RESUMO
OBJECTIVES: To determine whether early treatment of cystic fibrosis made possible by diagnosis after newborn screening results in improved pulmonary outcomes in adolescence. STUDY DESIGN: Both screening (SG) and non-screening groups (NSG) comprised a cohort of children from Australia previously studied at 1, 5, and 10 years of age. The groups were compared on measures of clinical status obtained during their comprehensive annual review conducted at or near the 15th birthday of the subjects. RESULTS: Data were collected on 48 of 57 original subjects in the NSG (7 had died; 2 were lost to follow-up) and 52 of 60 original subjects in the SG (4 had died; 2 transferred out of the country; 2 were lost to follow-up). Those dying in the SG were significantly older (by 48 months, P < .05) than those in the NSG. No statistically significant differences were found between the groups in nutritional status. However, subjects in the SG displayed statistically better total Shwachman-Kulczycki scores (7.0, P Assuntos
Fibrose Cística/diagnóstico
, Triagem Neonatal/organização & administração
, Adolescente
, Fatores Etários
, Estatura
, Peso Corporal
, Criança
, Mortalidade da Criança
, Transtornos da Nutrição Infantil/etiologia
, Pré-Escolar
, Fibrose Cística/complicações
, Fibrose Cística/mortalidade
, Fibrose Cística/terapia
, Diagnóstico Precoce
, Medicina Baseada em Evidências
, Feminino
, Seguimentos
, Volume Expiratório Forçado
, Humanos
, Recém-Nascido
, Masculino
, New South Wales/epidemiologia
, Estado Nutricional
, Avaliação de Resultados em Cuidados de Saúde
, Índice de Gravidade de Doença
, Espirometria
, Taxa de Sobrevida
, Capacidade Vital
RESUMO
OBJECTIVES: To evaluate which factors might contribute to raised resting energy expenditure (REE) in patients with cystic fibrosis (CF). STUDY DESIGN: REE and anthropometry were measured in 134 (males = 68) children with CF and 100 (males = 51) controls (range, 3-18.7 years) in an outpatient setting. Bacterial colonization, liver disease, inhaled steroid use, pancreatic and pulmonary function, sex, and genotype were determined and regression analysis was used to determine the predictors of REE in the group with CF. RESULTS: REE for children with CF was increased on average by 7.2% compared with controls. This increase was greater for females than for males. REE in males was positively associated with fat-free mass (FFM), pancreatic insufficiency (PI), and liver disease, and negatively associated with pulmonary function, whereas in females, REE was positively associated with FFM and PI. REE (adjusted for FFM) was higher in children with a severe mutation (5495 +/- 47 kJ) compared with a mild mutation (5,176 +/- 124 kJ, P <.02). CONCLUSIONS: PI, severe mutations, and female sex are the main contributing factors to elevated REE in patients with CF with near normal pulmonary function.