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J Pediatr ; 159(4): 628-32.e1, 2011 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-21592502

RESUMO

OBJECTIVES: To measure resting energy expenditure (REE) and determine whether increased REE (hypermetabolism) is associated with failure to thrive (FTT) in patients with severe combined immunodeficiency (SCID) at diagnosis. STUDY DESIGN: REE was measured in 26 patients with SCID in a single transplant center. Predicted REE was determined with World Health Organization standards. Measured REE >110% of predicted REE was classified as hypermetabolism. Other data collected included FTT status, infections, genotype, phenotype, and the feeding methods used. RESULTS: Fifteen of 26 patients (57.7%) had FTT, and 18 of 26 patients (69.2%) were hypermetabolic. Hypermetabolism occured in 14 of 15 patients (93%) with FTT, and only 4 of 11 patients (36%) without FTT had hypermetabolism (P = .003). There was a significant difference between the measured REE (71.75 ± 16.6 kcal/kg) and the predicted REE (52.85 ± 2.8 kcal/kg; P < .0001). Eleven of 17 patients (65%) required nasogastric feeding, parenteral nutrition, or both to meet their energy needs. CONCLUSIONS: Hypermetabolism is common in patients with SCID and may contribute to the development of FTT. The hypermetabolism in these patients may necessitate intensive nutrition support.


Assuntos
Metabolismo Energético/fisiologia , Insuficiência de Crescimento/fisiopatologia , Descanso/fisiologia , Imunodeficiência Combinada Severa/fisiopatologia , Calorimetria Indireta , Diarreia/epidemiologia , Insuficiência de Crescimento/terapia , Fezes/virologia , Humanos , Lactente , Fórmulas Infantis , Recém-Nascido , Infecções/epidemiologia , Intubação Gastrointestinal , Modelos Logísticos , Mutação , Nutrição Parenteral , Pneumonia/epidemiologia , Receptores de Interleucina-2/genética , Estudos Retrospectivos
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