Highlights from the nutrition guidelines for cystic fibrosis in Australia and New Zealand

Haak, Natalie van der; King, Sontoria D.; Crowder, Tory; Kench, Andrea; Painter, C.; Saxby, Nicole

doi:10.1016/j.jcf.2019.05.007

Cited by 42 publications

(41 citation statements)

References 19 publications

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“…Some patients may have the same energy requirements as healthy peers with no need for a high fat intake. The particular emphasis should be put on the qualitative aspect of the diet comprising, among others, the determination of the fatty acid profile [13,54,55].…”

Section: Discussionmentioning

confidence: 99%

Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis

Drzymała-Czyż

Krzyżanowska-Jankowska

Dziedzic

et al. 2021

Biomolecules

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Background: Several factors could lead to lipid disturbances observed in cystic fibrosis (CF). This study aimed to assess sterol homeostasis in CF and define potential exogenous and endogenous determinants of lipid dysregulation. Methods: The study involved 55 CF patients and 45 healthy subjects (HS). Sterol concentrations (μg/dL) were measured by gas chromatography/mass spectrometry. CF was characterised by lung function, pancreatic status, liver disease and diabetes coexistence, Pseudomonas aeruginosa colonisation and BMI. CFTR genotypes were classified as severe or other. Results: Campesterol and β-sitosterol concentrations were lower (p = 0.0028 and p < 0.0001, respectively) and lathosterol levels (reflecting endogenous cholesterol biosynthesis) were higher (p = 0.0016) in CF patients than in HS. Campesterol and β-sitosterol concentrations were lower in patients with a severe CFTR genotype, pancreatic insufficiency and lower pancreatic enzyme dose (lipase units/gram of fat). In multiple regression analyses, β-sitosterol and campesterol concentrations were predicted by genotype and pancreatic insufficiency, whereas cholesterol and its fractions were predicted by phytosterol concentrations, age, dose of pancreatic enzymes, nutritional status and genotype. Conclusions: Independent determinants of lipid status suggest that malabsorption and pancreatic enzyme supplementation play a significant role in sterol abnormalities. The measurement of campesterol and β-sitosterol concentrations in CF patients may serve for the assessment of the effectiveness of pancreatic enzyme replacement therapy and/or compliance, but further research is required.

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Section: Discussionmentioning

confidence: 99%

Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis

Drzymała-Czyż

Krzyżanowska-Jankowska

Dziedzic

et al. 2021

Biomolecules

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“…Dietary intake data were then compared to the Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand [ 27 ], which are considered as the most “complete” CF dietary guidelines in terms of CF-specific recommendations [ 28 ]. Participants were categorized according to those adhering and/or exceeding dietary recommendations, or not.…”

Section: Methodsmentioning

confidence: 99%

Adherence to Dietary Recommendations, Nutrient Intake Adequacy and Diet Quality among Pediatric Cystic Fibrosis Patients: Results from the GreeCF Study

et al. 2020

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Nutrition is an important component of cystic fibrosis (CF) therapy, with a high-fat diet being the cornerstone of treatment. However, adherence to the dietary recommendations for CF appears suboptimal and burdensome for most children and adolescents with CF, leading to malnutrition, inadequate growth, compromised lung function and increased risk for respiratory infections. A cross-sectional approach was deployed to examine the degree of adherence to the nutrition recommendations and diet quality among children with CF. A total of 76 children were recruited from Aghia Sophia’s Children Hospital, in Athens, Greece. In their majority, participants attained their ideal body weight, met the recommendations for energy and fat intake, exceeding the goal for saturated fatty acids consumption. Carbohydrate and fiber intake were suboptimal and most participants exhibited low or mediocre adherence to the Mediterranean diet prototype. It appears that despite the optimal adherence to the energy and fat recommendations, there is still room for improvement concerning diet quality and fiber intake.

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“…recommend dosing based on frequent assessments of growth and symptoms. 13,76 In cases of inadequate response to enzymes, other causes of malabsorption must be considered.…”

Section: Pancreatic Insufficiencymentioning

confidence: 99%

“…Growth should be evaluated in infants and children <24 months of age using the weight-for-length percentile, and attention should be focused on the longitudinal growth trajectory. 12,13,76 Guidelines vary on frequency of assessment, from monthly to every 3 months in the first year of life.…”

Section: Nutrition Considerations By Agementioning

confidence: 99%

“…This increased risk is supported by the observation that patients with CF who are PS may be deficient in one or more fat-soluble vitamins, and PERT use alone does not correct deficiencies. 76,126,127 Additional conditions associated with CF such as liver disease or short bowel syndrome can further predispose a patient to malabsorption and deficiency. Deficiencies may present as early as 4 weeks of age.…”

Section: Fat-soluble Vitaminsmentioning

confidence: 99%

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Growth and Nutrition in Cystic Fibrosis

Brownell

Bashaw

Stallings

2019

Semin Respir Crit Care Med

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Optimal nutrition support has been integral in the management of cystic fibrosis (CF) since the disease was initially described. Nutritional status has a clear relationship with disease outcomes, and malnutrition in CF is typically a result of chronic negative energy balance secondary to malabsorption. As the mechanisms underlying the pathology of CF and its implications on nutrient absorption and energy expenditure have been elucidated, nutrition support has become increasingly sophisticated. Comprehensive nutrition monitoring and treatment guidelines from professional and advocacy organizations have unified the approach to nutrition optimization around the world. Newborn screening allows for early nutrition intervention and improvement in short- and long-term growth and other clinical outcomes. The nutrition support goal in CF care includes achieving optimal nutritional status to support growth and pubertal development in children, maintenance of optimal nutritional status in adult life, and optimizing fat soluble vitamin and essential fatty acid status. The mainstay of this approach is a high calorie, high-fat diet, exceeding age, and sex energy intake recommendations for healthy individuals. For patients with exocrine pancreatic insufficiency, enzyme replacement therapy is required to improve fat and calorie absorption. Enzyme dosing varies by age and dietary fat intake. Multiple potential impediments to absorption, including decreased motility, altered gut luminal bile salt and microbiota composition, and enteric inflammation must be considered. Fat soluble vitamin supplementation is required in patients with pancreatic insufficiency. In this report, nutrition support across the age and disease spectrum is discussed, with a focus on the relationships among nutritional status, growth, and disease outcomes.

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Highlights from the nutrition guidelines for cystic fibrosis in Australia and New Zealand

Cited by 42 publications

References 19 publications

Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis

Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis

Adherence to Dietary Recommendations, Nutrient Intake Adequacy and Diet Quality among Pediatric Cystic Fibrosis Patients: Results from the GreeCF Study

Growth and Nutrition in Cystic Fibrosis

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