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Nutrition and Gastroenterology

Neria LCL, Bergamaschia DP,  Silva Filho LVRF.
Rev. paul. pediatr. vol.37 no.1 São Paulo Jan./Mar. 2019  Epub Aug 09, 2018



To evaluate the nutritional profile of the population assisted at a reference center for cystic fibrosis treatment.

Cross-sectional study including patients with cystic fibrosis assisted at a pediatric reference center in São Paulo, Brazil, in 2014. All patients attending regular visits who agreed to participate in the study were included. A questionnaire on dietary habits (24-hour diet recall) and socioeconomic characteristics was applied. Anthropometric data (compared with the reference from the World Health Organization, 2006 and 2007) and pulmonary function data were collected from medical records. Patients were stratified into age groups for statistical analysis.

A total of 101 patients were included in the study (59.4% male, 86.4% Caucasian), with median age of 10 years old. Most patients (n=77; 75%) were classified as eutrophic, but lower values of body mass index (BMI) Z scores were observed in schoolchildren and adolescents. The proportion of underweight patients was 10% (n=2) among preschoolers and 35% (n=6) of the school age group. Dietary intake was adequate, and the use of only two supplements (medium chain triglycerides and complete powdered supplement) was associated with adequacy of macronutrient intake. The socioeconomic characteristics did not show any statistical association with the nutritional state or nutritional consumption. Lung function was not significantly different between neither adolescents nor individuals with worse nutritional status in this sample.

Most of the patients presented adequate nutritional status and adequate consumption of calories and macronutrients, indicating appropriate nutritional management. New studies focusing on preschool children should be performed to assess if it is possible to reduce the nutritional risk of patients with cystic fibrosis at older ages.

Keywords: Cystic fibrosis; Nutrition; Child, Adolescent.

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Hauschild DB, Barbosa E, Moreira EA, Ludwig Neto N, Platt VB, Filho EP, Wazlawik E, Moreno YM.

Nutr Clin Pract. 2016 Jun;31(3):378-86. doi: 10.1177/0884533615627157. Epub 2016 Feb 26.


(1) To compare nutrition and hydration status between a group of children/adolescents with cystic fibrosis (CFG; n = 46; median age, 8.5 years) and a control group without cystic fibrosis (CG). (2) To examine the association of nutrition and hydration status with lung function in the CFG.

Material and Methods
A cross-sectional study. Nutrition screening, anthropometric parameters, and bioelectrical impedance analysis (BIA) were assessed. The z scores for body mass index for age, height for age, mid upper arm circumference, triceps and subscapular skinfold thickness, mid upper arm muscle area, resistance/height, and reactance/height were calculated. Bioelectrical impedance vector analysis was conducted. Forced expiratory volume in 1 second < 80% was considered lung function impairment. An adjusted logistic regression was applied (P < 0.05).

In the CFG, lung function impairment was observed in 51.1%. All anthropometric parameters were lower, and the mean z-resistance/height and z-reactance/height were higher in the CFG (P < 0.05) compared with the CG. In the CFG, 43% were severely/mildly dehydrated, while none were in the CG (P = 0.007). In the CFG, there was an association between high nutrition risk-via nutrition screening (odds ratio [OR], 22.28; P < 0.05), lower values of anthropometric parameters, higher z-resistance/height (OR, 2.23; P < 0.05) and z-reactance/height (OR, 1.81; P < 0.05), and dehydration (OR, 4.94; P < 0.05)-and lung function impairment.

The CFG exhibited a compromised nutrition status assessed by anthropometric and BIA parameters. Nutrition screening, anthropometric and BIA parameters, and hydration status were associated with lung function.

© 2016 American Society for Parenteral and Enteral Nutrition.

Keywords: Cystic fibrosis; electrical impedance; forced expiratory volume; nutrition assessment; nutrition status; pediatrics.

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Barrio R.

Eur J Endocrinol. 2015 Apr;172(4):R131-41. doi: 10.1530/EJE-14-0644. Epub 2014 Oct 21.


Cystic fibrosis (CF) is a recessive genetic disease caused by mutations in the CF transmembrane conductance regulator (CFTR). CFTR is primarily present in epithelial cells of the airways, intestine and in cells with exocrine and endocrine functions. Mutations in the gene encoding the channel protein complex (CFTR) cause alterations in the ionic composition of secretions from the lung, gastrointestinal tract, liver, and also the pancreas. CF-related diabetes (CFRD), the most common complication of CF, has a major detrimental impact on pulmonary function, nutrition and survival. Glucose derangements in CF seem to start from early infancy and, even when the pathophysiology is multifactorial, insulin insufficiency is clearly a major component. Consistently, recent evidence has confirmed that CFTR is an important regulator of insulin secretion by islet β-cells. In addition, several other mechanisms were also recognized from cellular and animals models also contributing to either β-cell mass reduction or β-cell malfunction. Understanding such mechanisms is crucial for the development of the so-called ‘transformational’ therapies in CF, including the preservation of insulin secretion. Innovative therapeutic approaches aim to modify specific CFTR mutant proteins or positively modulate their function. CFTR modulators have recently shown in vitro capacity to enhance insulin secretion and thereby potential clinical utility in CFDR, including synergistic effects between corrector and potentiator drugs. The introduction of incretins and the optimization of exocrine pancreatic replacement complete the number of therapeutic options of CFRD besides early diagnosis and implementation of insulin therapy. This review focuses on the recently identified pathogenic mechanisms leading to CFRD relevant for the development of novel pharmacological avenues in CFRD therapy.

© 2015 European Society of Endocrinology.

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Del Ciampo IR, Del Ciampo LA, Sawamura R, de Oliveira LR, Fernandes MI.

Ital J Pediatr. 2015 Jul 30;41:51. doi: 10.1186/s13052-015-0159-x.


Several factors can interfere with the full physical and emotional growth of adolescents, among them chronic diseases. The aim was to determine the nutritional status of adolescents and to associate it with puberty, pancreatic sufficiency, lung function and age range of Cystic Fibrosis (CF) diagnosis.

An observational, cross-sectional, retrospective and analytical study was conducted using the data of medical records.

Reference center in the northeastern region of the state of São Paulo – Brazil.

All adolescents with CF attended in 2010 were included. Some variables included: pancreatic sufficiency (steatocrit >2 %), pancreatic enzymes replacement (yes/no), pubertal status-Tanner criteria (prepubertal: M1/G1, pubertal: M2/G2 to M4/G4, postpubertal: M5/G5), age at CF diagnosis (<2 and ≥2 years of age), Lung function, measured as a predicted forced expiratory volume in 1 s (FEV1). Main outcome measures Nutritional indicators: body mass index for age (BMI/A) and height for age (H/A) with z-score calculated with Anthro Plus software. Cut-off reference points: ≥ z-score -3 and < z-score -2 (thinness); z-score -2 and ≤ z-score-z +1 (normal weight); >z-score +1 (overweight or obesity), and z-score <-2 (low or very low H/A). The groups were compared by the Kruskal-Wallis test. Level of significance: p<0.05.

Thirty adolescents. Median (min;max) age: 14.4 (10.1;19.8) years. BMI/A and H/A z-score, respectively: early diagnosis of CF (-0.8; -1.1) or late diagnosis of CF (-0.5;-0.8); with pancreatic insufficiency (-0.7; -0.8) or without pancreatic insufficiency (-0.8; -0.5) and prepubertal (-0.8; -0.7) pubertal (-0.2; -1.5) or postpubertal (-0.7; -0.5). No significant difference (p>0.05) was observed. Patients with and without pancreatic insufficiency, presented H/A borderline z-score (p=0.05). Association between H/A and FEV1 was borderline (p=0.05).

Adolescents presented adequate nutritional status, although with slightly lower values than those of developed countries. FEV1 lower levels occurred more frequently in adolescents with low H/A.

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Penafortes JT, Guimarães FS, Moço VJ, Almeida VP, Menezes SL, Lopes AJ.

Braz J Phys Ther. 2013 Sep-Oct;17(5):450-7. doi: 10.1590/S1413-35552012005000111. Epub 2013 Sep 10.


Cystic fibrosis (CF) is a hereditary condition in which lung disease affects all patients. In addition to pulmonary involvement, the multisystemic components of CF cause significant physical limitations. However, the impact of lung function on balance control in CF has not been studied.

To assess body balance in adults with CF and to test its possible associations with lung function, nutritional status, and functional capacity.

This was a cross-sectional study in which 14 adults with CF underwent pulmonary function testing (spirometry, body plethysmography, and carbon monoxide diffusing capacity (DLco), respiratory muscle strength, 6-min walking distance (6MWD), Berg balance scale (BBS), nutritional analysis (body mass index and bioelectrical impedance), and stabilometry. Body balance was quantified using stabilometry; all participants performed the following two trials: opened base, eyes open (OBEO); closed base, eyes closed (CBEC).

In stabilometry, the median for the lateral range and anterior-posterior range in the CBEC trial was 0.10 (0.08-0.11) and 0.13 (0.11-0.22), respectively (p<0.05). The maximal inspiratory pressure (MIP) correlated inversely with the lateral standard deviation (ρ=-0.61; p<0.05) as the DLco correlated positively with the anterior-posterior range (ρ=0.54; p<0.05). There were significant relationships between body composition indexes and almost all stabilometric variables measured. There were no relationships of the BBS and 6MWD with the stabilometric variables.

In adults with CF, imbalance occurs mainly in the anterior-posterior direction and is especially associated with body composition.

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Souza dos Santos Simon MI, Drehmer M, de Abreu E Silva FA, Hoffmann A, Druck Ricachinewsky C, de Fonseca Andrade Procianoy E, Scattolin I,Saldanha Menna Barreto S.

Nutr Hosp. 2011 Nov-Dec;26(6):1322-7. doi: 10.1590/S0212-16112011000600019.


Malnutrition is related with pulmonary disease. The aim was to analyze the association of lung function respectively to nutritional status, identified pulmonary pathogens and socioeconomic condition of patients attending a pediatric CF reference center.

Cross-sectional study performed with CF patients aged 6 to 18 years attending a CF-Center in southern Brazil. Nutritional status, plasma albumin level and pulmonary bacterial colonization were assessed. The outcome studied was forced expiratory volume in 1 second (FEV1).

Eighty-five patients were included in this study. FEV1 was significantly associated with body mass index (BMI) percentiles, plasma albumin level and methicillin resistant Staphylococcus aureus (MRSA) pulmonary colonization. Regression analysis showed that BMI below the 10th percentile was associated with a 25.58% drop in FEV1, and plasma albumin levels equal to or lower than 4.1 mg/dL was associated with 18.6% FEV1 reduction. FEV1 was 14.4% lower in the MRSA infected patients. Plasma albumin of 4.25 mg/dL predicted FEV1 of 60% with 76.9% sensitivity and 72.2% specificity, and 85.7% accuracy. The socioeconomic status was not association with pulmonary function.

BMI below the 10th percentile and albumin below 4.1 mg/dL were predictors of low FEV1. Chronic MRSA infection was associated with lower FEV1. Longitudinal studies may better complement these results.

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Brazilian Society of Parenteral and Enteral Nutrition
Brazilian Society of Clinical Medicine
Brazilian Association of Nutrology

Final version: August 15, 2011
Participants: Epifanio M, Spolidoro JVN

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RosaI FR, Dias FG, NobreI LN, Morais HA.
Rev. Nutr. vol.21 no.6 Campinas Nov./Dec. 2008 


Cystic fibrosis is the most common, lethal, genetic disease in Caucasian populations and is characterized by chronic and recurrent lung infections, pancreatic insufficiency and high sweat chloride levels. It is an autosomal recessive hereditary disease caused by a mutation in the cystic fibrosis transmembrane conductance regulator gene which induces the body to produce thick and viscous mucus secretions that obstruct the lungs, pancreas and bile duct. Many patients have pancreatic insufficiency which leads to malabsorption of nutrients, especially proteins and fats and to gastrointestinal complications such as rectal prolapse, intestinal obstruction syndrome, constipation and hepatic cirrhosis. Cystic fibrosis is usually diagnosed during childhood by neonatal screening programs or sweat test. Because of the multiple systems involved and the variability and chronicity of the disease, a multidisciplinary team is essential to help patients and their families understand the disease and adhere to treatment. Current cystic fibrosis therapy includes maintaining the nutritional status, clearing the airways with physiotherapy and mucolytics, preventing and treating infections with antibiotics and prescribing energy supplements, high-fat and high-protein diets, as well as minerals and fat-soluble vitamins. The purpose of this study was to present a brief literature review of the clinical and nutritional aspects of cystic fibrosis.

Keywords: Malnutrition; Cystic fibrosis; Nutrition therapy.

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