title: Increased resting energy expenditure is associated with failure to thrive in infants with severe combined immunodeficiency.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
authors: Division of Immunology and Allergy, The Canadian Centre for Primary Immunodeficiency, The Jeffrey Modell Research Laboratory for the Diagnosis of Primary Immunodeficiency, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Mary A
authors: MA
authors: Barron
authors: Melanie
authors: M
authors: Makhija
authors: Lorrie E M
authors: LE
authors: Hagen
authors: Paul
authors: P
authors: Pencharz
authors: Eyal
authors: E
authors: Grunebaum
authors: Chaim M
authors: CM
authors: Roifman