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Nutrição e Gastroenterologia

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



Avaliar o perfil nutricional da população atendida em centro de referência em fibrose cística.

Estudo transversal incluindo pacientes com fibrose cística de um centro pediátrico de referência de São Paulo, em 2014. Todos os sujeitos que concordaram em participar do estudo foram incluídos. Foi aplicado um questionário sobre hábitos alimentares (recordatório de 24 horas) e características socioeconômicas. Dados antropométricos (comparados com referencial da Organização Mundial da Saúde de 2006 e 2007) e função pulmonar foram coletados do prontuário. Os integrantes da pesquisa foram estratificados em faixas etárias para análise estatística.

Dos 101 pacientes incluídos no estudo, 59,4% eram masculinos, sendo a maioria caucasiana (86,4%), com mediana de idade de 10 anos. A maioria dos pacientes (n=77, 75%) foi classificada como eutrófica, mas valores menores de escore Z de índice de massa corpórea (IMC) foram observados em escolares e adolescentes. A proporção de pacientes com peso abaixo do esperado foi de 10% (n=2) na faixa etária pré-escolar e de 35% (n=6) em escolares. O consumo alimentar mostrou-se adequado, e somente dois suplementos (triglicérides de cadeia média e suplemento em pó completo) tiveram utilização ligada à adequação da ingesta de macronutrientes. Características socioeconômicas não apresentaram associação com o estado nutricional ou com o consumo nutricional. Valores de função pulmonar não apontaram diferença significante em adolescentes nem em indivíduos com pior estado nutricional no grupo estudado.

A maioria dos pacientes apresentou estado nutricional e consumo alimentar adequados, indicando bom manejo das ações nutricionais. Novos estudos com foco em pré-escolares devem ser realizados para avaliar se é possível reduzir o risco nutricional de pacientes com fibrose cística em idades posteriores.

Palavras-chave: Fibrose cística; Nutrição; Criança; Adolescente.

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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.

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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Sociedade Brasileira de Nutrição Parenteral e Enteral
Sociedade Brasileira de Clínica Médica
Associação Brasileira de Nutrologia

Elaboração final: 15 de agosto de 2011
Participantes: 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 


A fibrose cística é a doença genética letal mais comum em populações caucasianas e é caracterizada por infecções crônicas e recorrentes do pulmão, insuficiência pancreática e elevados níveis de cloro no suor. É uma doença de herança autossômica recessiva causada pela mutação no gene do Regulador da Condutância Transmembrana da Fibrose Cística, que induz o organismo a produzir secreções espessas e viscosas que obstruem os pulmões, o pâncreas e o ducto biliar. Muitos pacientes apresentam insuficiência pancreática, que leva a má-absorção de nutrientes especialmente de proteínas e lipídeos e a complicações gastrintestinais tais como prolapso retal, síndrome da obstrução intestinal, constipação e cirrose hepática. A fibrose cística normalmente é diagnosticada na infância, pelos programas de triagem neonatal ou pelo teste do suor. Devido aos vários sistemas envolvidos e à variabilidade e cronicidade da doença, uma abordagem multidisciplinar é essencial para auxiliar o paciente e sua família a compreenderem a doença e aderirem ao tratamento. A terapia atual da fibrose cística inclui a manutenção do estado nutricional, a remoção das secreções das vias aéreas com fisioterapia e mucolíticos, o uso de antibióticos para prevenção e tratamento de infecções, a prescrição de suplementos energéticos, dietas hiperlípidicas e hiperprotéicas, bem como a suplementação de minerais e vitaminas lipossolúveis. O objetivo deste trabalho foi realizar breve revisão de literatura sobre os aspectos clínicos e nutricionais da fibrose cística.

Descritores: Desnutrição, fibrose cística, terapia nutricional.

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