RESUMO
OBJECTIVE: The objective of this study was to document alterations in resting energy expenditure (REE), energy intake, and body composition that occur during admission for management of a pulmonary exacerbation in children with cystic fibrosis. STUDY DESIGN: A prospective study of 14 children with cystic fibrosis (8 male, 12.5 +/- 3.3 years) was conducted throughout a 14-day hospitalization and again 3 weeks after discharge. Repeated measures analysis of variance was used to examine the change in variables across time. RESULTS: Significant increases were documented from days 1 to 14 in weight (37.1 +/- 13.1 kg to 38.4 +/- 13.5 kg) and pulmonary function (forced expiratory volume in 1 second: 67.6% +/- 19.8% to 78.5% +/- 24.5%). Energy intake was not different between hospitalization and follow-up and averaged 175% of the Recommended Dietary Allowance. REE on day 1 was elevated (122% +/- 11% predicted); however, when expressed with fat free mass as a covariate, no significant changes were noted in REE throughout the eight measurements. CONCLUSIONS: These data suggest that an acute pulmonary exacerbation is not associated with an increase above a subject's baseline REE in children with cystic fibrosis with mild to moderate lung disease. Management of an acute pulmonary exacerbation should be associated with sustained improvement in pulmonary function and nutritional status; in contrast, weight loss or failure to improve pulmonary function should be seen as atypical.
Assuntos
Metabolismo Basal , Fibrose Cística/metabolismo , Doença Aguda , Adolescente , Composição Corporal , Criança , Fibrose Cística/complicações , Ingestão de Energia , Feminino , Hospitalização , Humanos , Masculino , Distúrbios Nutricionais/complicações , Estado Nutricional , Estudos Prospectivos , Testes de Função RespiratóriaRESUMO
OBJECTIVES: Prospective, 3-year longitudinal dietary intakes of 25 prepubertal, pancreatic-insufficient children with cystic fibrosis (CF) and mild lung disease, and the intakes of 26 control children were compared, and relationships among energy intake, nutritional status, and pulmonary function were determined. STUDY DESIGN: Intakes from 3-day weighed food records were compared with CF recommendations, recommended dietary allowances (RDA), and the recommendations of the Third National Health and Nutrition Examination Survey (NHANES III). Energy and nutrient intakes were analyzed by repeated-measures analysis of variance. RESULTS: Children with CF consumed more energy than control children (p = 0.025) in terms of calories per day, percentage of RDA by age and by age and weight, energy adjusted for fat malabsorption, and percentage of NHANES III recommendations. Energy intake was similar between boys and girls with CF. The percentage of energy from fat was greater (p = 0.0004) in the CF group (3-year mean, 33%) than in the control group. Height and weight z scores declined in the CF group (p <0.05) with time. Vitamin and mineral intakes were generally adequate in the CF group. CONCLUSIONS: The children in this sample did not consume the CF recommended intakes of 120% RDA for energy or a high-fat (40% of energy) diet. Energy intakes may be insufficient in this group to meet requirements for optimal growth.
Assuntos
Fibrose Cística , Registros de Dieta , Gorduras na Dieta/administração & dosagem , Proteínas Alimentares/administração & dosagem , Ingestão de Energia , Insuficiência Pancreática Exócrina , Crescimento , Criança , Fibrose Cística/complicações , Fibrose Cística/fisiopatologia , Insuficiência Pancreática Exócrina/complicações , Insuficiência Pancreática Exócrina/fisiopatologia , Feminino , Fluxo Expiratório Forçado , Volume Expiratório Forçado , Humanos , Masculino , Micronutrientes , Estudos ProspectivosRESUMO
Because growth and anthropometric measurements are frequently below normal in patients with cystic fibrosis (CF), the body composition of 23 children (6-9 y of age) who had mild manifestations of CF was examined and compared with a control group of 24 subjects similar in age, sex, and weight. Skinfold measurements, total body water (TBW) by deuterium oxide dilution, and total body electroconductivity (TOBEC) were measured. Skinfold and TBW measurements demonstrated that fat mass and fat-free mass did not differ significantly between the two groups. No significant difference in percent body fat was found by using the skinfold equations of Slaughter et al (13.8% vs 15.3%), although percent body fat (TBW method) differed between the groups (P < 0.05). TOBEC measurements (CF, n = 14) were not used in the group comparison. The majority of the methods demonstrated that the CF group achieved normal growth and body composition, with a possible trend of fat depletion.
Assuntos
Composição Corporal , Fibrose Cística/fisiopatologia , Tecido Adiposo/fisiopatologia , Água Corporal , Criança , Humanos , Dobras CutâneasRESUMO
A rapid, simple, nonradioactive method for detection of four common mutations causing cystic fibrosis (CF) has been developed combining multiplexing with allele-specific polymerase chain reaction amplification. This approach (MASPCR) provides an easy assay for direct genotyping of normal and mutant CF alleles in homozygotes and heterozygotes. The strategy involves multiplex PCR of exons 10, 11, and 21 within the cystic fibrosis transmembrane conductance regulator (CFTR) gene in a single reaction containing three common oligoprimers and either the four normal or four mutant oligos corresponding to the delta F508, G551D, G542X, and N1303K mutations. Primers are chosen so that the size of the four PCR products differ, thereby facilitating detection on agarose gels following amplification in the same reaction. Patient samples are primed with either four normal or four mutant oligo mixtures, and PCR products run in parallel on gels to detect band presence or absence. This approach provides a simple and potentially automated method for cost-effective population screening.
Assuntos
Fibrose Cística/genética , Proteínas de Membrana/genética , Mutação , Reação em Cadeia da Polimerase/métodos , Alelos , Sequência de Bases , Regulador de Condutância Transmembrana em Fibrose Cística , DNA de Cadeia Simples , Heterozigoto , Homozigoto , Humanos , Dados de Sequência Molecular , População Branca/genéticaRESUMO
A 13-year-old girl with cystic fibrosis (CF), severe lung disease, purpura on the lower extremities and with elevated serum mixed cryoglobulins is described. The possible relationship of the purpura in CF with cryoglobulinaemia is discussed.