Nutritional status of adolescents with cystic fibrosis treated at a reference center in the southeast region of Brazil (original) (raw)
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Evaluation of Nutritional Status in Patients with Cystic Fibrosis According to Age Group
Revista Paulista de Pediatria, 2019
Objective: To evaluate the nutritional profile of the population assisted at a reference center for cystic fibrosis treatment. Methods: 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. Results: 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 un...
The nutritional status of children with cystic fibrosis
British Journal of Nutrition, 2006
The importance of nutritional intervention for children with cystic fibrosis (CF) is well recognised. It would be expected that the increase in knowledge over the past decade would be reflected in improvements in nutritional status for the CF paediatric population. The aim of the present paper was to evaluate the nutritional status of children with CF, cross-sectionally and longitudinally. Body cell mass adjusted for gender and size (BCM/Ht p ) was measured in sixty-four children with CF to represent nutritional status and expressed as a Z-score. The cross-sectional results showed a mean BCM/Ht p Z-score of 0·54 (SD 1·21), with males having a slightly higher Z-score than females but with a larger variation. At the initial measurement, only one female and one male were considered sub-optimally nourished. The longitudinal analysis after 2 years showed that the mean population had a significantly decreased BCM/Ht p Z-score; however, when each gender was analysed separately, this decrease was significant only in the males. At the final measurement, only two females and three males were considered sub-optimally nourished. It is evident from our results that children with CF are well nourished, with only a small percentage considered malnourished. It appears that nutritional status decreases with age, with this decline being more evident in males. These results signify that although children with CF are better nourished with current treatment support, intervention needs to continue throughout a CF patient's life to counteract the changes that occur with age.
2020
Malnutrition prevails in considerable proportions of children with Cystic Fibrosis (CF), and is often associated with adverse outcomes. For this, routine screening for malnutrition is pivotal. In the present cross-sectional study, we aimed to assess the risk for malnutrition in pediatric outpatients with CF. A total of 76 outpatients (44 girls, 11.9 ± 3.9 years old, 39.5% adolescents) were recruited and anthropometric, clinical, dietary and respiratory measures were collected. All outpatients were screened for malnutrition risk with a validated disease-specific instrument. Most children exhibited a low risk for malnutrition (78.9%), whereas none of the participants were characterized as having a high malnutrition risk. In the total sample, malnutrition risk was positively associated with age (r = 0.369, p = 0.001), and inversely related to the body mass index (r = −0.684, p < 0.001), height z-score (r = −0.264, p = 0.021), and forced expiratory volume (FEV 1 %, r = −0.616, p < 0.001). Those classified as having a low malnutrition risk were younger (p = 0.004), heavier (p < 0.001) and taller (p = 0.009) than their counterparts with a moderate risk. On the other hand, patients in the moderate risk group were more likely pubertal (p = 0.034), with a reduced mid-upper arm fat area (p = 0.011), and worse pulmonary function (p < 0.001). Interestingly, none of the children attaining ideal body weight were classified as having a moderate malnutrition. risk, whereas 37.5% of the patients allocated at the moderate risk group exhibited physiological lung function. In this cohort of outpatients with CF that were predominantly well-nourished and attained physiological lung function, malnutrition risk was identified only in small proportions of the sample. Our data support that patients that are older, pubertal, or have diminished fat mass are at greater risk for malnutrition.
Clinical Nutrition, 2018
(BMI) and results as Z-scores in pediatric population. Statistical analysis by SPSS v.20. Results: Mean of FEV 1 and FVC were 88.4±22 and 89±21.5, with respiratory colonization by Pseudomonas aeruginosa and Methicillin-sensitive Staphylococcus aureus (6,5%). FEV 1 was positively correlated with weight Z-score (r¼0.37), WI for height (r¼0.29) and inversely correlated with age (r¼À0.32), and serum C-reactive protein (CRP) (r¼À0.46) (all, at least p<0.05). FEV 1 < 65% was found in 31 CF-patients. By comparing cutoff points of FEV 1 (moderate to severe; <65% and mild: >65%), significant differences with Z-scores for height (p¼0.016), BMI Z-scores (p¼0.012) and CRP (p<0.001) were found. Age, BMI Z-score and CRP were significant predictors of lung function in linear regression analysis (R 2 : 0.31; p<0.001). Conclusions: Pulmonary function is significantly related with nutritionalinflammatory status in CF pediatric patients. Early CF diagnosis and monitoring play a pivotal role in CF-pediatric patients.
BMI fails to identify poor nutritional status in stunted children with CF
Journal of Cystic Fibrosis, 2017
Background: Body mass index (BMI) is currently emphasized for evaluating nutritional status in children with cystic fibrosis (CF). Weight for age (WFA) and height for age (HFA) may get less attention. Methods: Data from the Epidemiologic Study of Cystic Fibrosis were used to compare patient WFA, HFA, and BMI percentiles for children age 2 to 18 years. Results: For children with BMI between the 25th and 50th percentiles, 16.8% had WFA b 10th percentile and 26.6% had HFA b 10th percentile. Conclusions: BMI fails to identify a substantial proportion of children with CF who have stunting or potentially poor nutritional status as measured by WFA and/or HFA.
Evaluation of nutritional status and related factors in children with cystic fibrosis
The Turkish Journal of Pediatrics
Cystic fibrosis (CF) is an autosomal recessive, multi-systemic disorder which forms as a result of mutation in the protein encoding gene known as cystic fibrosis transmembrane conductance regulator (CFTR). The two most significant problems determining survival are malnutrition and pulmonary disease. 1 The nutritional status in CF is related to insufficient macro/micronutrient intake, maldigestion/ malabsorption, increased energy requirement, and genotype. 2 Maldigestion and malabsorption associated with exocrine pancreatic failure cause loss of energy. This loss may be increased in conditions such as accompanying intestinal inflammation, small intestinal bacterial overgrowth, insulin resistance and impaired liver functions. In addition, total and resting energy expenditure (TEE, REE) are increased
Growth assessment in Egyptian children with cystic fibrosis: A single center study
The Gazette of the Egyptian Paediatric Association, 2017
Background: Poor clinical outcomes in cystic fibrosis are often associated with undernutrition. Nutritional status is an important prognostic marker, regardless of the level of respiratory function in the patient. Careful and repeated nutritional assessments allow for early detection of nutritional deterioration. Objectives: To assess the growth of cystic fibrosis children using different parameters as body mass index percentiles (BMIP) and standard anthropometric indexes, including height-forage percentile (HAP), weight-forage percentile (WAP) and compare between them as measures for nutritional failure in children with cystic fibrosis (CF). Methodology: This was a cross sectional study including fifty children of both sexes with CF below 12 years of age, recruited from the cystic fibrosis clinic, Specialized Children's Hospital, Cairo University. Patients were subjected to full history taking including age at diagnosis, frequency of hospital admissions (per year), current medications and presence of complications. Thorough clinical examination was performed with special emphasis on anthropometric measurements including body weight, length/ height, BMI calculation. Results: This study included 30 boys and 20 girls between 2 and 11 years old with a mean of 4.25 ± 2.30 yrs. Pancreatic insufficiency was found in 36% of patients, 28% had failure to thrive as the main complaint while 72% had recurrent chest infection as the main complaint. Growth assessment revealed that 21 patients (42%) were below À2 SDS for weight. As for BMI, 14 patients (28%) were below the 10th percentile. A highly significant negative correlation was reported between the weight SDS and the frequency of hospital admission (r = À0.412, p = 0.003). Furthermore, there was a highly statistical significant difference between the malnourished patients and the patients with average weight regarding the frequency of hospital admission (p < 0.01). As for BMI, there was a statistically significant negative correlation between the BMI and the frequency of hospital admission (r = À0.350, p = 0.013), with the different categories of BMI (malnourished, those who need nutritional reassessment, normal and obese) having highly statistical significant difference as regards the frequency of hospital admission (p < 0.01). Conclusion: Malnourished CF patients had the highest frequency of hospital admission. BMIP predicts nutritional failure more sensitively and accurately than conventional anthropometric indexes (WAP and HAP) in children with CF.
Journal of Cystic Fibrosis, 2016
Objectives: To compare nutritional outcomes in patients diagnosed by NS with those presenting with MI. Methods: Patients who have only received care at our centre since 1999 were included. All patients were reviewed 2-8 weekly by the same paediatric dietitian. Pancreatic sufficient (PS) and insufficient patients are included in this initial analysis. The primary outcome measures were differences in weight, height and BMI standard deviation scores (SDS). Results: In December 2015 169 patients received full care at our centre. 95 patients were included in the analysis. 71 (42M) were diagnosed by NS and 24 (14M) presented with MI (22 had surgery). 74 patients were excluded (late diagnosis, treatment elsewhere, prematurity, tube feeding, liver transplant). Of the NS (MI) infants, 12 (3) were PS, 46 (19) were homozygous and 21 (3) heterozygous for pPhe508del. Median (range) age at diagnosis for the NS infants was 25.6 days (1.8-51.1) compared to 17.2 days (5.1-38) for the MI infants. Median weight and height SDS for NS (MI) infants at 3,