특발성 폐섬유증 환자에게 흡입하는 제논 가스를 활용한 MRI로 폐 기능 변화를 관찰하는 안전한 초기 연구입니다.

Whether magnetic resonance imaging (MRI) using inhaled hyper-polarized 129 Xenon gas can help visualize impaired lung function to detect changes over time in Idiopathic Pulmonary Fibrosis (IPF) patients receiving approved IPF treatments

Hyperpolarized 129 Xenon Gas Comparing Idiopathic Pulmonary Fibrosis (IPF) Treatment

Participants will complete an baseline series of questionnaires, and then meet with the psychologist on their cystic fibrosis care team for 3 weekly meetings to complete the PACE CF program and learn mind-body strategies for pain management. Following completion of the program, they will complete a second assessment that includes another set of questionnaires and a brief qualitative interview to provide feedback about the program.

The Pain Acknowledgment Coping and Empowerment in CF (PACE CF) program is a newly developed 3-session mind-body pain management intervention for adults with cystic fibrosis (CF), developed with CF community input. The sessions are delivered by the the CF care team psychologist. Each session will teach mind-body strategies to increase comfort and reduce the impact of pain on the participant\&#39;s life. These strategies include understanding pain in CF from a mind-body perspective, learning relaxation and mindfulness-based skills, identifying and practicing individualized cognitive and behavioral strategies that improve comfort and well-being, and discussing communication skills that empower people living with CF and pain. Participants will receive a workbook that will provide related education and resources and reinforce skill practice.

Pain Acknowledgment Coping and Empowerment in CF (PACE CF)

낭포성 섬유증 형제를 둔 건강한 아이들의 정신 건강과 관계 개선을 위해 인터넷 프로그램 효과를 확인하는 시험입니다.

Internet-delivered Cystic Fibrosis Mental Health Prevention, Wellness, and Resource (iCF-PWR) program is comprised of five text/voice-delivered, animated, interactive modules. The pathways are comprised of the same modules that take approximately 15 to 20 minutes to complete. Modules are comprised of web pages and/or screens with numerous illustrations and interactive components. Written module content will be presented verbally as well as in text format.

The program modules include: (1) What is CF? (i.e., physiological explanation of CF, prevalence rate); (2) How does my sibling with CF stay healthy? (i.e., review of medication, nutrition, physiotherapy treatment, and why treatment is important); (3) How does CF affect me?; (4) Mental health awareness (i.e., introduction to cognitive behaviour model of emotions-thoughts, feelings, bodily sensations, and behaviours); and (5) Strategies (i.e., ways to challenge unhelpful thoughts, talking about emotions, relaxation).

The iCF-PWR program is a self-guided mental health prevention program designed for families with CF.

Internet-delivered Cystic Fibrosis Mental Health Prevention, Wellness, and Resource (iCF-PWR) program

인터넷을 통해 제공되는 낭포성 섬유증 정신 건강 예방, 웰빙 및 자원(iCF-PWR) 프로그램은 텍스트/음성으로 전달되는 5개의 애니메이션 대화형 모듈로 구성되어 있습니다. 각 과정은 완료하는 데 약 15~20분이 소요되는 동일한 모듈로 구성되어 있습니다. 모듈은 다양한 그림과 대화형 구성 요소가 포함된 웹 페이지 및/또는 화면으로 구성됩니다. 서면 모듈 콘텐츠는 텍스트 형식뿐만 아니라 구두로도 제공됩니다.

프로그램 모듈은 다음과 같습니다. (1) 낭포성 섬유증이란 무엇인가? (즉, 낭포성 섬유증의 생리적 설명, 유병률); (2) 낭포성 섬유증이 있는 내 형제자매는 어떻게 건강을 유지하는가? (즉, 약물, 영양, 물리치료에 대한 검토 및 치료의 중요성); (3) 낭포성 섬유증이 나에게 어떤 영향을 미치는가? (4) 정신 건강 인식 (즉, 감정(생각, 감정, 신체 감각, 행동)에 대한 인지 행동 모델 소개); (5) 전략(즉, 도움이 되지 않는 생각에 도전하는 방법, 감정에 대해 이야기하기, 이완하기).

인터넷을 통한 낭포성 섬유증 정신 건강 예방, 웰빙 및 자원(iCF-PWR) 프로그램

낭포성 섬유증 환자의 건강한 형제자매를 위한 iCF-PWR의 개발 및 평가

낭포성 섬유증 어린이와 형제를 위한 온라인 정신 건강 프로그램의 효과를 알아보는 시험으로, 삶의 질 개선을 목표로 합니다.

Parents are encouraged to review the program along with their child and then children are encouraged to complete the program 1-2 additional times (or as many times as they like). It is suggested that modules be completed at a rate of 1-2 per week, with program completion ranging from 3-6 weeks. Additional mental health resources are provided at the end of the program.

The iCF-PWR program is a self-guided mental health prevention program designed for families with CF. Upon logging on to the iCF-PWR, the viewer will see two paths (i.e., child with CF or sibling) and will be encouraged by program narrator to choose the appropriate path. Each pathway (i.e., child with CF or sibling) is comprised of five text/voice-delivered, animated, interactive modules: (1) CF education, (2) CF health, (3) emotions and CF, (4) cognitive behaviour model of emotions, and (5) coping strategies. Each module takes 15-20 minutes to complete.

Participants will continue to receive their usual standard care related to CF (i.e., accessing services through their local health authority and CF clinic). Following the proposed maximum program completion time-frame (i.e., 6 weeks) and follow-up time period (i.e., 3 months), those in the standard care groups will be provided access to iCF-PWR.

부모님께서는 자녀와 함께 프로그램을 검토해 보시고, 자녀는 1~2회(또는 원하는 횟수만큼) 추가적으로 프로그램을 이수하시기 바랍니다. 모듈은 주 1~2회 이수하는 것이 좋으며, 프로그램 완료 기간은 3~6주입니다. 프로그램 종료 시 추가적인 정신 건강 관련 자료가 제공됩니다.

참가자들은 낭포성 섬유증(CF) 관련 기존 표준 치료(즉, 지역 보건 당국 및 낭포성 섬유증 클리닉을 통한 서비스 이용)를 계속 받게 됩니다. 제안된 최대 프로그램 완료 기간(즉, 6주) 및 후속 조치 기간(즉, 3개월)이 지나면 표준 치료 그룹에 속한 사람들에게 iCF-PWR이 제공됩니다.

Mark Chilvers, MRCPCH, MD

Julian Tam, MD

Niki Asiff, RN

인터넷을 통한 낭포성 섬유증 정신 건강 예방, 웰빙, 자원 프로그램: 어떻게 작동하나요?

듀크 대학교에서 진행하는 이 연구는 과분극 129 제논 가스를 이용해 진행성 폐섬유증 환자의 치료 효과를 MRI로 비교하는 시험입니다.

Whether magnetic resonance imaging (MRI) using inhaled hyper-polarized 129 Xenon gas can help visualize impaired lung function to detect changes over time in Progressive Pulmonary Fibrosis patients receiving approved treatments.

Whether magnetic resonance imaging (MRI) using inhaled hyper-polarized 129 Xenon gas can help visualize impaired lung function to detect changes over time in Progressive Pulmonary Fibrosis

Hyperpolarized 129 Xenon Gas Comparing Progressive Pulmonary Fibrosis Treatment

This study aims to identify inherited genetic variants and somatic mutations associated with the progression of advanced Metabolic Dysfunction-Associated Steatotic Liver Disease MASLD.The research utilizes two distinct and extensively characterized cohorts for Whole-Genome Sequencing WGS analysis:

* MASLD Cohort: Consists of 800 patients whose peripheral blood will undergo WGS at 20x coverage.
* Metabolic Controls} Cohort: Comprises 2000 individuals matched for sex and metabolic risk factors who meet at least two criteria for metabolic syndrome but are free of MASLD. Their peripheral blood DNA will also be sequenced.

The focusing is the attention on the genetic and somatic variants involved in its progression toward advanced fibrosis and hepatocellular carcinoma. Through Whole-Genome Sequencing WGS of 800 patients with advanced MASLD, 80 liver tissue samples, and 2000 controls, the study aims to:

* Identify rare and structural genetic variants associated with the disease
* Analyze the role of clonal hematopoiesis of indeterminate potential CHIP in MASLD progression.
* Develop polygenic risk scores to improve risk stratification.

genetic and somatic variants involved in its progression toward advanced fibrosis and hepatocellular carcinoma

Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico

Luca Vittorio Carlo Valenti, Doctor

A pilot study to test the ability of qMT to quantify fibrosis in the post-stenotic human kidney, in comparison to innovative biomarkers of renal dysfunction and tissue damage. We will pursue the Specific Aim that qMT in stenotic human kidneys is feasible and reproducible.

The hypothesis underlying this proposal is that qMT would reliably detect development of renal fibrosis at both 1.5T and 3.0T in subjects with RVD. Patients will be studied with MRI (for fibrosis) and CT (for renal function).

과분극화된 129 크세논 가스를 이용한 특발성 폐섬유증(IPF) 치료 비교

낭포성 섬유증 환자의 통증 인지, 대처 및 역량 강화(PACE CF)

낭포성 섬유증 환자의 건강한 형제자매를 위한 iCF-PWR의 개발 및 평가

인터넷을 통한 낭포성 섬유증 정신 건강 예방, 웰빙, 자원 프로그램: 어떻게 작동하나요?

과분극 129 제논 가스를 이용한 진행성 폐섬유증 치료 비교

진행성 섬유증 및 간세포암으로의 진행에 관여하는 유전적 및 체세포적 변이

섬유증을 감지하기 위한 qMT-MRI.