Abstract
Context:
Patients with pseudohypoparathyroidism type 1a (PHP-1a) develop early-onset obesity. The abnormality in energy expenditure and/or energy intake responsible for this weight gain is unknown.
Objective:
The aim of this study was to evaluate energy expenditure in children with PHP-1a compared with obese controls.
Patients:
We studied 6 obese females with PHP-1a and 17 obese female controls. Patients were recruited from a single academic center.
Measurements:
Resting energy expenditure (REE) and thermogenic effect of a high fat meal were measured using whole room indirect calorimetry. Body composition was assessed using whole body dual energy x-ray absorptiometry. Fasting glucose, insulin, and hemoglobin A1C were measured.
Results:
Children with PHP-1a had decreased REE compared with obese controls (P<0.01). After adjustment for fat-free mass, the PHP-1a group’s REE was 346.4 kcals day−1 less than obese controls (95% CI (−585.5–−106.9), P<0.01). The thermogenic effect of food (TEF), expressed as percent increase in postprandial energy expenditure over REE, was lower in PHP-1a patients than obese controls, but did not reach statistical significance (absolute reduction of 5.9%, 95% CI (−12.2–0.3%), P=0.06).
Conclusions:
Our data indicate that children with PHP-1a have decreased REE compared with the obese controls, and that may contribute to the development of obesity in these children. These patients may also have abnormal diet-induced thermogenesis in response to a high-fat meal. Understanding the causes of obesity in PHP-1a may allow for targeted nutritional or pharmacologic treatments in the future.
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Acknowledgements
We thank Rachel Lippert for her assistance with the mutation analysis and Lauren Whitaker for her assistance with the indirect calorimetry. We would like to acknowledge all the patients and their families who made this research possible. This study was supported in part by NIH grants 5T32HD060554-02 (AHS), the Vanderbilt CTSA grant URL1 RR024975-01 from NCRR/NIH, and 5 M01 RR-000095 from the NCRR/NIH (AHS), RO1DK070332 (RDC), and by a Fellows Development Research Grant in Diabetes, Obesity and Fat Cell Biology from the Endocrine Fellows Foundation (AHS).
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Shoemaker, A., Lomenick, J., Saville, B. et al. Energy expenditure in obese children with pseudohypoparathyroidism type 1a. Int J Obes 37, 1147–1153 (2013). https://doi.org/10.1038/ijo.2012.200
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DOI: https://doi.org/10.1038/ijo.2012.200
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