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Article ; Online: Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges.

Mailhot, Geneviève / Denis, Marie-Hélène / Beauchamp-Parent, Caroline / Jomphe, Valérie

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

2023 Volume 36, Issue 5, Page(s) 1675–1691

Abstract: Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding for the ion channel cystic fibrosis transmembrane conductance regulator (CFTR). The management of CF disease has evolved in recent decades from treating downstream disease ...

Abstract	Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding for the ion channel cystic fibrosis transmembrane conductance regulator (CFTR). The management of CF disease has evolved in recent decades from treating downstream disease manifestations affecting the airways, the lungs and the gastrointestinal system to addressing the CFTR gene defect. The advent of CFTR modulators, which correct the functionality of the defective CFTR, contributes to reshaping the landscape of CF demographics, prognosis and therapies, including nutritional management. A spectrum of clinical manifestations is emerging within the same patient population where undernutrition and nutritional deficiencies coexist with excessive weight gain and metabolic derangements. Such contrasting presentations challenge current practices, require adjustments to traditional approaches, and involve more individualised interventions. This narrative review examines the current state of knowledge on the nutritional management of people living with cystic fibrosis from early life to adulthood in the era of CFTR modulation.
MeSH term(s)	Humans ; Cystic Fibrosis/complications ; Cystic Fibrosis/genetics ; Cystic Fibrosis/therapy ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Cystic Fibrosis Transmembrane Conductance Regulator/therapeutic use ; Prognosis ; Lung ; Precision Medicine
Chemical Substances	Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6)
Language	English
Publishing date	2023-07-28
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.13214
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Article ; Online: Vitamin D and Immunity in Infants and Children.

Mailhot, Geneviève / White, John H

Nutrients

2020 Volume 12, Issue 5

Abstract: The last couple of decades have seen an explosion in our interest and understanding of the role of vitamin D in the regulation of immunity. At the molecular level, the hormonal form of vitamin D signals through the nuclear vitamin D receptor (VDR), a ... ...

Abstract	The last couple of decades have seen an explosion in our interest and understanding of the role of vitamin D in the regulation of immunity. At the molecular level, the hormonal form of vitamin D signals through the nuclear vitamin D receptor (VDR), a ligand-regulated transcription factor. The VDR and vitamin D metabolic enzymes are expressed throughout the innate and adaptive arms of the immune system. The advent of genome-wide approaches to gene expression profiling have led to the identification of numerous VDR-regulated genes implicated in the regulation of innate and adaptive immunity. The molecular data infer that vitamin D signaling should boost innate immunity against pathogens of bacterial or viral origin. Vitamin D signaling also suppresses inflammatory immune responses that underlie autoimmunity and regulate allergic responses. These findings have been bolstered by clinical studies linking vitamin D deficiency to increased rates of infections, autoimmunity, and allergies. Our goals here are to provide an overview of the molecular basis for immune system regulation and to survey the clinical data from pediatric populations, using randomized placebo-controlled trials and meta-analyses where possible, linking vitamin D deficiency to increased rates of infections, autoimmune conditions, and allergies, and addressing the impact of supplementation on these conditions.
MeSH term(s)	Adaptive Immunity ; Age Factors ; Autoimmune Diseases/etiology ; Autoimmunity ; Child ; Child Nutritional Physiological Phenomena/immunology ; Child, Preschool ; Communicable Diseases/etiology ; Dietary Supplements ; Female ; Humans ; Hypersensitivity/etiology ; Immunity, Innate ; Immunologic Factors ; Infant ; Male ; Receptors, Calcitriol/metabolism ; Receptors, Calcitriol/physiology ; Signal Transduction/physiology ; Vitamin D/metabolism ; Vitamin D/pharmacology ; Vitamin D/physiology ; Vitamin D Deficiency/complications ; Vitamin D Deficiency/immunology
Chemical Substances	Immunologic Factors ; Receptors, Calcitriol ; VDR protein, human ; Vitamin D (1406-16-2)
Keywords	covid19
Language	English
Publishing date	2020-04-27
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2518386-2
ISSN	2072-6643 ; 2072-6643
ISSN (online)	2072-6643
ISSN	2072-6643
DOI	10.3390/nu12051233
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Article: Vitamin D and Immunity in Infants and Children

Mailhot, Geneviève / White, John H

Nutrients. 2020 Apr. 27, v. 12, no. 5

2020

Abstract	The last couple of decades have seen an explosion in our interest and understanding of the role of vitamin D in the regulation of immunity. At the molecular level, the hormonal form of vitamin D signals through the nuclear vitamin D receptor (VDR), a ligand-regulated transcription factor. The VDR and vitamin D metabolic enzymes are expressed throughout the innate and adaptive arms of the immune system. The advent of genome-wide approaches to gene expression profiling have led to the identification of numerous VDR-regulated genes implicated in the regulation of innate and adaptive immunity. The molecular data infer that vitamin D signaling should boost innate immunity against pathogens of bacterial or viral origin. Vitamin D signaling also suppresses inflammatory immune responses that underlie autoimmunity and regulate allergic responses. These findings have been bolstered by clinical studies linking vitamin D deficiency to increased rates of infections, autoimmunity, and allergies. Our goals here are to provide an overview of the molecular basis for immune system regulation and to survey the clinical data from pediatric populations, using randomized placebo-controlled trials and meta-analyses where possible, linking vitamin D deficiency to increased rates of infections, autoimmune conditions, and allergies, and addressing the impact of supplementation on these conditions.
Keywords	adaptive immunity ; autoimmunity ; calcitriol receptors ; children ; clinical trials ; enzymes ; gene expression regulation ; genes ; hypersensitivity ; immune response ; immune system ; infants ; innate immunity ; meta-analysis ; nutrients ; objectives ; pathogens ; population ; surveys ; transcription factors ; vitamin D ; vitamin D deficiency
Language	English
Dates of publication	2020-0427
Publishing place	Multidisciplinary Digital Publishing Institute
Document type	Article
Note	NAL-light
ZDB-ID	2518386-2
ISSN	2072-6643
ISSN	2072-6643
DOI	10.3390/nu12051233
Database	NAL-Catalogue (AGRICOLA)

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Article ; Online: Vitamin D bioavailability in cystic fibrosis: a cause for concern?

Mailhot, Geneviève

Nutrition reviews

2012 Volume 70, Issue 5, Page(s) 280–293

Abstract: Despite the inclusion of extra vitamin D in their regimen of fat-soluble vitamin supplementation, cystic fibrosis patients remain chronically depleted of vitamin D. The persistence of suboptimal vitamin D status is often blamed on the maldigestion and ... ...

Abstract	Despite the inclusion of extra vitamin D in their regimen of fat-soluble vitamin supplementation, cystic fibrosis patients remain chronically depleted of vitamin D. The persistence of suboptimal vitamin D status is often blamed on the maldigestion and malabsorption of fat. However, the mitigated success of recent clinical trials with high-dose vitamin D supplementation suggests that vitamin D bioavailability might be impaired in these patients. Given the growing understanding of the importance of this vitamin in the regulation of multiple biological functions beyond skeletal health, the present review analyzes the current literature by addressing each step of vitamin D metabolism and action in the context of this life-limiting pathology. In addition, it highlights the importance of vitamin D in relation to organs and or conditions affected by cystic fibrosis.
MeSH term(s)	Biological Availability ; Cystic Fibrosis/complications ; Cystic Fibrosis/metabolism ; Humans ; Intestinal Absorption ; Vitamin D/metabolism ; Vitamin D/pharmacokinetics ; Vitamin D Deficiency/prevention & control ; Vitamins/metabolism ; Vitamins/pharmacokinetics
Chemical Substances	Vitamins ; Vitamin D (1406-16-2)
Language	English
Publishing date	2012-05
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	82067-2
ISSN	1753-4887 ; 0029-6643
ISSN (online)	1753-4887
ISSN	0029-6643
DOI	10.1111/j.1753-4887.2012.00471.x
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Article: Vitamin D and Immunity in Infants and Children

Mailhot, Geneviève / White, John H

Nutrients

Abstract	The last couple of decades have seen an explosion in our interest and understanding of the role of vitamin D in the regulation of immunity. At the molecular level, the hormonal form of vitamin D signals through the nuclear vitamin D receptor (VDR), a ligand-regulated transcription factor. The VDR and vitamin D metabolic enzymes are expressed throughout the innate and adaptive arms of the immune system. The advent of genome-wide approaches to gene expression profiling have led to the identification of numerous VDR-regulated genes implicated in the regulation of innate and adaptive immunity. The molecular data infer that vitamin D signaling should boost innate immunity against pathogens of bacterial or viral origin. Vitamin D signaling also suppresses inflammatory immune responses that underlie autoimmunity and regulate allergic responses. These findings have been bolstered by clinical studies linking vitamin D deficiency to increased rates of infections, autoimmunity, and allergies. Our goals here are to provide an overview of the molecular basis for immune system regulation and to survey the clinical data from pediatric populations, using randomized placebo-controlled trials and meta-analyses where possible, linking vitamin D deficiency to increased rates of infections, autoimmune conditions, and allergies, and addressing the impact of supplementation on these conditions.
Keywords	covid19
Publisher	WHO
Document type	Article
Note	WHO #Covidence: #828212
Database	COVID19

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Article: Vitamin D bioavailability in cystic fibrosis: a cause for concern?

Mailhot, Geneviève

Nutrition reviews. 2012 May, v. 70, no. 5

2012

Abstract: Despite the inclusion of extra vitamin D in their regimen of fat‐soluble vitamin supplementation, cystic fibrosis patients remain chronically depleted of vitamin D. The persistence of suboptimal vitamin D status is often blamed on the maldigestion and ... ...

Abstract	Despite the inclusion of extra vitamin D in their regimen of fat‐soluble vitamin supplementation, cystic fibrosis patients remain chronically depleted of vitamin D. The persistence of suboptimal vitamin D status is often blamed on the maldigestion and malabsorption of fat. However, the mitigated success of recent clinical trials with high‐dose vitamin D supplementation suggests that vitamin D bioavailability might be impaired in these patients. Given the growing understanding of the importance of this vitamin in the regulation of multiple biological functions beyond skeletal health, the present review analyzes the current literature by addressing each step of vitamin D metabolism and action in the context of this life‐limiting pathology. In addition, it highlights the importance of vitamin D in relation to organs and or conditions affected by cystic fibrosis.
Keywords	bioavailability ; clinical trials ; cystic fibrosis ; lipids ; malabsorption ; patients ; vitamin D ; vitamin metabolism
Language	English
Dates of publication	2012-05
Size	p. 280-293.
Publishing place	Blackwell Publishing Inc
Document type	Article
ZDB-ID	82067-2
ISSN	1753-4887 ; 0029-6643
ISSN (online)	1753-4887
ISSN	0029-6643
DOI	10.1111/j.1753-4887.2012.00471.x
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Article: Effectiveness of two vitamin D

Mailhot, Geneviève / Lamarche, Josée / Gagnon, Dany H

Spinal cord series and cases

2018 Volume 4, Page(s) 96

Abstract: Study design: Prospective case series.: Objectives: To assess the effectiveness and safety of two vitamin D: Setting: Publicly-funded intensive inpatient rehabilitation center, Montreal, Canada.: Methods: Thirty adults with recent SCI complete ... ...

Abstract	Study design: Prospective case series. Objectives: To assess the effectiveness and safety of two vitamin D Setting: Publicly-funded intensive inpatient rehabilitation center, Montreal, Canada. Methods: Thirty adults with recent SCI complete or incomplete sensorimotor impairments were recruited upon admission from designated regional SCI trauma centers. Participants with serum 25OHD ≤ 30 nmol/L were given 10,000 IU of weekly and 1000 IU of daily vitamin D Results: At baseline, 34 and 66% of participants had serum 25OHD < 30 and >30 nmol/L. Both protocols induced a rise in serum 25OHD with a greater increase in the HD vs. LD regimen (31.4 [95% CI: 16.7, 46.0] vs. 11.7 nmol/L [95% CI: 2.2, 21.2]). None of the participants given the HD remained vitamin D deficient, but only one achieved vitamin D sufficiency. Nearly all individuals on the LD regimen remained vitamin D insufficient with only two reaching vitamin D sufficiency. No adverse effects were observed over the course of the supplementation. Conclusions: Although 1000 IU of daily vitamin D
Language	English
Publishing date	2018-10-29
Publishing country	England
Document type	Journal Article
ISSN	2058-6124
ISSN	2058-6124
DOI	10.1038/s41394-018-0129-9
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Article ; Online: Occult Pneumothorax Identification on Extended Focused Assessment with Sonography for Trauma Examination in Children.

Malek, Daniel / Santillanes, Genevieve / Hsiao, Victor / Mailhot, Thomas / Claudius, Ilene

Pediatric emergency care

2020 Volume 37, Issue 10, Page(s) e599–e601

Abstract: Objective: Occult pneumothoraces (OPTXs) are defined by air within the pleural space that is not visible on conventional chest radiographs (CXR). The aim of this study was to understand how frequently the Extended Focused Assessment with Sonography for ... ...

Abstract	Objective: Occult pneumothoraces (OPTXs) are defined by air within the pleural space that is not visible on conventional chest radiographs (CXR). The aim of this study was to understand how frequently the Extended Focused Assessment with Sonography for Trauma (eFAST) examination identifies occult PTX in a pediatric blunt trauma population as compared with a criterion standard of chest computed tomography (CCT). Methods: This study is a secondary analysis of blunt trauma patients younger than 18 years who underwent CCT at Los Angeles County +USC Medical Center Emergency Department from October 2015 to April 2017. The eFAST examination was performed and documented by an emergency medicine resident with attending oversight or by an emergency medicine attending for each trauma. The eFAST results were reviewed for patients diagnosed with small or trace pneumothoraces identified on CCT. Results: Of 168 pediatric trauma patients undergoing CCT, 16 had OPTXs not seen on CXR and 4 patients had a small/trace PTX without a corresponding CXR performed. None were identified on eFAST. Conclusions: Although the sample size in this data set was small, our eFAST examinations identified none of 16 proven and 4 presumed OPTXs. The standard eFAST examination performed poorly in the detection of OPTXs in this single-center study of pediatric blunt trauma victims.
MeSH term(s)	Child ; Focused Assessment with Sonography for Trauma ; Humans ; Pneumothorax/diagnostic imaging ; Pneumothorax/etiology ; Sensitivity and Specificity ; Thoracic Injuries/complications ; Thoracic Injuries/diagnostic imaging ; Wounds, Nonpenetrating/complications ; Wounds, Nonpenetrating/diagnostic imaging
Language	English
Publishing date	2020-11-26
Publishing country	United States
Document type	Journal Article
ZDB-ID	632588-9
ISSN	1535-1815 ; 0749-5161
ISSN (online)	1535-1815
ISSN	0749-5161
DOI	10.1097/PEC.0000000000002303
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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
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Article ; Online: CFTR deletion affects mouse osteoblasts in a gender-specific manner.

Orlando, Valérie / Morin, Geneviève / Laffont, Alisson / Lénart, Déborah / Solórzano Barrera, Carolina / Mustafy, Tanvir / Sankhe, Safiétou / Villemure, Isabelle / Mailhot, Geneviève

Journal of cellular physiology

2020 Volume 235, Issue 10, Page(s) 6736–6753

Abstract: Advancements in research and care have contributed to increase life expectancy of individuals with cystic fibrosis (CF). With increasing age comes a greater likelihood of developing CF bone disease, a comorbidity characterized by a low bone mass and ... ...

Abstract	Advancements in research and care have contributed to increase life expectancy of individuals with cystic fibrosis (CF). With increasing age comes a greater likelihood of developing CF bone disease, a comorbidity characterized by a low bone mass and impaired bone quality, which displays gender differences in severity. However, pathophysiological mechanisms underlying this gender difference have never been thoroughly investigated. We used bone marrow-derived osteoblasts and osteoclasts from Cftr
MeSH term(s)	Animals ; Bone and Bones/metabolism ; Bone and Bones/physiology ; Cell Differentiation/physiology ; Cystic Fibrosis/metabolism ; Cystic Fibrosis/pathology ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Disease Models, Animal ; Female ; Male ; Mice ; Mice, Inbred BALB C ; Mice, Knockout ; Osteoblasts/metabolism ; Osteoblasts/physiology ; Osteoclasts/metabolism ; Osteoclasts/physiology ; Osteogenesis/physiology ; Signal Transduction/physiology
Chemical Substances	Cftr protein, mouse ; Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6)
Language	English
Publishing date	2020-01-27
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	3116-1
ISSN	1097-4652 ; 0021-9541
ISSN (online)	1097-4652
ISSN	0021-9541
DOI	10.1002/jcp.29568
Database	MEDical Literature Analysis and Retrieval System OnLINE

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