Energy expenditure in obese children with pseudohypoparathyroidism type 1a.

Shoemaker AH, Lomenick JP, Saville BR, Wang W, Buchowski MS, Cone RD
Int J Obes (Lond). 2013 37 (8): 1147-53

PMID: 23229731 · PMCID: PMC3610772 · DOI:10.1038/ijo.2012.200

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.

MeSH Terms (23)

Absorptiometry, Photon Adolescent Age of Onset Basal Metabolism Blood Glucose Body Composition Calorimetry, Indirect Child Disease Susceptibility Energy Metabolism Female Glycated Hemoglobin A Humans Insulin Pediatric Obesity Phenotype Polymorphism, Single Nucleotide Postprandial Period Pseudohypoparathyroidism Rest Thermogenesis United States Weight Gain

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