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  1. Article: Association between cystic fibrosis transmembrane regulator genotype and clinical outcomes, glucose homeostasis indices and CF-related diabetes risk in adults with CF.

    Bélanger, Noémie / Bonhoure, Anne / Kherani, Tamizan / Boudreau, Valérie / Tremblay, François / Lavoie, Annick / Carricart, Maite / Marwaha, Ashish / Rabasa-Lhoret, Rémi / Potter, Kathryn J

    Genetics and molecular biology

    2024  Volume 47, Issue 1, Page(s) e20230021

    Abstract: People living with cystic fibrosis (pwCF) homozygous for F508del present more severe phenotypes. PwCF with compound heterozygous genotypes F508del /A455E and F508del /L206W may have milder cystic fibrosis (CF) phenotypes. We compared F508del homozygotes ... ...

    Abstract People living with cystic fibrosis (pwCF) homozygous for F508del present more severe phenotypes. PwCF with compound heterozygous genotypes F508del /A455E and F508del /L206W may have milder cystic fibrosis (CF) phenotypes. We compared F508del homozygotes and common compound heterozygotes (F508del and a second pathogenic variant) in adult patients. Nutritional, pulmonary function and glucose homeostasis indices data were collected from the prospective Montreal CF cohort. Two-hundred and three adults with CF having at least one F508del variant were included. Individuals were divided into subgroups: homozygous F508del/F508del (n=149); F508del/621+1G>T (n=17); F508del/711+1G>T (n=11); F508del/A455E (n=12); and F508del/L206W (n=14). Subgroups with the F508del/L206W and F508del/A455E had a lower proportion with pancreatic exocrine insufficiency (p<0.0001), a higher fat mass (p<0.0001), and lower glucose area under the curve (AUC) (p=0.027). The F508del/L206W subgroup had significantly higher insulin secretion (AUC; p=0.027) and body mass index (p<0.001). Pulmonary function (FEV1) was significantly higher for the F508del/L206W subgroup (p<0.0001). Over a median of 7.37 years, the risk of developing CFRD in 141 patients was similar between groups. PwCF with heterozygous F508del/L206W and F508del/A455E tended to have pancreatic exocrine sufficiency, better nutritional status, improved pulmonary function and better diabetogenic indices, but this does not translate into lower risk of CF-related Diabetes.
    Language English
    Publishing date 2024-03-29
    Publishing country Brazil
    Document type Journal Article
    ZDB-ID 1445712-x
    ISSN 1678-4685 ; 1415-4757
    ISSN (online) 1678-4685
    ISSN 1415-4757
    DOI 10.1590/1678-4685-GMB-2023-0021
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 3079: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)

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  2. Article ; Online: New-onset Obesity After Lung Transplantation: Incidence, Risk Factors, and Clinical Outcomes.

    Jomphe, Valérie / Bélanger, Noémie / Beauchamp-Parent, Caroline / Poirier, Charles / Nasir, Basil S / Ferraro, Pasquale / Lands, Larry C / Mailhot, Geneviève

    Transplantation

    2022  Volume 106, Issue 11, Page(s) 2247–2255

    Abstract: Background: Lung transplant (LTx) recipients who gain weight after transplantation may experience an upward shift in body mass index (BMI) that places them in the obese category. The incidence, risk factors, and impact on metabolic health and mortality ... ...

    Abstract Background: Lung transplant (LTx) recipients who gain weight after transplantation may experience an upward shift in body mass index (BMI) that places them in the obese category. The incidence, risk factors, and impact on metabolic health and mortality of new-onset obesity have not been documented in the LTx setting.
    Methods: This single-center retrospective study included 564 LTx recipients. Individuals were stratified according to their BMI trajectories from pretransplant evaluation up to 10 y posttransplant. New-onset obesity was defined as a pretransplant BMI <30 kg/m 2 and posttransplant BMI >30 kg/m 2 . The incidence, risk factors, and posttransplant diabetes mellitus, metabolic syndrome, and mortality of recipients with new-onset obesity were compared with those of nonobese (BMI <30 kg/m 2 , pre/post-LTx), consistently obese (BMI >30 kg/m 2 , pre/post-LTx), and obese recipients with weight loss (BMI >30 kg/m 2 pre-LTx, BMI <30 kg/m 2 post-LTx).
    Results: We found that 14% of recipients developed obesity after transplantation. Overweight individuals (odds ratio [OR]: 9.01; 95% confidence interval [CI] [4.86-16.69]; P < 0.001) and candidates with chronic obstructive pulmonary disease (OR: 6.93; 95% CI [2.30-20.85]; P = 0.001) and other diagnoses (OR: 4.28; 95% CI [1.22-14.98]; P = 0.023) were at greater risk. Multivariable regression analysis showed that new-onset obesity was associated with a greater risk of metabolic syndrome (hazard ratio: 1.70; 95% CI [1.17-2.46]; P = 0.005), but not of posttransplant diabetes mellitus, than nonobesity. Recipients with new-onset obesity had a survival comparable to that of consistently obese individuals.
    Conclusions: A greater understanding of the multifaceted nature of post-LTx obesity may lead to interventions that are better tailored to the characteristics of these individuals.
    MeSH term(s) Humans ; Incidence ; Retrospective Studies ; Metabolic Syndrome/complications ; Obesity/complications ; Obesity/epidemiology ; Body Mass Index ; Lung Transplantation/adverse effects ; Risk Factors ; Diabetes Mellitus/diagnosis ; Diabetes Mellitus/epidemiology ; Diabetes Mellitus/etiology
    Language English
    Publishing date 2022-06-24
    Publishing country United States
    Document type Journal Article
    ZDB-ID 208424-7
    ISSN 1534-6080 ; 0041-1337
    ISSN (online) 1534-6080
    ISSN 0041-1337
    DOI 10.1097/TP.0000000000004222
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 488: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)
    Zs.MO 509: Show issues

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  3. Article ; Online: A glycosylated hemoglobin A1c above 6% (42 mmol/mol) is associated with a high risk of developing Cystic Fibrosis-Related Diabetes and a lower probability of weight gain in both adults and children with Cystic Fibrosis.

    Potter, Kathryn J / Racine, Florence / Bonhoure, Anne / Boudreau, Valérie / Bélanger, Noémie / Coriati, Adèle / Shohoudi, Azadeh / Lavoie, Annick / Senior, Peter A / Mailhot, Geneviève / Rabasa-Lhoret, Rémi

    Diabetes & metabolism

    2023  Volume 49, Issue 4, Page(s) 101455

    Abstract: Objectives: The classical glycosylated hemoglobin A1c threshold of 6.5% is an insensitive screening test for cystic fibrosis-related diabetes (CFRD). We sought to identify CF-specific A1C thresholds associated with 1) risk of progression to CFRD and 2) ... ...

    Abstract Objectives: The classical glycosylated hemoglobin A1c threshold of 6.5% is an insensitive screening test for cystic fibrosis-related diabetes (CFRD). We sought to identify CF-specific A1C thresholds associated with 1) risk of progression to CFRD and 2) changes in body mass index (BMI) and forced expiratory volume (FEV1).
    Methods: We studied the cross sectional and longitudinal associations between A1c, BMI, and FEV1 in 2 cohorts of 223 children (followed for up to 8 years) and 289 adults (followed for a mean of 7.5 ± 4.3 years) with CF but without diabetes at baseline and undergoing regular assessments including Oral Glucose Tolerance Test (OGTT).
    Results: For the onset of OGTT-defined CFRD optimal A1c threshold was 5.9% in adults (sensitivity: 67% and specificity: 71%) and 5.7% for children (sensitivity: 60% and specificity: 47%). Kaplan-Meier analysis of progression to CFRD according to baseline A1C showed increased the risk of developing CFRD for A1c ≥ 6.0% in adults (P = 0.002) and ≥ 5.5% in children (p = 0.012). Temporal changes in BMI and FEV1 according to baseline A1C in adults were assessed with a linear mixed-effect model, BMI significantly increased over time in subjects with a baseline A1c < 6%, but those with a A1C ≥ 6.0% gained significantly less weight over time (P = 0.05). There was no difference in FEV1 according to baseline A1c category.
    Conclusion: An A1C above 6% may be associated with a high risk of developing CFRD and a lower probability of weight gain in both adults and children with CF.
    MeSH term(s) Humans ; Adult ; Child ; Glycated Hemoglobin ; Cystic Fibrosis/complications ; Cystic Fibrosis/epidemiology ; Cystic Fibrosis/diagnosis ; Blood Glucose ; Cross-Sectional Studies ; Diabetes Mellitus/epidemiology ; Diabetes Mellitus/diagnosis ; Weight Gain ; Glucose Intolerance/complications
    Chemical Substances Glycated Hemoglobin ; Blood Glucose
    Language English
    Publishing date 2023-06-02
    Publishing country France
    Document type Journal Article
    ZDB-ID 1315751-6
    ISSN 1878-1780 ; 1262-3636 ; 0338-1684
    ISSN (online) 1878-1780
    ISSN 1262-3636 ; 0338-1684
    DOI 10.1016/j.diabet.2023.101455
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1267: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  4. Article ; Online: Loss of Mitochondrial Function Impairs Lysosomes.

    Demers-Lamarche, Julie / Guillebaud, Gérald / Tlili, Mouna / Todkar, Kiran / Bélanger, Noémie / Grondin, Martine / Nguyen, Angela P / Michel, Jennifer / Germain, Marc

    The Journal of biological chemistry

    2016  Volume 291, Issue 19, Page(s) 10263–10276

    Abstract: Alterations in mitochondrial function, as observed in neurodegenerative diseases, lead to disrupted energy metabolism and production of damaging reactive oxygen species. Here, we demonstrate that mitochondrial dysfunction also disrupts the structure and ... ...

    Abstract Alterations in mitochondrial function, as observed in neurodegenerative diseases, lead to disrupted energy metabolism and production of damaging reactive oxygen species. Here, we demonstrate that mitochondrial dysfunction also disrupts the structure and function of lysosomes, the main degradation and recycling organelle. Specifically, inhibition of mitochondrial function, following deletion of the mitochondrial protein AIF, OPA1, or PINK1, as well as chemical inhibition of the electron transport chain, impaired lysosomal activity and caused the appearance of large lysosomal vacuoles. Importantly, our results show that lysosomal impairment is dependent on reactive oxygen species. Given that alterations in both mitochondrial function and lysosomal activity are key features of neurodegenerative diseases, this work provides important insights into the etiology of neurodegenerative diseases.
    MeSH term(s) Animals ; Apoptosis Inducing Factor/genetics ; Apoptosis Inducing Factor/metabolism ; Cell Line ; Electron Transport Chain Complex Proteins/genetics ; Electron Transport Chain Complex Proteins/metabolism ; GTP Phosphohydrolases/genetics ; GTP Phosphohydrolases/metabolism ; Lysosomes/genetics ; Lysosomes/metabolism ; Lysosomes/pathology ; Mice ; Mice, Knockout ; Mitochondria/genetics ; Mitochondria/metabolism ; Mitochondria/pathology ; Neurodegenerative Diseases/genetics ; Neurodegenerative Diseases/metabolism ; Neurodegenerative Diseases/pathology ; Protein Kinases/genetics ; Protein Kinases/metabolism ; Reactive Oxygen Species/metabolism
    Chemical Substances Apoptosis Inducing Factor ; Electron Transport Chain Complex Proteins ; AIFM1 protein, mouse ; Reactive Oxygen Species ; Protein Kinases (EC 2.7.-) ; PTEN-induced putative kinase (EC 2.7.11.1) ; GTP Phosphohydrolases (EC 3.6.1.-) ; Opa1 protein, mouse (EC 3.6.1.-)
    Language English
    Publishing date 2016-03-17
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2997-x
    ISSN 1083-351X ; 0021-9258
    ISSN (online) 1083-351X
    ISSN 0021-9258
    DOI 10.1074/jbc.M115.695825
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Uc I Zs.108: Show issues Location:
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    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

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