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  1. Article ; Online: Evolution of nutritional management in children with cystic fibrosis - a narrative review.

    Smith, Chris / Lowdon, Jacqueline / Noordhoek, Jacqueline / Wilschanski, Michael

    Journal of human nutrition and dietetics : the official journal of the British Dietetic Association

    2024  

    Abstract: Nutrition has played a central role in the management and outcomes of people with cystic fibrosis (pwCF) since the 1970s. Advances in therapies and practices in recent decades have led to a significant change in the patient landscape with dramatic ... ...

    Abstract Nutrition has played a central role in the management and outcomes of people with cystic fibrosis (pwCF) since the 1970s. Advances in therapies and practices in recent decades have led to a significant change in the patient landscape with dramatic improvements in life expectancy, as well as quality of life, bringing with it new issues. Historically, cystic fibrosis was a condition associated with childhood and malnutrition; however, changes in patient demographics, nutritional assessment and fundamental nutritional management have evolved, and it has become an increasingly prevalent adult disease with new nutritional challenges, including obesity. This paper aims to describe these changes and the impact and challenges they bring for those working in this field. Nutritional professionals will need to evolve, adapt and remain agile to the wider range of situations and support required for a new generation of pwCF. Specialised nutrition support will continue to be required, and it will be additionally important to improve and optimise quality of life and long-term health.
    Language English
    Publishing date 2024-04-25
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 645183-4
    ISSN 1365-277X ; 0952-3871 ; 1465-8178
    ISSN (online) 1365-277X
    ISSN 0952-3871 ; 1465-8178
    DOI 10.1111/jhn.13298
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 2551: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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  2. Article ; Online: 3.22 Nutrition in Cystic Fibrosis.

    Slae, Mordechai / Wilschanski, Michael

    World review of nutrition and dietetics

    2022  Volume 124, Page(s) 374–381

    MeSH term(s) Cystic Fibrosis/complications ; Enteral Nutrition ; Humans ; Nutritional Status
    Language English
    Publishing date 2022-03-03
    Publishing country Switzerland
    Document type Journal Article ; Review
    ISSN 1662-3975 ; 0512-3593 ; 0084-2230
    ISSN (online) 1662-3975
    ISSN 0512-3593 ; 0084-2230
    DOI 10.1159/000517004
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  3. Article ; Online: Nutritional and metabolic management for cystic fibrosis in a post-cystic fibrosis transmembrane conductance modulator era.

    Wilschanski, Michael / Peckham, Daniel

    Current opinion in pulmonary medicine

    2022  Volume 28, Issue 6, Page(s) 577–583

    Abstract: Purpose of review: The introduction of highly effective cystic fibrosis transmembrane conductance regulator modulators has resulted in a paradigm shift towards treating underlying cause of cystic fibrosis (CF) rather than the ensuing complications. In ... ...

    Abstract Purpose of review: The introduction of highly effective cystic fibrosis transmembrane conductance regulator modulators has resulted in a paradigm shift towards treating underlying cause of cystic fibrosis (CF) rather than the ensuing complications. In this review, we will describe the impact of these small molecules on growth, nutrition, and metabolic status in people with CF (pwCF).
    Recent finding: Results of clinical trials and real world data demonstrate that these small molecules are having a significant impact of on augmenting body weight, improving nutritional status and reducing gastrointestinal symptom burden. Early treatment can also positively impact on pancreatic endocrine and exocrine function.
    Summary: Nutritional and metabolic management of pwCF needs to change in order to maximize long term health and avoid future complications relating to obesity and increased cardiovascular risk. Longitudinal registry studies will be key to improve our understanding of the longer-term outcome of these new therapies.
    MeSH term(s) Cystic Fibrosis ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans ; Lung ; Mutation ; Nutritional Status
    Chemical Substances Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6)
    Language English
    Publishing date 2022-09-14
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 1285505-4
    ISSN 1531-6971 ; 1070-5287 ; 1078-1641
    ISSN (online) 1531-6971
    ISSN 1070-5287 ; 1078-1641
    DOI 10.1097/MCP.0000000000000917
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    In stock of ZB MED Cologne/Königswinter

    Zs.A 4900: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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    ab Jg. 2022: Lesesaal (EG)

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  4. Article ; Online: Cystic Fibrosis Related Gastrointestinal Manifestations - Moving Forward.

    Birimberg-Schwartz, Liron / Wilschanski, Michael

    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

    2021  Volume 20, Issue 4, Page(s) 562–563

    MeSH term(s) Cystic Fibrosis/complications ; Cystic Fibrosis/diagnosis ; Gastrointestinal Diseases/diagnosis ; Gastrointestinal Diseases/etiology ; Humans
    Language English
    Publishing date 2021-08-25
    Publishing country Netherlands
    Document type Editorial ; Comment
    ZDB-ID 2084724-5
    ISSN 1873-5010 ; 1569-1993
    ISSN (online) 1873-5010
    ISSN 1569-1993
    DOI 10.1016/j.jcf.2021.07.011
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    In stock of ZB MED Cologne/Königswinter

    Zs.A 6028: Show issues Location:
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    Zs.MO 459: Show issues

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  5. Article ; Online: 3.19 Nutrition in cystic fibrosis.

    Wilschanski, Michael

    World review of nutrition and dietetics

    2015  Volume 113, Page(s) 244–249

    MeSH term(s) Adolescent ; Body Weight/physiology ; Child ; Child Development/physiology ; Child, Preschool ; Cystic Fibrosis/complications ; Cystic Fibrosis/diet therapy ; Cystic Fibrosis/metabolism ; Humans ; Infant ; Nutrition Assessment ; Nutrition Disorders/etiology ; Nutrition Disorders/prevention & control ; Nutritional Status
    Language English
    Publishing date 2015
    Publishing country Switzerland
    Document type Journal Article ; Review
    ISSN 1662-3975 ; 0512-3593 ; 0084-2230
    ISSN (online) 1662-3975
    ISSN 0512-3593 ; 0084-2230
    DOI 10.1159/000367876
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  6. Article: Cystic fibrosis and the gut.

    Slae, Mordechai / Wilschanski, Michael

    Frontline gastroenterology

    2020  Volume 12, Issue 7, Page(s) 622–628

    Abstract: Cystic fibrosis (CF) is a recessive disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. The gene product, CFTR protein, has important manifestations in the intestine, pancreas and hepatobiliary system. Increased ... ...

    Abstract Cystic fibrosis (CF) is a recessive disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. The gene product, CFTR protein, has important manifestations in the intestine, pancreas and hepatobiliary system. Increased survival has caused CF to be primarily an adult disease today. Physicians must be knowledgeable as to the varied phenotype in the gastrointestinal tract. This review will outline the main gastrointestinal manifestations including a section on gastrointestinal malignancy in CF. Novel treatments treating the basic effect in CF are now being introduced and their effects on the gastrointestinal tract are discussed.
    Language English
    Publishing date 2020-10-27
    Publishing country England
    Document type Journal Article
    ZDB-ID 2521857-8
    ISSN 2041-4137
    ISSN 2041-4137
    DOI 10.1136/flgastro-2020-101610
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    In stock of ZB MED Cologne/Königswinter

    Zs.A 6880: Show issues Location:
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  7. Article ; Online: A Green Light for Stop Mutations.

    Cohen-Cymberknoh, Malena / Slae, Mordechai / Wilschanski, Michael

    American journal of respiratory cell and molecular biology

    2021  Volume 64, Issue 5, Page(s) 531–532

    MeSH term(s) Mutation
    Language English
    Publishing date 2021-03-11
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 1025960-0
    ISSN 1535-4989 ; 1044-1549
    ISSN (online) 1535-4989
    ISSN 1044-1549
    DOI 10.1165/rcmb.2021-0060ED
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    In stock of ZB MED Cologne/Königswinter

    Zs.A 2851: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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    ab Jg. 2022: Lesesaal (EG)

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  8. Article ; Online: Prevention of malnutrition in cystic fibrosis.

    Slae, Mordechai / Wilschanski, Michael

    Current opinion in pulmonary medicine

    2019  Volume 25, Issue 6, Page(s) 674–679

    Abstract: Purpose of review: Malnutrition is one of the major burdens of disease in cystic fibrosis. The prevention of malnutrition remains a priority throughout the life of a patient with cystic fibrosis. Literature and guidelines on the management of nutrition ... ...

    Abstract Purpose of review: Malnutrition is one of the major burdens of disease in cystic fibrosis. The prevention of malnutrition remains a priority throughout the life of a patient with cystic fibrosis. Literature and guidelines on the management of nutrition in cystic fibrosis have been published; however, here we review updated findings in cystic fibrosis nutrition as well as the role of novel treatments.
    Recent findings: We review the latest studies on the importance and consequences of nutrition in cystic fibrosis. Novel findings on specific nutrients such as vitamin D and sodium can improve our care and thereby health and growth outcomes. The role of exercise has been further studied. In the field of new treatments, we review the role of cystic fibrosis transmembrane-conductance regulator potentiators and modulators in cystic fibrosis nutrition. A new feeding tube fat-digesting device has been developed and shows promise in cystic fibrosis enteral nutrition.
    Summary: Advances in the nutritional care of cystic fibrosis are forming and believed to further develop in the near future, adding to the recent progress in cystic fibrosis patients' health, survival, and quality of life.
    MeSH term(s) Cystic Fibrosis/metabolism ; Cystic Fibrosis/therapy ; Humans ; Malnutrition/etiology ; Malnutrition/prevention & control ; Nutrition Therapy/methods ; Nutrition Therapy/trends ; Nutritional Status
    Language English
    Publishing date 2019-09-30
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1285505-4
    ISSN 1531-6971 ; 1070-5287 ; 1078-1641
    ISSN (online) 1531-6971
    ISSN 1070-5287 ; 1078-1641
    DOI 10.1097/MCP.0000000000000629
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 4900: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)

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  9. Article ; Online: Novel therapeutic approaches for cystic fibrosis.

    Wilschanski, Michael

    Discovery medicine

    2013  Volume 15, Issue 81, Page(s) 127–133

    Abstract: Cystic fibrosis (CF) is the most common lethal monogenic disorder. Life expectancy of CF patients is rising towards a mean of 40 years with advances in all aspects of therapy apart from treating the basic molecular defect. In the twenty three years since ...

    Abstract Cystic fibrosis (CF) is the most common lethal monogenic disorder. Life expectancy of CF patients is rising towards a mean of 40 years with advances in all aspects of therapy apart from treating the basic molecular defect. In the twenty three years since the discovery of the gene that causes cystic fibrosis, our knowledge of how mutations in this gene cause the varied pathophysiological manifestations of this disease has increased substantially. This knowledge has led to the possibility of new therapeutic approaches aimed at the basic defect. Apart from gene therapy, several novel compounds have recently been discovered using high-throughput screening which appear promising enough to develop into effective drugs to cure the basic defect. This article will summarize our current knowledge of mutation specific therapy and will focus on orally bioavailable potentiators and correctors and suppressors of premature termination codons. Further development of these drugs will enable treatment of the basic defect in diseases like CF and open the door for treatment of disease according to gene sequencing -- true personalized medicine.
    MeSH term(s) Cystic Fibrosis/genetics ; Cystic Fibrosis/pathology ; Cystic Fibrosis/therapy ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans
    Chemical Substances Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6)
    Language English
    Publishing date 2013-02
    Publishing country United States
    Document type Journal Article ; Review
    ISSN 1944-7930
    ISSN (online) 1944-7930
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Class 1 CF Mutations.

    Wilschanski, Michael

    Frontiers in pharmacology

    2012  Volume 3, Page(s) 117

    Language English
    Publishing date 2012-06-20
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2587355-6
    ISSN 1663-9812 ; 1663-9812
    ISSN (online) 1663-9812
    ISSN 1663-9812
    DOI 10.3389/fphar.2012.00117
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