Increased resting energy expenditure is associated with failure to thrive in infants with severe combined immunodeficiency

J Pediatr. 2011 Oct;159(4):628-32.e1. doi: 10.1016/j.jpeds.2011.03.041. Epub 2011 May 17.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Calorimetry, Indirect
  • Diarrhea / epidemiology
  • Energy Metabolism / physiology*
  • Failure to Thrive / physiopathology*
  • Failure to Thrive / therapy
  • Feces / virology
  • Humans
  • Infant
  • Infant Formula
  • Infant, Newborn
  • Infections / epidemiology
  • Intubation, Gastrointestinal
  • Logistic Models
  • Mutation
  • Parenteral Nutrition
  • Pneumonia / epidemiology
  • Receptors, Interleukin-2 / genetics
  • Rest / physiology*
  • Retrospective Studies
  • Severe Combined Immunodeficiency / physiopathology*

Substances

  • Receptors, Interleukin-2