Nutrients

Research

15 pages, 686 KiB

Open AccessArticle

Vitamin Status in Children with Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutation

by Paulina Wysocka-Wojakiewicz, Halina Woś, Tomasz Wielkoszyński, Aleksandra Pyziak-Skupień and Urszula Grzybowska-Chlebowczyk

Nutrients 2022, 14(21), 4661; https://doi.org/10.3390/nu14214661 - 4 Nov 2022

Cited by 1 | Viewed by 1484

Abstract

Background: The issue of vitamin metabolism in children with cystic fibrosis screen positive, inconclusive diagnosis (CFSPID) is not well known. The aim of this study was to determine the status of vitamins A, D, E, and C in the blood of a group [...] Read more.

Background: The issue of vitamin metabolism in children with cystic fibrosis screen positive, inconclusive diagnosis (CFSPID) is not well known. The aim of this study was to determine the status of vitamins A, D, E, and C in the blood of a group of children with CFSPID. Material and Methods: A total of 89 children were enrolled in the study (Me: 3.6 years, 52.8% boys), as follows: 28 with CFSPID, 31 with CF (cystic fibrosis), and 30 HC (healthy children). Their blood concentrations of vitamins A, D, E, and C, and their dietary intake of these vitamins were analysed in the study groups on the basis of a three-day food diary. Results: The patients with CFSPID had significantly higher serum vitamin D (p = 0.01) and E (p = 0.04) concentrations, compared to the children with CF. None of the children with CFSPID revealed vitamin A or E deficiencies. Patients with CF had been consuming significantly higher vitamin D and E amounts (p = 0.01). The vitamin concentrations did not depend either on the pancreatic/liver function or on anthropometric parameters. In total, 32.14% of patients with CF did not cover the baseline recommended calorie intake, and 53.6% and 36% did not take the recommended vitamin E and vitamin A intake, respectively. Conclusion: Children with CF and CFSPID did not fully cover the dietary recommendations for vitamin supply, but vitamin deficiency was found only in CF. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

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13 pages, 1029 KiB

Open AccessArticle

α-Tocopherol Pharmacokinetics in Adults with Cystic Fibrosis: Benefits of Supplemental Vitamin C Administration

by Maret G. Traber, Scott W. Leonard, Vihas T. Vasu, Brian M. Morrissey, Huangshu (John) Lei, Jeffrey Atkinson and Carroll E. Cross

Nutrients 2022, 14(18), 3717; https://doi.org/10.3390/nu14183717 - 9 Sep 2022

Cited by 6 | Viewed by 5240

Abstract

Background: Numerous abnormalities in cystic fibrosis (CF) could influence tocopherol absorption, transportation, storage, metabolism and excretion. We hypothesized that the oxidative distress due to inflammation in CF increases vitamin E utilization, which could be positively influenced by supplemental vitamin C administration. Methods: Immediately [...] Read more.

Background: Numerous abnormalities in cystic fibrosis (CF) could influence tocopherol absorption, transportation, storage, metabolism and excretion. We hypothesized that the oxidative distress due to inflammation in CF increases vitamin E utilization, which could be positively influenced by supplemental vitamin C administration. Methods: Immediately before and after receiving vitamin C (500 mg) twice daily for 3.5 weeks, adult CF patients (n = 6) with moderately advanced respiratory tract (RT) disease consumed a standardized breakfast with 30% fat and a capsule containing 50 mg each hexadeuterium (d₆)-α- and dideuterium (d₂)-γ-tocopheryl acetates. Blood samples were taken frequently up to 72 h; plasma tocopherol pharmacokinetics were determined. During both trials, d₆-α- and d₂-γ-tocopherols were similarly absorbed and reached similar maximal plasma concentrations ~18–20 h. As predicted, during vitamin C supplementation, the rates of plasma d₆-α-tocopherol decline were significantly slower. Conclusions: The vitamin C-induced decrease in the plasma disappearance rate of α-tocopherol suggests that vitamin C recycled α-tocopherol, thereby augmenting its concentrations. We conclude that some attention should be paid to plasma ascorbic acid concentrations in CF patients, particularly to those individuals with more advanced RT inflammatory disease and including those with severe exacerbations. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

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14 pages, 1157 KiB

Open AccessArticle

Usefulness of Muscle Ultrasonography in the Nutritional Assessment of Adult Patients with Cystic Fibrosis

by Francisco José Sánchez-Torralvo, Nuria Porras, Ignacio Ruiz-García, Cristina Maldonado-Araque, María García-Olivares, María Victoria Girón, Montserrat Gonzalo-Marín, Casilda Olveira and Gabriel Olveira

Nutrients 2022, 14(16), 3377; https://doi.org/10.3390/nu14163377 - 17 Aug 2022

Cited by 8 | Viewed by 2469

Abstract

Background: Muscle ultrasonography of the quadriceps rectus femoris (QRF) is a technique on the rise in the assessment of muscle mass in application of nutritional assessment. The aim of the present study is to assess the usefulness of muscle ultrasonography in patients with [...] Read more.

Background: Muscle ultrasonography of the quadriceps rectus femoris (QRF) is a technique on the rise in the assessment of muscle mass in application of nutritional assessment. The aim of the present study is to assess the usefulness of muscle ultrasonography in patients with cystic fibrosis, comparing the results with other body composition techniques such as anthropometry, bioelectrical impedance analysis (BIA), dual-energy X-ray absorptiometry (DXA), and handgrip strength (HGS). At the same time, we intend to assess the possible association with the nutritional and respiratory status. Methods: This was a prospective observational study in adult patients with cystic fibrosis in a clinically stable situation. Muscle ultrasonography of the QRF was performed, and the results were compared with other measures of body composition: anthropometry, BIA, and DXA. HGS was used to assess muscle function. Respiratory parameters were collected, and nutritional status was assessed using Global Leadership Initiative on Malnutrition (GLIM) criteria. Results: A total of 48 patients were included, with a mean age of 34.1 ± 8.8 years. In total, 24 patients were men, and 24 patients were women. Mean BMI was 22.5 ± 3.8 kg/m². Mean muscular area rectus anterior (MARA) was 4.09 ± 1.5 cm², and mean muscular circumference rectus was 8.86 ± 1.61 cm. A positive correlation was observed between the MARA and fat-free mass index (FFMI) determined by anthropometry (r = 0.747; p < 0.001), BIA (r = 0.780; p < 0.001), and DXA (r = 0.678; p < 0.001), as well as muscle function (HGS: r = 0.790; p < 0.001) and respiratory parameters (FEV1; r = 0.445, p = 0.005; FVC: r = 0.376, p = 0.02; FEV1/FVC: r = 0.344, p = 0.037). A total of 25 patients (52.1%) were diagnosed with malnutrition according to GLIM criteria. Differences were observed when comparing the MARA based on the diagnosis of malnutrition (4.75 ± 1.65 cm² in normo-nourished vs. 3.37 ± 1.04 in malnourished; p = 0.014). Conclusions: In adults with cystic fibrosis, the measurements collected by muscle ultrasound of the QRF correlate adequately with body composition techniques such as anthropometry, BIA, DXA, and handgrip strength. Muscle ultrasound measurements, particularly the MARA, are related to the nutritional status and respiratory function of these patients. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

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9 pages, 240 KiB

Open AccessArticle

Experiences and Perspectives of Individuals with Cystic Fibrosis and Their Families Related to Food Insecurity

by Montserrat A. Corbera-Hincapie, Samar E. Atteih, Olivia M. Stransky, Daniel J. Weiner, Iris M. Yann and Traci M. Kazmerski

Nutrients 2022, 14(13), 2573; https://doi.org/10.3390/nu14132573 - 21 Jun 2022

Cited by 9 | Viewed by 2200

Abstract

Food insecurity (FI) rates among people with cystic fibrosis (CF) are significantly higher than in the general US population. This study explored the experiences and perceptions of adults and parents of children with CF surrounding FI. We recruited parents of children with CF [...] Read more.

Food insecurity (FI) rates among people with cystic fibrosis (CF) are significantly higher than in the general US population. This study explored the experiences and perceptions of adults and parents of children with CF surrounding FI. We recruited parents of children with CF ages 0–18 years and adults with CF ages 18 years and older from a large, accredited U.S. CF care center and the Cystic Fibrosis Foundation Community Voice to participate in a qualitative study using semi-structured telephone interviews to explore experiences and preferences related to food insecurity. Two coders independently reviewed each transcript to apply the codebook and identify any emerging codes using an ongoing, iterative process to identify central themes. We interviewed 20 participants (six adults with CF and 14 parents of children with CF) and identified five major themes: (1) FI in CF is influenced by a variety of factors, ranging from nutritional demands to competing financial barriers; (2) FI impacts CF health outcomes; (3) Open patient-provider communication around FI is vital; (4) FI screening and discussions should be normalized in CF care; (5) Comprehensive FI resources are vital. FI is an important topic that should routinely be addressed with the CF care team to destigmatize and encourage individuals to be more forthcoming about their FI status. Results from this study will inform future larger investigations on the impact of FI on CF health and aid in the design and planning of targeted interventions and advocacy efforts. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

9 pages, 250 KiB

Open AccessArticle

Utilization of the Healthy Eating Index in Cystic Fibrosis

by Rosara Milstein Bass, Alyssa Tindall and Saba Sheikh

Nutrients 2022, 14(4), 834; https://doi.org/10.3390/nu14040834 - 16 Feb 2022

Cited by 6 | Viewed by 4439

Abstract

(1) Background: Malnutrition has been a hallmark of cystic fibrosis (CF) for some time, and improved nutritional status is associated with improved outcomes. While individuals with CF historically required higher caloric intake than the general population, new CF therapies and improved health in [...] Read more.

(1) Background: Malnutrition has been a hallmark of cystic fibrosis (CF) for some time, and improved nutritional status is associated with improved outcomes. While individuals with CF historically required higher caloric intake than the general population, new CF therapies and improved health in this population suggest decreased metabolic demand and prevalence of overweight and obesity have increased. This study aimed to (a) examine diet quality in a population of young adults with CF using the Healthy Eating Index, a measure of diet quality in accordance with the U.S. Dietary Guidelines for Americans and (b) evaluate and describe how subcomponents of the HEI might apply to individuals with CF (2) Methods: 3-day dietary recalls from healthy adolescents and young adults with CF were obtained and scored based on the Healthy Eating Index (3) Results: Dietary recalls from 26 (14M/12F) adolescents and young adults with CF (ages 16–23), were obtained. Individuals with CF had significantly lower HEI scores than the general population and lower individual component scores for total vegetables, greens and beans, total fruits, whole fruits, total protein, seafood and plant protein and sodium (p values < 0.01 for all). (4) Conclusion: Dietary quality was poor in these healthy adolescents and young adults with CF. Given the increased prevalence of overweight and obesity in CF, updated dietary guidance is urgently needed for this population. The Healthy Eating Index may be a valuable tool for evaluating dietary quality in CF. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

11 pages, 465 KiB

Open AccessFeature PaperArticle

The Relationship between Body Composition, Dietary Intake, Physical Activity, and Pulmonary Status in Adolescents and Adults with Cystic Fibrosis

by Kevin J. Scully, Laura T. Jay, Steven Freedman, Gregory S. Sawicki, Ahmet Uluer, Joel S. Finkelstein and Melissa S. Putman

Nutrients 2022, 14(2), 310; https://doi.org/10.3390/nu14020310 - 12 Jan 2022

Cited by 11 | Viewed by 3637

Abstract

Measures of body fat and lean mass may better predict important clinical outcomes in patients with cystic fibrosis (CF) than body mass index (BMI). Little is known about how diet quality and exercise may impact body composition in these patients. Dual X-ray absorptiometry [...] Read more.

Measures of body fat and lean mass may better predict important clinical outcomes in patients with cystic fibrosis (CF) than body mass index (BMI). Little is known about how diet quality and exercise may impact body composition in these patients. Dual X-ray absorptiometry (DXA) body composition, 24-h dietary recall, and physical activity were assessed in a cross-sectional analysis of 38 adolescents and adults with CF and 19 age-, race-, and gender-matched healthy volunteers. Compared with the healthy volunteers, participants with CF had a lower appendicular lean mass index (ALMI), despite no observed difference in BMI, and their diets consisted of higher glycemic index foods with a greater proportion of calories from fat and a lower proportion of calories from protein. In participants with CF, pulmonary function positively correlated with measures of lean mass, particularly ALMI, and negatively correlated with multiple measures of body fat after controlling for age, gender, and BMI. Higher physical activity levels were associated with greater ALMI and lower body fat. In conclusion, body composition measures, particularly ALMI, may better predict key clinical outcomes in individuals with CF than BMI. Future longitudinal studies analyzing the effect of dietary intake and exercise on body composition and CF-specific clinical outcomes are needed. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

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8 pages, 387 KiB

Open AccessCommunication

Most Short Children with Cystic Fibrosis Do Not Catch Up by Adulthood

by Margaret P. Marks, Sonya L. Heltshe, Arthur Baines, Bonnie W. Ramsey, Lucas R. Hoffman and Michael S. Stalvey

Nutrients 2021, 13(12), 4414; https://doi.org/10.3390/nu13124414 - 10 Dec 2021

Cited by 8 | Viewed by 2569

Abstract

Poor linear growth is common in children with cystic fibrosis (CF) and predicts pulmonary status and mortality. Growth impairment develops in infancy, prior to pulmonary decline and despite aggressive nutritional measures. We hypothesized that growth restriction during early childhood in CF is associated [...] Read more.

Poor linear growth is common in children with cystic fibrosis (CF) and predicts pulmonary status and mortality. Growth impairment develops in infancy, prior to pulmonary decline and despite aggressive nutritional measures. We hypothesized that growth restriction during early childhood in CF is associated with reduced adult height. We used the Cystic Fibrosis Foundation (CFF) patient registry to identify CF adults between 2011 and 2015 (ages 18–19 y, n = 3655) and had height for age (HFA) records between ages 2 and 4 y. We found that only 26% CF adults were ≥median HFA and 25% were <10th percentile. Between 2 and 4 years, those with height < 10th percentile had increased odds of being <10th percentile in adulthood compared to children ≥ 10th percentile (OR = 7.7). Of HFA measured between the 10th and 25th percentiles at ages 2–4, 58% were <25th percentile as adults. Only 13% between the 10th and 25th percentile HFA at age 2–4 years were >50th percentile as adults. Maximum height between ages 2 and 4 highly correlated with adult height. These results demonstrate that low early childhood CF height correlates with height in adulthood. Since linear growth correlates with lung growth, identifying both risk factors and interventions for growth failure (nutritional support, confounders of clinical care, and potential endocrine involvement) could lead to improved overall health. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

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9 pages, 420 KiB

Open AccessArticle

Vitamin D Status and Risk of Cystic Fibrosis-Related Diabetes: A Retrospective Single Center Cohort Study

by Yiqing Peng, Malinda Wu, Jessica A. Alvarez and Vin Tangpricha

Nutrients 2021, 13(11), 4048; https://doi.org/10.3390/nu13114048 - 12 Nov 2021

Cited by 6 | Viewed by 2965

Abstract

Objective: Cystic fibrosis-related diabetes (CFRD) affects up to half of the people with cystic fibrosis (CF) by adulthood. CFRD is primarily caused by pancreatic dysfunction that leads to insufficient insulin release and/or insulin resistance. Exocrine pancreatic insufficiency in people with CF is associated [...] Read more.

Objective: Cystic fibrosis-related diabetes (CFRD) affects up to half of the people with cystic fibrosis (CF) by adulthood. CFRD is primarily caused by pancreatic dysfunction that leads to insufficient insulin release and/or insulin resistance. Exocrine pancreatic insufficiency in people with CF is associated with fat-soluble vitamin malabsorption, including vitamins A, D, E, and K. This study examined the relationship between vitamin D status, assessed by serum 25-hydroxyvitamin D (25(OH)D), and the development of CF-related diabetes (CFRD) in adults with CF. Methods: This was a retrospective cohort study of adults seen at a single CF center. The data were extracted from the electronic medical records and the Emory Clinical Data Warehouse, a data repository of health information from patients seen at Emory Healthcare. We collected age, race, the first recorded serum 25-hydroxyvitamin D (25(OH)D) concentration, body mass index (BMI), and onset of diabetes diagnosis. Log-rank (Mantel–Cox) tests were used to compare the relative risk of CFRD onset in the subjects with stratified vitamin D status and weight status. A sub-group analysis using chi-square tests assessed the independence between vitamin D deficiency and CFRD risk factors, including gender and CF mutation types (homozygous or heterozygous for F508del, or others). Unpaired t-tests were also used to compare the BMI values and serum 25(OH)D between the CF adults based on the CFRD development. Results: This study included 253 subjects with a mean age of 27.1 years (±9.0), a mean follow-up time period of 1917.1 (±1394.5) days, and a mean serum 25(OH)D concentration of 31.8 ng/mL (±14.0). The majority (52.6%) of the subjects developed CFRD during the study period. Vitamin D deficiency (defined as 25(OH)D < 20 ng/mL) was present in 25.3% of the subjects. Close to two thirds (64.1%) of the subjects with vitamin D deficiency developed CFRD during the study. Vitamin D deficiency increased the risk of developing CFRD (chi-square, p = 0.03) during the course of the study. The time to the onset of CFRD stratified by vitamin D status was also significant (25(OH)D < 20 ng/mL vs. 25(OH)D ≥ 20 ng/mL) (95% CI: 1.2, 2.7, p < 0.0078). Conclusion: Our findings support the hypothesis that adults with CF and vitamin D deficiency are at a higher risk of developing CFRD and are at risk for earlier CFRD onset. The maintenance of a serum 25(OH)D concentration above 20 ng/mL may decrease the risk of progression to CFRD. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

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11 pages, 3114 KiB

Open AccessArticle

Geospatial Analysis of Food Deserts and Their Impact on Health Outcomes in Children with Cystic Fibrosis

by Montserrat A. Corbera-Hincapie, Kristen S. Kurland, Mark R. Hincapie, Anthony Fabio, Daniel J. Weiner, Sandra C. Kim and Traci M. Kazmerski

Nutrients 2021, 13(11), 3996; https://doi.org/10.3390/nu13113996 - 10 Nov 2021

Cited by 5 | Viewed by 3952

Abstract

Food insecurity (FI) is defined as “the limited or uncertain access to adequate food.” One root cause of FI is living in a food desert. FI rates among people with cystic fibrosis (CF) are higher than the general United States (US) population. There [...] Read more.

Food insecurity (FI) is defined as “the limited or uncertain access to adequate food.” One root cause of FI is living in a food desert. FI rates among people with cystic fibrosis (CF) are higher than the general United States (US) population. There is limited data on the association between food deserts and CF health outcomes. We conducted a retrospective review of people with CF under 18 years of age at a single pediatric CF center from January to December 2019 using demographic information and CF health parameters. Using a Geographic Information System, we conducted a spatial overlay analysis at the census tract level using the 2015 Food Access Research Atlas to assess the association between food deserts and CF health outcomes. We used multivariate logistic regression analysis and adjusted for clinical covariates and demographic covariates, using the Child Opportunity Index (COI) to calculate odds ratios (OR) with confidence intervals (CI) for each health outcome. People with CF living in food deserts and the surrounding regions had lower body mass index/weight-for-length (OR 3.18, 95% CI: 1.01, 9.40, p ≤ 0.05 (food desert); OR 4.41, 95% CI: 1.60, 12.14, p ≤ 0.05 (600 ft buffer zone); OR 2.83, 95% CI: 1.18, 6.76, p ≤ 0.05 (1200 ft buffer zone)). Food deserts and their surrounding regions impact pediatric CF outcomes independent of COI. Providers should routinely screen for FI and proximity to food deserts. Interventions are essential to increase access to healthy and affordable food. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

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Review

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11 pages, 3013 KiB

Open AccessReview

Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis

by Peter N. Freswick, Elizabeth K. Reid and Maria R. Mascarenhas

Nutrients 2022, 14(7), 1341; https://doi.org/10.3390/nu14071341 - 23 Mar 2022

Cited by 17 | Viewed by 7803

Abstract

While typically considered a pulmonary disease, cystic fibrosis patients develop significant nutritional complications and comorbidities, especially those who are pancreatic insufficient. Clinicians must have a high suspicion for cystic fibrosis among patients with clinical symptoms of pancreatic insufficiency, and pancreatic enzymatic replacement therapy [...] Read more.

While typically considered a pulmonary disease, cystic fibrosis patients develop significant nutritional complications and comorbidities, especially those who are pancreatic insufficient. Clinicians must have a high suspicion for cystic fibrosis among patients with clinical symptoms of pancreatic insufficiency, and pancreatic enzymatic replacement therapy (PERT) must be urgently initiated. PERT presents a myriad of considerations for patients and their supporting dieticians and clinicians, including types of administration, therapy failures, and complications. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

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18 pages, 358 KiB

Open AccessEditor’s ChoiceReview

The Changing Landscape of Nutrition in Cystic Fibrosis: The Emergence of Overweight and Obesity

by Julianna Bailey, Stefanie Krick and Kevin R. Fontaine

Nutrients 2022, 14(6), 1216; https://doi.org/10.3390/nu14061216 - 13 Mar 2022

Cited by 30 | Viewed by 5490

Abstract

Cystic fibrosis has historically been characterized by malnutrition, and nutrition strategies have placed emphasis on weight gain due to its association with better pulmonary outcomes. As treatment for this disease has significantly improved, longevity has increased and overweight and obesity have emerged issues [...] Read more.

Cystic fibrosis has historically been characterized by malnutrition, and nutrition strategies have placed emphasis on weight gain due to its association with better pulmonary outcomes. As treatment for this disease has significantly improved, longevity has increased and overweight and obesity have emerged issues in this population. The effect of excess weight and adiposity on CF clinical outcomes is unknown but may produce similar health consequences and obesity-related diseases as those observed in the general population. This review examines the prevalence of overweight and obesity in CF, the medical and psychological impact, as well as the existing evidence for treatment in the general population and how this may be applied to people with CF. Clinicians should partner with individuals with CF and their families to provide a personalized, interdisciplinary approach that includes dietary modification, physical activity, and behavioral intervention. Additional research is needed to identify the optimal strategies for preventing and addressing overweight and obesity in CF. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

17 pages, 347 KiB

Open AccessReview

Understanding Cystic Fibrosis Comorbidities and Their Impact on Nutritional Management

by Dhiren Patel, Albert Shan, Stacy Mathews and Meghana Sathe

Nutrients 2022, 14(5), 1028; https://doi.org/10.3390/nu14051028 - 28 Feb 2022

Cited by 6 | Viewed by 4203

Abstract

Cystic fibrosis (CF) is a chronic, multisystem disease with multiple comorbidities that can significantly affect nutrition and quality of life. Maintaining nutritional adequacy can be challenging in people with cystic fibrosis and has been directly associated with suboptimal clinical outcomes. Comorbidities of CF [...] Read more.

Cystic fibrosis (CF) is a chronic, multisystem disease with multiple comorbidities that can significantly affect nutrition and quality of life. Maintaining nutritional adequacy can be challenging in people with cystic fibrosis and has been directly associated with suboptimal clinical outcomes. Comorbidities of CF can result in significantly decreased nutritional intake and intestinal absorption, as well as increased metabolic demands. It is crucial to utilize a multidisciplinary team with expertise in CF to optimize growth and nutrition, where patients with CF and their loved ones are placed in the center of the care model. Additionally, with the advent of highly effective modulators (HEMs), CF providers have begun to identify previously unrecognized nutritional issues, such as obesity. Here, we will review and summarize commonly encountered comorbidities and their nutritional impact on this unique population. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

27 pages, 1080 KiB

Open AccessReview

What Do We Know about the Microbiome in Cystic Fibrosis? Is There a Role for Probiotics and Prebiotics?

by Josie M. van Dorst, Rachel Y. Tam and Chee Y. Ooi

Nutrients 2022, 14(3), 480; https://doi.org/10.3390/nu14030480 - 22 Jan 2022

Cited by 24 | Viewed by 6679

Abstract

Cystic fibrosis (CF) is a life-shortening genetic disorder that affects the cystic fibrosis transmembrane conductance regulator (CFTR) protein. In the gastrointestinal (GI) tract, CFTR dysfunction results in low intestinal pH, thick and inspissated mucus, a lack of endogenous pancreatic enzymes, and reduced motility. [...] Read more.

Cystic fibrosis (CF) is a life-shortening genetic disorder that affects the cystic fibrosis transmembrane conductance regulator (CFTR) protein. In the gastrointestinal (GI) tract, CFTR dysfunction results in low intestinal pH, thick and inspissated mucus, a lack of endogenous pancreatic enzymes, and reduced motility. These mechanisms, combined with antibiotic therapies, drive GI inflammation and significant alteration of the GI microbiota (dysbiosis). Dysbiosis and inflammation are key factors in systemic inflammation and GI complications including malignancy. The following review examines the potential for probiotic and prebiotic therapies to provide clinical benefits through modulation of the microbiome. Evidence from randomised control trials suggest probiotics are likely to improve GI inflammation and reduce the incidence of CF pulmonary exacerbations. However, the highly variable, low-quality data is a barrier to the implementation of probiotics into routine CF care. Epidemiological studies and clinical trials support the potential of dietary fibre and prebiotic supplements to beneficially modulate the microbiome in gastrointestinal conditions. To date, limited evidence is available on their safety and efficacy in CF. Variable responses to probiotics and prebiotics highlight the need for personalised approaches that consider an individual’s underlying microbiota, diet, and existing medications against the backdrop of the complex nutritional needs in CF. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

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Other

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14 pages, 1300 KiB

Open AccessSystematic Review

Effects of Exercise on Nutritional Status in People with Cystic Fibrosis: A Systematic Review

by William B. Nicolson, Julianna Bailey, Najlaa Z. Alotaibi, Stefanie Krick and John D. Lowman

Nutrients 2022, 14(5), 933; https://doi.org/10.3390/nu14050933 - 22 Feb 2022

Cited by 4 | Viewed by 3133

Abstract

Background: Physical exercise is an important part of regular care for people with cystic fibrosis (CF). It is unknown whether such exercise has beneficial or detrimental effects on nutritional status (body composition). Thus, the objective of this review was to evaluate the effect [...] Read more.

Background: Physical exercise is an important part of regular care for people with cystic fibrosis (CF). It is unknown whether such exercise has beneficial or detrimental effects on nutritional status (body composition). Thus, the objective of this review was to evaluate the effect of exercise on measures of nutritional status in children and adults with CF. Methods: Standardized reporting guidelines for systematic reviews were followed and the protocol was prospectively registered. Multiple databases were utilized (e.g., PubMed, Scopus, and CINHAL). Two reviewers independently reviewed titles/abstracts and then the full text for selected studies. Results: In total, 924 articles were originally identified; data were extracted from 4 eligible studies. These four studies included only children; pulmonary function ranged from severe to normal, and the majority of participants were at or below their recommended weight. Exercise training did not worsen nutritional status in any study; two studies that included resistance exercise reported an increase in fat-free mass. Three of the four studies also reported increased aerobic capacity and/or muscle strength. Conclusions: Exercise training can produce positive physiologic changes in children with CF without impairing their nutritional status. In fact, resistance exercise can help improve body mass. Much less is known about how exercise may affect adults or those who are overweight. Full article

(This article belongs to the Special Issue Nutritional Management of Cystic Fibrosis)

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