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

Mary A Barron1, Melanie Makhija, Lorrie E M Hagen

  • 1Division 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.

The Journal of Pediatrics
|May 20, 2011
PubMed

Insights

Hypermetabolism, or increased resting energy expenditure, is common in patients with severe combined immunodeficiency (SCID) and is linked to failure to thrive. This finding suggests a need for intensive nutritional support in these patients.

Area of Science:

  • Immunology
  • Pediatrics
  • Metabolic Medicine

Background:

  • Severe combined immunodeficiency (SCID) presents significant challenges, including nutritional deficits.
  • Failure to thrive (FTT) is a critical concern in infants and children diagnosed with SCID.
  • Understanding metabolic derangements is crucial for managing SCID patients.

Purpose of the Study:

  • To quantify resting energy expenditure (REE) in SCID patients at diagnosis.
  • To investigate the association between elevated REE (hypermetabolism) and FTT in SCID.
  • To inform nutritional management strategies for SCID.

Main Methods:

  • Measured REE in 26 SCID patients.
  • Defined hypermetabolism as measured REE >110% of predicted REE (WHO standards).
  • Collected data on FTT, infections, genetics, and feeding methods.

Main Results:

  • 57.7% of SCID patients exhibited FTT.
  • 69.2% of SCID patients were hypermetabolic.
  • Hypermetabolism was significantly more prevalent in patients with FTT (93%) compared to those without (36%) (P = .003).
  • Measured REE was significantly higher than predicted REE (71.75 ± 16.6 vs. 52.85 ± 2.8 kcal/kg; P < .0001).
  • 65% of patients required specialized nutrition support (nasogastric feeding or parenteral nutrition).

Conclusions:

  • Hypermetabolism is a frequent condition in SCID patients.
  • Increased REE may be a contributing factor to FTT development in SCID.
  • Intensive nutritional support is often necessary for SCID patients experiencing hypermetabolism and FTT.
Abstract

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