slope morphology in an agricultural field, northeastern China based on radionuclide 137Cs, unmanned aerial vehicle derived high resolution digital elevation model, and soil sampling. 137Cs method yielded an average soil erosion rate of - 275 t km-2 yr-1 ranging from - 1870 to 1557 t km-2 yr-1. The soil quality index derived from total dataset (SQI_TDS) can be well explained by that derived from minimum data set (SQI_MDS) with a determination coefficient R2 of 0.874. SOM, sand, and cation exchange capacity in the MDS play more important roles than other soil indicators. Soil quality was significantly affected by soil erosion, with Adj. R2 of 0.29 and 0.33 for SQI_TDS and SQI_MDS, respectively. The spatial variations of soil erosion and soil quality were both affected by slope topography. Soil erosion must be controlled according to topographic and erosion characteristics in northeastern China.
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  • 文章类型: Journal Article
    低碳钢在酸性环境中的劣化在各种行业中提出了重大挑战。有效腐蚀抑制剂的出现引起了人们对旨在减少腐蚀有害后果的研究的关注。在这项研究中,首次通过各种电化学和重量分析技术研究了Prinivil在1MHCl溶液中的腐蚀抑制效率。结果表明,Prinivil的抑制效率从50ppm时的61.37%扩展到298K时500ppm浓度时的97.35%。在500ppm的抑制剂浓度下,回归系数(R2)为0.987,Kads值为0.935,Ea值为43.024kJ/mol,已发现Prinivil对金属表面的吸附具有很强的亲和力。扫描电子显微镜(SEM)和接触角测量分析进一步支持Prinivil的抑制行为,证明了在低碳钢表面产生了防御层。此外,采用分子动力学(MD)和蒙特卡罗模拟研究了原子水平上Prinivil与金属表面(Fe(110))之间的稳定性和相互作用。计算结果表明,Prinivil在钢表面具有很强的吸附作用,确认其作为腐蚀抑制剂的可行性。
    The deterioration of mild steel in an acidic environment poses a significant challenge in various industries. The emergence of effective corrosion inhibitors has drawn attention to studies aimed at reducing the harmful consequences of corrosion. In this study, the corrosion inhibition efficiency of Prinivil in a 1M HCl solution through various electrochemical and gravimetric techniques has been investigated for the first time. The results demonstrated that the inhibition efficiency of Prinivil expanded from 61.37% at 50 ppm to 97.35% at 500 ppm concentration at 298 K. With a regression coefficient (R 2) of 0.987, Kads value of 0.935 and Ea value of 43.024 kJ/mol at 500 ppm concentration of inhibitor, a strong affinity of Prinivil for adsorption onto the metal surface has been significantly found. Scanning electron microscopy (SEM) and contact angle measurement analyses further support the inhibitory behavior of Prinivil, demonstrating the production of a defensive layer on the surface of mild steel. Additionally, molecular dynamics (MD) and Monte Carlo simulations were employed to investigate the stability and interactions between Prinivil and the metallic surface (Fe (1 1 0)) at the atomic level. The computed results reveal strong adsorption of Prinivil upon the steel surface, confirming its viability as a corrosion inhibitor.
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  • 文章类型: Journal Article
    背景:严重疾病对话可以帮助患者避免不必要的治疗。我们先前为患有急性髓细胞性白血病和骨髓增生异常综合征的老年人试行了远程健康严重疾病护理计划(SICP)。
    目的:在本研究中,我们旨在从临床医生的角度了解远程医疗SICP的经验。
    方法:我们研究了10名临床医生,他们向20名患有急性髓细胞性白血病或骨髓增生异常综合征的老年人提供了远程医疗SICP。定量结果包括置信度和可接受性。使用22项调查(范围1-7;得分越高越好)来衡量信心。使用11项调查(5点Likert量表)测量可接受性。由于试点性质和样本量小,在α=.10(2尾)进行了假设检验。临床医生在研究结束时参加了音频记录的定性访谈,以讨论他们的经验。
    结果:共有8名临床医生完成了置信度测量,7名临床医生完成了可接受性测量。我们发现总体置信度有统计学上的显着增加(平均增加0.5,SD0.6;P=0.03)。信心增加最大的是帮助家庭和解和告别(平均1.4,标准差1.5;P=.04)。大多数临床医生同意该格式简单(6/7,86%)且易于使用(6/7,86%)。临床医生认为远程医疗SICP可有效了解患者对临终关怀的价值(7/7,100%)。总共出现了三个定性主题:(1)远程医疗SICP加深了关系并重新建立了信任;(2)每次远程医疗SICP访问都以积极的方式感到独特和个性化;(3)不间断,不匆忙的时间优化了访问体验。
    结论:远程医疗SICP增加了进行严重疾病对话的信心,同时加深了患者与临床医生的关系。
    背景:ClinicalTrials.govNCT04745676;https://www.临床试验.gov/研究/NCT04745676。
    BACKGROUND: Serious illness conversations may help patients avoid unwanted treatments. We previously piloted the telehealth Serious Illness Care Program (SICP) for older adults with acute myeloid leukemia and myelodysplastic syndrome.
    OBJECTIVE: In this study, we aimed to understand the experience of the telehealth SICP from the clinician\'s perspective.
    METHODS: We studied 10 clinicians who delivered the telehealth SICP to 20 older adults with acute myeloid leukemia or myelodysplastic syndrome. Quantitative outcomes included confidence and acceptability. Confidence was measured using a 22-item survey (range 1-7; a higher score is better). Acceptability was measured using an 11-item survey (5-point Likert scale). Hypothesis testing was performed at α=.10 (2-tailed) due to the pilot nature and small sample size. Clinicians participated in audio-recorded qualitative interviews at the end of the study to discuss their experience.
    RESULTS: A total of 8 clinicians completed the confidence measure and 7 clinicians completed the acceptability measure. We found a statistically significant increase in overall confidence (mean increase of 0.5, SD 0.6; P=.03). The largest increase in confidence was in helping families with reconciliation and goodbye (mean 1.4, SD 1.5; P=.04). The majority of clinicians agreed that the format was simple (6/7, 86%) and easy to use (6/7, 86%). Clinicians felt that the telehealth SICP was effective in understanding their patients\' values about end-of-life care (7/7, 100%). A total of three qualitative themes emerged: (1) the telehealth SICP deepened relationships and renewed trust; (2) each telehealth SICP visit felt unique and personal in a positive way; and (3) uninterrupted, unrushed time optimized the visit experience.
    CONCLUSIONS: The telehealth SICP increased confidence in having serious illness conversations while deepening patient-clinician relationships.
    BACKGROUND: ClinicalTrials.gov NCT04745676; https://www.clinicaltrials.gov/study/NCT04745676.
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