Energy expenditure in obese children with pseudohypoparathyroidism type 1a

A H Shoemaker, J P Lomenick, B R Saville, W Wang, M S Buchowski, R D Cone, A H Shoemaker, J P Lomenick, B R Saville, W Wang, M S Buchowski, R D Cone

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.

Figures

Figure 1
Figure 1
Patients with pseudohypoparathyroidism 1a (PHP-1a) have decreased resting energy expenditure (REE) after adjusting for fat free mass (−346.4 kcals/day, 95% CI [−585.5, −106.9], p= 0.007, linear regression). The linear regression equation is represented on the graph as a solid line for obese controls and a dotted line for PHP-1a patients. Patients with PHP-1a are represented by open circles and obese control patients are represented by black dots. REE was measured using whole-room indirect calorimetry. Fat free mass was determined using whole-body dual energy x-ray absorptiometry.
Figure 2
Figure 2
Estimated means of the percent increase in thermogenic effect of food over time stratified by mutation type. Due to the sample size, the model was constrained to have a constant mean difference between groups. Patients with PHP-1a are represented by the dotted line and obese control patients are represented by the solid black line. The light gray shading represents 95% CI; areas where the 95% CI overlap are represented by dark gray shading. Patients stood up to receive the meal which resulted in an increase in energy expenditure at time 0; they were instructed to remain seated for the remainder of the study.

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