Biological status of adult patients with cystic fibrosis (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.
Malnutrition in adults with cystic fibrosis
European Journal of Clinical Nutrition, 2004
Objective: To determine the prevalence and clinical features of malnutrition and its relationship with the CFTR genotype in a cystic fibrosis (CF) adult population.
Nutrition status of adult patients with cystic fibrosis
New Medicine, 2014
Introduction. The number of adults with cystic fibrosis (CF) is gradually increasing. The nutritional status has an important role in the progression of the disease. Aim. To investigate the malnutrition risk and the nutritional status of adults with CF, and to analyse the obtained data according to gender, transplantation, dominantly affected system organ class, comorbidity, bacterial infection and sports habits. Material and methods. The nutritional status was assessed by a bioelectrical impedance machine, and the malnutrition risk screening was measured by NRS2002. Results. Forty-four patients participated (20 males, 24 females). Mean BMI in males was significantly higher compared with females (p = 0.01). The mean muscle percentage of patients doing sport regularly was significantly higher than those who do not do sport (p = 0.0126). Between mean body fat percentages there was no significant difference. There was no significant difference in mean BMI and body fat percentage between patients with and without transplantation, patients who live with one dominantly affected system organ class and patients who live with more dominantly affected system organ classes, patients who have concomitant illness and patients who do not, or patients who have one concomitant illness and patients who have more concomitant illnesses. Moreover, there was no significant difference in mean BMI and body fat percentage between patients who suffer from a bacterial infection and who do not, or patients who suffer from one bacterial infection and patients who have several bacterial infections. The majority of those patients who participated in the malnutrition risk screening were malnourished (77.3%). Conclusions. The research can prove high prevalence of malnutrition among adult patients with CF, which needs nutrition intervention to recover from.
Nutrients, 2021
The purpose of this study was to compare the nutritional status between deltaF508 CFTR hetero- and homozygous paediatric patients with cystic fibrosis. We assessed the percentage profiles of fatty acids measured in erythrocyte membranes and the serum levels of vitamins A, D3, E and K1 in the studied groups. We also measured the weights and heights and calculated the body mass indexes (BMIs). The studied groups consisted of 34 heterozygous and 30 homozygous patients. No statistically significant differences were found in the serum vitamins or erythrocyte membrane fatty acid profiles between the hetero- and homozygous patient groups, except for heptadecanoic acid (p = 0.038). The mean percentiles of height, weight and BMI did not differ significantly between the two groups. The homozygous and heterozygous paediatric patients with cystic fibrosis were similar in terms of their nutritional statuses.
Cystic Fibrosis: Correlations between Genotype and Phenotype
Journal of Clinical & Cellular Immunology, 2015
Cystic fibrosis is a clinical entity with multiple representations. Despite acquired knowledge, there is still unknown information about this disease. We tried to define the relationship between classes of mutations and clinical manifestations. We also tried to structure clinical manifestations depending on the most commonly found mutations, not minimizing intervention of environmental factors and modifier genes. We found that patients from the same family with the same mutation had different clinical manifestations, thus highlighting intervention of environmental factors and modifier genes. Diagnosis of cystic fibrosis is not easy because there are sometimes symptoms blurred, sometimes suggestive, but support the diagnosis by laboratory methods is not always possible. It is important that this condition be diagnosed as early as possible, even at birth, in order to prevent complications of the disease.
Factors associated with malnutrition in adolescent and adult patients with cystic fibrosis
Jornal Brasileiro de Pneumologia, 2017
Objective: To determine the prevalence of malnutrition in patients attending an adult cystic fibrosis (CF) program and to investigate the associations of malnutrition with the clinical characteristics of those patients. Methods: This was a cross-sectional study involving patients with clinically stable CF patients (16 years of age or older). The patients underwent clinical assessment, nutritional assessments, pulmonary function tests, and pancreatic function assessment. They also completed a questionnaire regarding diet compliance. On the basis of their nutritional status, the patients were classified divided into three groups: adequate nutrition; at nutritional risk; and malnutrition. Results: The study has included 73 patients (mean age, 25.6 ± 7.3 years), 40 of whom (54.8%) were female. The mean body mass index was 21.0 ± 3.0 kg/m2 and the mean FEV1 was 59.7 ± 30.6% of predicted. In this sample of patients, 32 (43.8%), 23 (31.5%), and 18 (24.7%) of the patients were allocated to ...
European Journal of Pediatrics, 1993
In an attenmpt to ascertain a relationship between genotype and phenotype, we studied the pulmonary and nutritional status of 123 cystic fibrosis patients with known genotype at an age of 8.5-10 years. Patients represent a cohort as they are almost all those born and diagnosed in a given area and period. They were followed at a single centre using uniform diagnostic and treatment protocols. Pulmonary and nutritional status of homozygous AF508 patients did not differ from that of compound heterozygotes or of patients with other unspecified genotypes. Pulmonary manifestations varied widely in all genotype groups. With the given number of patients, a slightly higher mortality of AF508 homozygotes could have been coincidental. We conclude that up to the age of 8.5-10 years the severity of pulmonary lesions and nutritional deficiencies is not related to the AF508 mutation.
Relationship between nutritional status and pulmonary function in adult cystic fibrosis patients
2008
Cystic fibrosis (CF) is a multisystem autosomal recessive disorder caused by the mutation of a single gene that encodes for the CF transmembrane regulator protein. Clinically, CF is characterized by chronic pulmonary infection, pancreatic insufficiency, and excessive losses of sweat electrolytes. Along with lung function, nutritional status appears one of the most important prognostic indicators in CF patients. In this study we examined the relationship between nutritional status and pulmonary function in adult CF patients. A group of 39 CF patients (mean age 23.9 +/-3.7 years) was studied. The mean value of body mass index (BMI) was 19.5 +/-2.9kg/m(2) (12.8-24.9kg/m(2)). The patients were grouped according to the presence or absence of malnutrition. Malnutrition was established in 11 patients (28.2%), 5 patients suffered from severe malnutrition. 28 patients (71.8%) had a normal nutritional status, but according to ESPEN guidelines, 9 of those patients were at risk of malnutrition....
The clinical and laboratory manifestations of Iranian patients with cystic fibrosis
The Turkish journal of pediatrics
Cystic fibrosis (CF) is a hereditary disease, characterized by chronic pulmonary disease, pancreatic insufficiency and abnormal electrolytes in the sweat. In order to evaluate the clinical manifestations and laboratory findings of Iranian children with CF during a 10-year period, 243 CF patients, with a median age of 5 months, were investigated in this study. The most common manifestations were gastrointestinal disorders and respiratory manifestations. Cough was the most common symptom, followed by malnutrition, diarrhea, respiratory distress, and vomiting. The frequency of these findings after treatment was significantly decreased in comparison with the period before diagnosis. During the mean follow-up of 40.9 months, seven cases died due to severe infections. Cystic fibrosis as a common genetic disorder should be considered in any child with recurrent gastrointestinal and respiratory manifestations, since delayed diagnosis could lead to severe complications and even death in this...