Nutritional Status In Cystic Fibrosis Research Articles

Pulmonary function and body composition parameters of children with cystic fibrosis from a hight complexity institution between 2015 and 2018

BackgroundAn adequate nutritional status in patients with cystic fibrosis correlates with higher survival and better pulmonary function. Body mass index (BMI) is an anthropometric indicator with independent association with pulmonary function, and it can hide alterations of nutritional status in cystic fibrosis. ObjectiveTo determine the relationship of lung function with BMI and body composition parameters in children with cystic fibrosis in a Hight Complexity institution between 2015 and 2018. Materials and methodsIt was an observational study with analytical, cross-sectional, and retrospective scope, including 33 patients between 5 and 17 years old. Spearman's test was applied to evaluate correlation of FEV1 (forced expiratory volume in first second) with BMI and five body composition parameters by bioelectrical impedance using InbodyS10 equipment. Statistical significance was considered with p < 0.05. ResultsPositive correlation was obtained between FEV1 and appendicular skeletal muscle mass between lung function and percentage of fat-free mass (p = 0.006) and phase angle (p = 0.001). Percent fat mass had a negative correlation with FEV1 (p = 0.007). BMI and fat free mass index did not correlate with lung function (p = 0.085). ConclusionsAppendicular skeletal muscle mass correlated better than BMI with lung function in children with cystic fibrosis. It is recommended to perform anthropometric follow-up by BMI complemented with body composition study in children with cystic fibrosis. Analyses with larger populations are required to standardize its use.

The Risk of Developing of Malnutrition and the Principles of Correction of Nutritional Status Disorders in Children with Cystic Fibrosis

Cystic fibrosis is a severe hereditary disease with polysystemic manifestations and progressive course. Malnutrition in cystic fibrosis occurs as a result of exocrine insufficiency of the pancreas, an increase in energy losses in chronic inflammation in the bronchopulmonary system, manifested by increased stress on the respiratory system. The presented literature review highlights the modern principles of prevention and correction of malnutrition in children with cystic fibrosis, identifies the most promising methods for further development that correct nutritional status disorders. The review has shown that an active approach to nutrition at any age, the use of aggressive methods of nutritional support against the background of enzyme replacement therapy, timely and adequate therapy of respiratory tract pathology lead to an improvement in the indicators of nutritional status in cystic fibrosis. The most promising is the further development of targeted therapy, which allows, as a result of exposure to the etiopathogenetic mechanisms of the disease, to reduce the frequency and severity of bronchopulmonary exacerbations, partially restore the exocrine function of the pancreas, which is manifested in patients with cystic fibrosis by an increase in body weight and mass-growth index.

715 Impact of COVID-19 on lung function and nutritional status in cystic fibrosis: a global observational study

715 Impact of COVID-19 on lung function and nutritional status in cystic fibrosis: a global observational study

215 Genetic modifiers of nutritional status in cystic fibrosis are unique to cystic fibrosis and shared with the general population

215 Genetic modifiers of nutritional status in cystic fibrosis are unique to cystic fibrosis and shared with the general population

323 Evaluation of nutritional status in cystic fibrosis (CF) patients with percutaneous endoscopic gastrostomy (PEG) followed up at Tuscan center

323 Evaluation of nutritional status in cystic fibrosis (CF) patients with percutaneous endoscopic gastrostomy (PEG) followed up at Tuscan center

Associação dos parâmetros de crescimento e nutricionais com função pulmonar na fibrose cística: revisão da literatura

ObjectiveTo review the literature addressing the relationship of growth and nutritional parameters with pulmonary function in pediatric patients with cystic fibrosis. Data sourceA collection of articles published in the last 15 years in English, Portuguese and Spanish was made by research in electronic databases – PubMed, Cochrane, Medline, Lilacs and Scielo – using the keywords cystic fibrosis, growth, nutrition, pulmonary function in varied combinations. Articles that addressed the long term association of growth and nutritional parameters, with an emphasis on growth, with pulmonary disease in cystic fibrosis, were included, and we excluded those that addressing only the relationship between nutritional parameters and cystic fibrosis and those in which the aim was to describe the disease. Data synthesisSeven studies were included, with a total of 12,455 patients. Six studies reported relationship between growth parameters and lung function, including one study addressing the association of growth parameters, solely, with lung function, and all the seven studies reported relationship between nutritional parameters and lung function. ConclusionsThe review suggests that the severity of the lung disease, determined by spirometry, is associated with body growth and nutritional status in cystic fibrosis. Thus, the intervention in these parameters can lead to the better prognosis and life expectancy for cystic fibrosis patients.

Association of growth and nutritional parameters with pulmonary function in cystic fibrosis: a literature review

ObjectiveTo review the literature addressing the relationship of growth and nutritional parameters with pulmonary function in pediatric patients with cystic fibrosis. Data sourceA collection of articles published in the last 15 years in English, Portuguese and Spanish was made by research in electronic databases – PubMed, Cochrane, Medline, Lilacs and Scielo – using the keywords cystic fibrosis, growth, nutrition, pulmonary function in varied combinations. Articles that addressed the long term association of growth and nutritional parameters, with an emphasis on growth, with pulmonary disease in cystic fibrosis, were included, and we excluded those that addressing only the relationship between nutritional parameters and cystic fibrosis and those in which the aim was to describe the disease. Data synthesisSeven studies were included, with a total of 12,455 patients. Six studies reported relationship between growth parameters and lung function, including one study addressing the association of growth parameters, solely, with lung function, and all the seven studies reported relationship between nutritional parameters and lung function. ConclusionsThe review suggests that the severity of the lung disease, determined by spirometry, is associated with body growth and nutritional status in cystic fibrosis. Thus, the intervention in these parameters can lead to the better prognosis and life expectancy for cystic fibrosis patients.

Lung function decline is accelerated in South Africans with cystic fibrosis

Background: Poor nutritional status has been shown to be associated with a significant decline in lung function in patients with cystic fibrosis. There are few data published on the lung function decline and the effects of nutritional status in cystic fibrosis (CF) in South Africa. Aim: To assess anthropometric parameters (weight, height, body mass index Z-score) in relation to lung function parameters in CF patients. Methodology: A retrospective chart review of clinical records of participants over the age of five years attending the CF clinic at Steve Biko Academic Hospital from 2005 to 2010. Results: Twenty files were reviewed for lung function, anthropometric measurements, gender and CF-causing mutations. For anthropometric measurements the average changes were –0.8, –0.5 and 2.0 for weight, BMI and height Z-scores, respectively. A decline in FEV1 of –25.3 (95% CI 39.4; –13.3) over the five-year period was noted, with an average decline of 5.3% per year. For FEF25-75, the average change was –22.4 (95% CI-34.6; –10.2) with a decline of 4.5% per year. Using multivariate analysis, the FEV1 was found to be significantly influenced by: age –3.96 (95% CI –7.4; –0.5); p = 0.03, weight 1.8 (95% CI –3.4; –0.9); p = 0.04, BMI Z-score 4.3 (95% CI 5.3; 23.3); p = 0.02 and gender (p = 0.02). The FEF25-75 was significantly influenced by BMI Z-score and gender. Conclusion: The average lung function decline per year for FEV1 was higher than that seen in developed countries. The decline in FEV1 was related to gender, age, weight and BMI. The decline in FEF25-75 was affected only by BMI Z-score and gender.

Lung function decline is accelerated in South Africans with cystic fibrosis

Background: Poor nutritional status has been shown to be associated with a significant decline in lung function in patients with cystic fibrosis. There are few data published on the lung function decline and the effects of nutritional status in cystic fibrosis (CF) in South Africa.Aim: To assess anthropometric parameters (weight, height, body mass index Z-score) in relation to lung function parameters in CF patients.Methodology: A retrospective chart review of clinical records of participants over the age of five years attending the CF clinic at Steve Biko Academic Hospital from 2005 to 2010.Results: Twenty files were reviewed for lung function, anthropometric measurements, gender and CF-causing mutations. For anthropometric measurements the average changes were –0.8, –0.5 and 2.0 for weight, BMI and height Z-scores, respectively. A decline in FEV1 of –25.3 (95% CI 39.4; –13.3) over the five-year period was noted, with an average decline of 5.3% per year. For FEF25-75, the average change was –22.4 (95% CI...

Cystic fibrosis-related diabetes

Cystic fibrosis-related diabetes is an entity distinct from diabetes mellitus type 1 and 2, but with symptoms characteristic to them. Along with the extension of the life expectancy, the prevalence of diabetes increased in association with a more severe decline of the lung function and a poorer nutritional status in comparison to that of people with cystic fibrosis but without diabetes. The authors present current data regarding the prevalence and physiopathology of the disease, the clinical picture, and the useful examinations in establishing the diagnosis, the therapeutic possibilities and disease prognostic. We conclude that the early diagnosis and appropriate therapeutic interventions may diminish the negative impact of diabetes on the lung function and the nutritional status in cystic fibrosis.

Genetic modifiers of nutritional status in cystic fibrosis

Improved nutrition early in life is associated with better pulmonary function for patients with cystic fibrosis (CF). However, nutritional status is poorly correlated with the CFTR genotype. We investigated the extent to which modifier genes influence nutrition in children with CF. BMI data were longitudinally collected from the CF Twin-Sibling Study and Cystic Fibrosis Foundation Patient Registry for twins and siblings from 2000 to 2010. A nutritional phenotype was derived for 1124 subjects by calculating the average BMI z score from 5-10 y of age (BMI-z(5to10)). The genetic contribution to the variation in BMI-z(5to10) (ie, heritability) was estimated by comparing the similarity of the phenotype in monozygous twins to that in dizygous twins and siblings. Linkage analysis identified potential modifier-gene loci. The median BMI-z(5to10) was -0.07 (range: -3.89 to 2.30), which corresponded to the 47th CDC percentile. BMI-z(5to10) was negatively correlated with pancreatic insufficiency, history of meconium ileus, and female sex but positively correlated with later birth cohorts and lung function. Monozygous twins showed greater concordance for BMI-z(5to10) than did dizygous twins and siblings; heritability estimates from same-sex twin-only analyses ranged from 0.54 to 0.82. For 1010 subjects with pancreatic insufficiency, genome-wide significant linkage was identified on chromosomes 1p36.1 [log of odds (LOD): 5.3] and 5q14 (LOD: 5.1). These loci explained ≥16% and ≥15%, respectively, of the BMI variance. The analysis of twins and siblings with CF indicates a prominent role for genes other than CFTR to BMI variation. Specifically, regions on chromosomes 1 and 5 appear to harbor genetic modifiers of substantial effect.

Developing a measure of eating attitudes and behaviours in cystic fibrosis

BackgroundEating disorders or disturbed eating attitudes and behaviours (EABs) may contribute to poor nutritional status in Cystic Fibrosis (CF). Existing measures of disturbed EABs can have different meanings in this population and do not assess CF-related EABs. A self-report measure of EABs in CF was developed to highlight areas of eating disturbance. MethodsThe content validity of a draft measure was evaluated via expert evaluation and literature review and an amended measure piloted with 8 CF patients using cognitive interviewing. A further amended measure was administered to 155 CF patients (11–62years) attending CF clinics. ResultsPrincipal components analyses revealed a three-factor structure (‘Desire for thinness and weight loss’, ‘Disturbed EABs’, and ‘Appetite’) with good internal consistencies for subscales and the 21-item whole measure. ConclusionsThe measure looks promising as a tool to highlight EAB disturbance in CF. Further work will establish its construct validity and clarify interpretation of subscales.

Markers of oxidative stress in exhaled breath condensate: prediction of one-year development of pulmonary function and nutritional status in cystic fibrosis

Few studies have been published on airway resistance measurements using the interrupter technique (IT). We performed a cross-sectional study, evaluating 38 children and adolescents with Cystic Fibrosis (CF), followed at the outpatient CF clinic of Hospital Sao Lucas from Pontificia Universidade Catolica do Rio Grande do Sul. Airway resistance (Rint) was measured by the IT, followed by spirometry in all patients. Measurements were repeated after inhalation of salbutamol in order to evaluate bronchodilator response. There was a strong corelation between inverse Rint and forced expiratory volume in one second (FEV1) (r = 0.8, p< 0.001) and fair correlations between the inverse Rint and mid expiratory flow (MEF) (r = 0.74 p< 0.001) and between inverse Rint and body mass index (BMI) (r = 0.62 p< 0.001). The accuracy of bronchodilator response by the IT was tested through the ROC (reciever operating curve), comparing results with spirometry bronchodilator response. An area of 0.75 under the curve was obtained, for the cutoff point of −28% of Rint, achieving a sensitivity of 66% and a specificity of 82%. The findings suggest that Rint shows good correlation with spirometry parameters, although the IT is not sufficiently acurate to replace spirometry in the evaluation of bronchodilator response.

Mealtime problems predict outcome in clinical trial to improve nutrition in children with CF

Better growth and nutritional status is strongly associated with better pulmonary function and survival in children with CF. Behavioral intervention is an efficacious treatment approach for improving calorie intake and weight gain in children with CF; and recently has been shown to facilitate maintenance of daily energy intake at 120% of the healthy population over a 2-year period. However, no study to date has examined factors that predict outcome with behavior intervention to promote weight gain in CF. The objectives of this study were to examine the influence of nutritional status, mealtime behavior problems, and maternal depressive symptoms on calorie intake and weight gain following participation in a randomized trial to improve nutritional status in cystic fibrosis. Sixty-seven children, aged 4-12 years with cystic fibrosis participated in a clinical trial targeting calorie and weight increases. Participants completed baseline measures of mealtime behavior problems, maternal depression, and fat absorption, and baseline and post-treatment caloric intake and weight. Assignment to behavioral group (R(2) change = 0.17), lower frequency of mealtime behavior problems (R(2) change = 0.11), and higher maternal depression (R(2) change = 0.06) predicted greater calorie increase baseline to post-treatment. Assignment to behavioral group (R(2) change = 0.09), higher baseline weight (R(2) change = 0.10), fat absorption (R(2) change = 0.02), and lower frequency of mealtime behavior problems (R(2) change = 0.06) predicted greater weight gain baseline to post-treatment. Less frequent mealtime behavior problems led to better calorie intake and weight gain in a 9-week clinical trial of behavior intervention and nutrition education to improve nutritional status in cystic fibrosis. The key implication from these findings is that early referral to behavioral intervention as soon as growth deficits become a concern will likely yield the best nutritional outcomes.

Nutrition in Cystic Fibrosis

Cystic fibrosis (CF) is mostly recognized for its pulmonary morbidity, but the earliest manifestations of the disease are related to its gastrointestinal and nutritional derangements. Destruction of acinar pancreatic tissue, pancreatic ductular obstruction, and lack of enzymatic activity lead to malabsorption (particularly of fats), diarrhea, and failure to thrive. A minority of CF patients carrying milder CF transmembrane conductance regulator (CFTR) mutations have preserved pancreatic secretory activity and are free from significant malabsorption early in life. However, these patients are at risk for losing pancreatic function over time. Nutritional status plays an important role in the progression of the pulmonary disease in CF. Further, CF patients with better nutritional status have a survival advantage. Several factors contribute to impaired nutritional status in CF (e.g., pancreatic insufficiency, chronic malabsorption, recurrent sinopulmonary infections, chronic inflammation, increased energy expenditure, suboptimal intake). Progressive lung disease further increases calorie requirements by increasing the work of breathing. Treatment programs that place an emphasis on higher caloric intake and more aggressive nutritional management in CF patients report better outcomes. Basic tenets of nutritional repletion in CF include the use of pancreatic enzyme replacement therapy and following a high calorie, high protein, unrestricted diet. At the Stanford Cystic Fibrosis Center, nutritional status is assessed on an ongoing basis through anthropometric parameters and annual assessment of body composition, bone density, glucose tolerance, and various biochemical and micronutrient levels. Based on the anthropometric data obtained on routine clinical encounters, patients are categorized as to their nutritional risk. This proactive approach for the early identification of nutritional risk has become a major theme within the network of US CF centers. Aggressive nutritional support with adequate pancreatic replacement management should lead to both normal growth and lung function preservation. In addition, nutritional status has to be monitored closely during routine encounters to allow for early intervention once derangements are noted. This will include increasing calories in the early stages of lung disease and being vigilant of gastrointestinal symptomatology and complications.

Appetite stimulants use in cystic fibrosis

Cystic fibrosis (CF) is an autosomal recessive disease. It affects multiple body organs. The lungs and pancreas are the most affected which results in progressive lung damage and pancreatic insufficiency. Due to the disease process, CF patients require significantly higher caloric intake than recommended for other individuals. The nutritional goal for CF patients is to achieve normal growth and development and, once genetic potential is reached, to maintain good nutritional status throughout life. Evidence has shown that lung function is closely associated with nutritional status in CF and that nutritional status is an independent predictor of survival. Most CF patients are on a high calorie diet to help achieve normal growth and development and maintain good lung function. Inadequate caloric intake in CF can lead to malnutrition. Malnutrition in CF requires careful, multidisciplinary history taking, physical exam, and overall patient/family assessment. Only by determining the actual cause of the malnutrition can appropriate and safe therapies be used to treat it. Appetite stimulants, although efficacious in treating malnutrition in CF, should only be prescribed if decreased food intake secondary to inadequate appetite is the principal cause of the malnutrition and all other contributing factors have been assessed, ruled-out or treated. In this review, we attempted to summarize the use of several appetite stimulants used in CF and other diseases to improve appetite and maximize caloric intake.

Improving nutritional status in cystic fibrosis (CF) by implementing a multidisciplinary approach to paediatric patients with malnutrition

Improving nutritional status in cystic fibrosis (CF) by implementing a multidisciplinary approach to paediatric patients with malnutrition

Classification of nutritional status in cystic fibrosis

Nutritional status impacts on the progression of cystic fibrosis. Current guidelines recommend the use of anthropometric indicators to classify nutritional status and identify malnutrition. However, the current nutrition-classification systems are problematic. I summarize these problems and review recent progress in the development of evidence-based anthropometric criteria for classifying nutritional status in cystic fibrosis patients. Percentage of ideal body weight as a malnutrition index is flawed. In children with cystic fibrosis, this index underestimates the severity of underweight in short patients and overestimates it in tall patients. In adults with cystic fibrosis, percentage of ideal body weight based on the Metropolitan Life Insurance reference weights for medium/large frames overestimates the severity of underweight. Body-mass-index percentile for children and body mass index for adults as underweight indices have been proven to be valid. Strong associations between body mass index and lung function are also observed, but cutoff values to maintain a desirable level of lung function can vary. Body mass index should replace the use of percentage of ideal body weight for classifying underweight in cystic fibrosis patients. More research is needed to identify appropriate indicators to classify short stature in children with cystic fibrosis.

Serum Leptin and Cytokines in Whole Blood in Relation to Clinical and Nutritional Status in Cystic Fibrosis

Leptin plays an important role in the energy balance and may be affected by hormonal and metabolic derangement associated with chronic disease. The aim of this study was to assess the correlation between leptin, proinflammatory cytokines and nutritional status with regard to clinical status in homozygous delta F 508 cystic fibrosis patients. Patients with mild (Shwachman score 71-100 points, group A) disease were compared with those with moderate disease (Shwachman score 41-55 points, group B) and age-matched controls (group C, n = 22). Leptin was assessed by enzyme-linked immunosorbent assay and cytokines (interleukin-8, tumor necrosis factor alpha) before and after stimulation with 5 ng lipopolysaccharide by a chemiluminescent immunometric assay. Twenty-two patients were recruited for each group (median A/B/C forced expiratory volume in 1 second 80%/59%/-; median age 12/13.5/12.5 years). Leptin (median 3.25/2.65/3.3 pg/mL; P = 0.083) and body mass index were lower (group A/B/C 18.55/16.75/20.5 kg/m(2); P = 0.023), but dietary intake was significantly higher (group A/B/C 50.5/68/43 kcal/kg body weight; P = 0.026) in moderate disease. Cytokines before stimulation with lipopolysaccharide were highest in moderate disease, but there was no significant difference after stimulation (interleukin-8 median A/B/C before--15/25.1/8.0 pg/mL, P < 0.005; after--570.5/573.5/415.5 pg/mL, not significant; tumor necrosis factor alpha median A/B/C 43/56/30 pg/mL, P < 0.0001; 580/427/720.5 pg/mL, not significant.). There is a physiological regulation of leptin even in more advanced states of disease with significantly lower body mass index than controls. However, our data do not support the idea of elevated cytokine levels inducing anorexia in homozygous delta F 508 cystic fibrosis patients.

Behavioral aspects of nutrition in children with cystic fibrosis

Nutritional status in cystic fibrosis is important to the health and long-term survival of patients; however, children with cystic fibrosis are not achieving the dietary recommendations or achieving normal growth. This paper reviews current knowledge about behavioral and environmental barriers to dietary adherence in this population. Children with cystic fibrosis do not have the same appreciation of the need to gain weight as their parents. Parents of children with cystic fibrosis describe meal times as 'battling' with the child. Direct observation studies of meal times demonstrate that school-aged children with cystic fibrosis do not differ from children without cystic fibrosis on the frequency of behaviors incompatible with eating, as was seen in younger age groups, but parents of school-aged children with cystic fibrosis continue to engage in ineffective management styles at a greater frequency than parents of children without cystic fibrosis. Assessment of family functioning during mealtime demonstrates that these interaction patterns have a negative effect on family functioning in families of children with cystic fibrosis compared with families of same-age peers in all age groups: infancy/toddler, preschool, and school age. Achieving the cystic fibrosis dietary recommendations is challenging, and typical parenting strategies to encourage eating not only are ineffective but may have a negative impact on family functioning. To improve dietary adherence and decrease conflict at meals, children with cystic fibrosis and their parents need to be taught more effective management strategies as part of dietary counseling.