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Journal of cystic fibrosis : official journal of t... 25건

  1. [해외논문]   Apolipoprotein E polymorphism determines vitamin K supplementation effectiveness in cystic fibrosis patients  

    Krzyż (Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan University of Medical Sciences, Poznan, Poland ) , anowska-Jankowska, Patrycja (Department Pediatric Pneumonology, Allergology and Clinical Immunology, Poznan University of Medical Sciences, Poznan, Poland ) , Sobkowiak, Paulina (Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan University of Medical Sciences, Poznan, Poland ) , Thalmann, Olaf (Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan University of Medical Sciences, Poznan, Poland ) , Drzymała-Czyż (Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan University of Medical Sciences, Poznan, Poland ) , , Sławomira (Department of Biochemistry and Molecular Biology, Poznan University of Medical Sciences, Poznan, Poland ) , Glapa, Aleksandra (Department of Organization and Management in Health Care, Poznan, Poland ) , Hołysz, Marcin (Lviv Cystic Fibrosis Centre, Lviv, Ukraine ) , Walkowiak, Dariusz (Lviv Cystic Fibrosis Centre, Lviv, Ukraine ) , Rohovyk, Nataliya (Department of Pulmonology and Cystic Fibrosis, Institute of Tuberculosis and Lung Diseases, Rabka-Zdrój, Polan) , Bober, Lyudmyla , Pogorzelski, Andrzej , Walkowiak, Jarosław
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society v.17 no.4 ,pp. e39 - e40 , 2018 , 1569-1993 ,

    초록

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  2. [해외논문]   Sweat test for cystic fibrosis: Wearable sweat sensor vs. standard laboratory test  

    Choi, Dong-Hoon (Institute for Nanobiotechnology, Johns Hopkins University, Baltimore, MD, USA ) , Thaxton, Abigail (Division of Pulmonary and Critical Care, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD, USA ) , Jeong, In cheol (Measurement Corps, Johns Hopkins Individualized Health Initiative, Johns Hopkins University, Baltimore, MD, USA ) , Kim, Kain (Institute for Nanobiotechnology, Johns Hopkins University, Baltimore, MD, USA ) , Sosnay, Patrick R. (Division of Pulmonary and Critical Care, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD, USA ) , Cutting, Garry R. (Institute of Genetic Medicine, Johns Hopkins University, Baltimore, MD, USA ) , Searson, Peter C. (Institute for Nanobiotechnology, Johns Hopkins University, Baltimore, MD, USA)
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society v.17 no.4 ,pp. e35 - e38 , 2018 , 1569-1993 ,

    초록

    Abstract Background Sweat chloride testing for diagnosis of cystic fibrosis (CF) involves sweat induction, collection and handling, and measurement in an analytical lab. We have developed a wearable sensor with an integrated salt bridge for real-time measurement of sweat chloride concentration. Here, in a proof-of-concept study, we compare the performance of the sensor to current clinical practice in CF patients and healthy subjects. Method Sweat was induced on both forearms of 10 individuals with CF and 10 healthy subjects using pilocarpine iontophoresis. A Macroduct sweat collection device was attached to one arm and sweat was collected for 30 min and then sent for laboratory analysis. A sensor was attached to the other arm and the chloride ion concentration monitored in real time for 30 min using a Bluetooth transceiver and smart phone app. Results Stable sweat chloride measurements were obtained within 15 min following sweat induction using the wearable sensor. We define the detection time as the time at which the standard deviation of the real-time chloride ion concentration remained below 2 mEq/L for 5 min. The sweat volume for sensor measurements at the detection time was 13.1 ± 11.4 μL (SD), in many cases lower than the minimum sweat volume of 15 μL for conventional testing. The mean difference between sweat chloride concentrations measured by the sensor and the conventional laboratory practice was 6.2 ± 9.5 mEq/L (SD), close to the arm-to-arm variation of about 3 mEq/L. The Pearson correlation coefficient between the two measurements was 0.97 highlighting the excellent agreement between the two methods. Conclusion A wearable sensor can be used to make real-time measurements of sweat chloride within 15 min following sweat induction, requiring a small sweat volume, and with excellent agreement to standard methods. Graphical abstract [DISPLAY OMISSION]

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  3. [해외논문]   Editorial Board  


    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society v.17 no.4 ,pp. ii - ii , 2018 , 1569-1993 ,

    초록

    Abstract Background Sweat chloride testing for diagnosis of cystic fibrosis (CF) involves sweat induction, collection and handling, and measurement in an analytical lab. We have developed a wearable sensor with an integrated salt bridge for real-time measurement of sweat chloride concentration. Here, in a proof-of-concept study, we compare the performance of the sensor to current clinical practice in CF patients and healthy subjects. Method Sweat was induced on both forearms of 10 individuals with CF and 10 healthy subjects using pilocarpine iontophoresis. A Macroduct sweat collection device was attached to one arm and sweat was collected for 30 min and then sent for laboratory analysis. A sensor was attached to the other arm and the chloride ion concentration monitored in real time for 30 min using a Bluetooth transceiver and smart phone app. Results Stable sweat chloride measurements were obtained within 15 min following sweat induction using the wearable sensor. We define the detection time as the time at which the standard deviation of the real-time chloride ion concentration remained below 2 mEq/L for 5 min. The sweat volume for sensor measurements at the detection time was 13.1 ± 11.4 μL (SD), in many cases lower than the minimum sweat volume of 15 μL for conventional testing. The mean difference between sweat chloride concentrations measured by the sensor and the conventional laboratory practice was 6.2 ± 9.5 mEq/L (SD), close to the arm-to-arm variation of about 3 mEq/L. The Pearson correlation coefficient between the two measurements was 0.97 highlighting the excellent agreement between the two methods. Conclusion A wearable sensor can be used to make real-time measurements of sweat chloride within 15 min following sweat induction, requiring a small sweat volume, and with excellent agreement to standard methods. Graphical abstract [DISPLAY OMISSION]

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  4. [해외논문]   No easy road to better cystic fibrosis care in Eastern Europe?  

    De Boeck, Kris (Pediatric Pulmonology and Infectious Diseases, University Hospital of Leuven, University of Leuven, Belgium ) , Fajac, Isabelle (Université)
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society v.17 no.4 ,pp. 423 - 424 , 2018 , 1569-1993 ,

    초록

    Abstract Background Sweat chloride testing for diagnosis of cystic fibrosis (CF) involves sweat induction, collection and handling, and measurement in an analytical lab. We have developed a wearable sensor with an integrated salt bridge for real-time measurement of sweat chloride concentration. Here, in a proof-of-concept study, we compare the performance of the sensor to current clinical practice in CF patients and healthy subjects. Method Sweat was induced on both forearms of 10 individuals with CF and 10 healthy subjects using pilocarpine iontophoresis. A Macroduct sweat collection device was attached to one arm and sweat was collected for 30 min and then sent for laboratory analysis. A sensor was attached to the other arm and the chloride ion concentration monitored in real time for 30 min using a Bluetooth transceiver and smart phone app. Results Stable sweat chloride measurements were obtained within 15 min following sweat induction using the wearable sensor. We define the detection time as the time at which the standard deviation of the real-time chloride ion concentration remained below 2 mEq/L for 5 min. The sweat volume for sensor measurements at the detection time was 13.1 ± 11.4 μL (SD), in many cases lower than the minimum sweat volume of 15 μL for conventional testing. The mean difference between sweat chloride concentrations measured by the sensor and the conventional laboratory practice was 6.2 ± 9.5 mEq/L (SD), close to the arm-to-arm variation of about 3 mEq/L. The Pearson correlation coefficient between the two measurements was 0.97 highlighting the excellent agreement between the two methods. Conclusion A wearable sensor can be used to make real-time measurements of sweat chloride within 15 min following sweat induction, requiring a small sweat volume, and with excellent agreement to standard methods. Graphical abstract [DISPLAY OMISSION]

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  5. [해외논문]   Back to the source – Modern insights into pulmonary exacerbations and lung function decline from CF registry data  

    Rayment, Jonathan H. (Division of Respiratory Medicine, Department of Pediatrics, University of British Columbia, Vancouver, BC, Canada ) , Quon, Bradley S. (Centre for Heart Lung Innovation, University of British Columbia, St. Paul's Hospital, Vancouver, BC, Canada)
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society v.17 no.4 ,pp. 425 - 427 , 2018 , 1569-1993 ,

    초록

    Abstract Background Sweat chloride testing for diagnosis of cystic fibrosis (CF) involves sweat induction, collection and handling, and measurement in an analytical lab. We have developed a wearable sensor with an integrated salt bridge for real-time measurement of sweat chloride concentration. Here, in a proof-of-concept study, we compare the performance of the sensor to current clinical practice in CF patients and healthy subjects. Method Sweat was induced on both forearms of 10 individuals with CF and 10 healthy subjects using pilocarpine iontophoresis. A Macroduct sweat collection device was attached to one arm and sweat was collected for 30 min and then sent for laboratory analysis. A sensor was attached to the other arm and the chloride ion concentration monitored in real time for 30 min using a Bluetooth transceiver and smart phone app. Results Stable sweat chloride measurements were obtained within 15 min following sweat induction using the wearable sensor. We define the detection time as the time at which the standard deviation of the real-time chloride ion concentration remained below 2 mEq/L for 5 min. The sweat volume for sensor measurements at the detection time was 13.1 ± 11.4 μL (SD), in many cases lower than the minimum sweat volume of 15 μL for conventional testing. The mean difference between sweat chloride concentrations measured by the sensor and the conventional laboratory practice was 6.2 ± 9.5 mEq/L (SD), close to the arm-to-arm variation of about 3 mEq/L. The Pearson correlation coefficient between the two measurements was 0.97 highlighting the excellent agreement between the two methods. Conclusion A wearable sensor can be used to make real-time measurements of sweat chloride within 15 min following sweat induction, requiring a small sweat volume, and with excellent agreement to standard methods. Graphical abstract [DISPLAY OMISSION]

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  6. [해외논문]   Hypoglycaemia in patients with cystic fibrosis- harbinger of poor outcomes or innocent bystander?  

    Moheet, Amir (Department of Medicine, University of Minnesota, Minneapolis, MN, USA ) , Ode, Katie Larson (Department of Pediatrics, University of Iowa, Iowa City, IA, USA)
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society v.17 no.4 ,pp. 428 - 429 , 2018 , 1569-1993 ,

    초록

    Abstract Background Sweat chloride testing for diagnosis of cystic fibrosis (CF) involves sweat induction, collection and handling, and measurement in an analytical lab. We have developed a wearable sensor with an integrated salt bridge for real-time measurement of sweat chloride concentration. Here, in a proof-of-concept study, we compare the performance of the sensor to current clinical practice in CF patients and healthy subjects. Method Sweat was induced on both forearms of 10 individuals with CF and 10 healthy subjects using pilocarpine iontophoresis. A Macroduct sweat collection device was attached to one arm and sweat was collected for 30 min and then sent for laboratory analysis. A sensor was attached to the other arm and the chloride ion concentration monitored in real time for 30 min using a Bluetooth transceiver and smart phone app. Results Stable sweat chloride measurements were obtained within 15 min following sweat induction using the wearable sensor. We define the detection time as the time at which the standard deviation of the real-time chloride ion concentration remained below 2 mEq/L for 5 min. The sweat volume for sensor measurements at the detection time was 13.1 ± 11.4 μL (SD), in many cases lower than the minimum sweat volume of 15 μL for conventional testing. The mean difference between sweat chloride concentrations measured by the sensor and the conventional laboratory practice was 6.2 ± 9.5 mEq/L (SD), close to the arm-to-arm variation of about 3 mEq/L. The Pearson correlation coefficient between the two measurements was 0.97 highlighting the excellent agreement between the two methods. Conclusion A wearable sensor can be used to make real-time measurements of sweat chloride within 15 min following sweat induction, requiring a small sweat volume, and with excellent agreement to standard methods. Graphical abstract [DISPLAY OMISSION]

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  7. [해외논문]   News  


    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society v.17 no.4 ,pp. 430 - 432 , 2018 , 1569-1993 ,

    초록

    Abstract Background Sweat chloride testing for diagnosis of cystic fibrosis (CF) involves sweat induction, collection and handling, and measurement in an analytical lab. We have developed a wearable sensor with an integrated salt bridge for real-time measurement of sweat chloride concentration. Here, in a proof-of-concept study, we compare the performance of the sensor to current clinical practice in CF patients and healthy subjects. Method Sweat was induced on both forearms of 10 individuals with CF and 10 healthy subjects using pilocarpine iontophoresis. A Macroduct sweat collection device was attached to one arm and sweat was collected for 30 min and then sent for laboratory analysis. A sensor was attached to the other arm and the chloride ion concentration monitored in real time for 30 min using a Bluetooth transceiver and smart phone app. Results Stable sweat chloride measurements were obtained within 15 min following sweat induction using the wearable sensor. We define the detection time as the time at which the standard deviation of the real-time chloride ion concentration remained below 2 mEq/L for 5 min. The sweat volume for sensor measurements at the detection time was 13.1 ± 11.4 μL (SD), in many cases lower than the minimum sweat volume of 15 μL for conventional testing. The mean difference between sweat chloride concentrations measured by the sensor and the conventional laboratory practice was 6.2 ± 9.5 mEq/L (SD), close to the arm-to-arm variation of about 3 mEq/L. The Pearson correlation coefficient between the two measurements was 0.97 highlighting the excellent agreement between the two methods. Conclusion A wearable sensor can be used to make real-time measurements of sweat chloride within 15 min following sweat induction, requiring a small sweat volume, and with excellent agreement to standard methods. Graphical abstract [DISPLAY OMISSION]

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  8. [해외논문]   Cystic fibrosis patient registries: A valuable source for clinical research  

    Dasenbrook, Elliott C. (Cleveland Clinic Respiratory Institute, Cleveland, OH, United States ) , Sawicki, Gregory S. (Division of Respiratory Diseases, Boston Children's Hospital, Boston, MA, United States)
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society v.17 no.4 ,pp. 433 - 440 , 2018 , 1569-1993 ,

    초록

    Abstract Cystic Fibrosis (CF) patient registries are valuable data sources for researchers studying the natural history, treatment paradigms, and long‐term health outcomes of individuals with CF. In this review, we discuss the role of CF patient registries in facilitating comparative effectiveness research, particularly evaluating therapies and variation in health care delivery. We also discuss the limitations of registry‐based research, particularly indication bias, as well as statistical methods that can be used to address these issues.

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  9. [해외논문]   Cystic fibrosis: Beyond the airways. Report on the meeting of the basic science working group in Loutraki, Greece  

    Amaral, Margarida D. (University of Lisboa, Faculty of Sciences, BioISI - Biosystems & Integrative Sciences Institute, Campo Grande, C8 bdg, 1749-016 Lisboa, Portugal ) , Boj, Sylvia F. (Hubrecht Organoid Technology, Yalelaan 62, 3584 CM Utrecht, The Netherlands ) , Shaw, James (Institute of Cellular Medicine, Newcastle University Institute, The Medical School, 4<sup>th</sup>Floor William Leech Building, Framlington Place, Newcastle upon Tyne NE2 4HH, UK ) , Leipziger, Jens (Department of Biomedicine, Physiology and Biophysics, University of Aahrus, Ole Worms Alle 3, build 1170, 8000 Aarhus, Denmark ) , Beekman, Jeffrey M. (Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, Regenerative Medicine Center Utrecht, University Medical Center Utrecht, The Netherlands)
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society v.17 no.4 ,pp. 441 - 443 , 2018 , 1569-1993 ,

    초록

    Abstract The European Cystic Fibrosis Society (ECFS) Basic Science Working Group (BSWG) organized a session on the topic “ Cystic Fibrosis: Beyond the Airways ”, within the 15th ECFS Basic Science Conference which gathered around 200 researchers working in the basic science of CF. The session was organized and chaired by Margarida Amaral (BioISI, University of Lisboa, Portugal) and Jeffrey Beekman (University Medical Centre Utrecht, Netherlands) as Chair and Vice-Chair of the BSWG and its purpose was to bring attention of participants of the ECFS Basic Science Conference to “more forgotten” organs in CF disease. In this report we attempt to review and integrate the ideas that emerged at the session.

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  10. [해외논문]   Molecular characterization of gene regulatory networks in primary human tracheal and bronchial epithelial cells  

    Gillen, Austin E. (Human Molecular Genetics Program, Lurie Children's Research Center, Chicago, IL, 60614, United States ) , Yang, Rui (Human Molecular Genetics Program, Lurie Children's Research Center, Chicago, IL, 60614, United States ) , Cotton, Calvin U. (Departments of Pediatrics, Physiology and Biophysics, Case Western Reserve University, Cleveland, OH 44106, United States ) , Perez, Aura (Departments of Pediatrics, Physiology and Biophysics, Case Western Reserve University, Cleveland, OH 44106, United States ) , Randell, Scott H. (Marsico Lung Institute, University of North Carolina at Chapel Hill School of Medicine, Chapel Hill, NC 27599, United States ) , Leir, Shih-Hsing (Human Molecular Genetics Program, Lurie Children's Research Center, Chicago, IL, 60614, United States ) , Harris, Ann (Human Molecular Genetics Program, Lurie Children's Research Center, Chicago, IL, 60614, United States)
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society v.17 no.4 ,pp. 444 - 453 , 2018 , 1569-1993 ,

    초록

    Abstract Background Robust methods to culture primary airway epithelial cells were developed several decades ago and these cells provide the model of choice to investigate many diseases of the human lung. However, the molecular signature of cells from different regions of the airway epithelium has not been well characterized. Methods We utilize DNase-seq and RNA-seq to examine the molecular signatures of primary cells derived from human tracheal and bronchial tissues, as well as healthy and diseased (cystic fibrosis (CF)) donor lung tissue. Results Our data reveal an airway cell signature that is divergent from other epithelial cell types and from common airway epithelial cell lines. The differences between tracheal and bronchial cells are clearly evident as are common regulatory features. Only minor variation is seen between bronchial cells from healthy or CF donors. Conclusions These data are a valuable resource for functional genomics analysis of airway epithelial tissues in human disease.

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