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1.
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
3.
J Pediatr ; 147(4): 555-7, 2005 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-16227049

RESUMO

To study the correlation between genotype and phenotype in x-linked SCID, we have characterized the presentation of 2 unrelated patients. Both had infections suggestive of immunodeficiency, but their immune function and lymphoid tissues were normal. They were found to have an identical R222C mutation in the gammac gene.


Assuntos
Receptores de Interleucina-7/genética , Imunodeficiência Combinada Severa/genética , Imunodeficiência Combinada Severa/imunologia , Transplante de Medula Óssea , Análise Mutacional de DNA , Genótipo , Humanos , Lactente , Subunidade gama Comum de Receptores de Interleucina , Fenótipo , Imunodeficiência Combinada Severa/cirurgia
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