Resting Energy Expenditure is Decreased in Pseudohypoparathyroidism Type 1A.

Resting Energy Expenditure is Decreased in Pseudohypoparathyroidism Type 1A.

J Clin Endocrinol Metab. 2015 Dec 28;:jc20153895

Authors: Roizen JD, Danzig J, Groleau V, McCormack S, Casella A, Harrington J, Sochett E, Tershakovec A, Zemel BS, Stallings VA, Levine MA

Abstract
CONTEXT: Pseudohypoparathyroidism type 1A (PHP1A) is caused by loss-of function mutations on the maternally-inherited GNAS allele, and is associated with early-onset obesity, neurocognitive defects and resistance to multiple hormones. The role of energy intake versus central regulation of energy expenditure in the pathophysiology of obesity remains unclear.
OBJECTIVE: The aim of this study was to evaluate resting energy expenditure (REE) in participants with PHP1A.
DESIGN: We assessed REE, biochemical, endocrine and auxological status of 12 participants with PHP1A who had normal or elevated body mass index, and used as controls a cohort of 156 obese participants.
SETTING: The Children's Hospital in Philadelphia and Sick Children's Hospital in Toronto.
PATIENTS: We assessed REE, biochemical, endocrine and auxological status of 12 participants with PHP1A who had normal or elevated body mass index, and used as controls a cohort of 156 obese participants.
MAIN OUTCOME MEASURE(S): REE as a percent of predicted REE.
RESULTS: PHP1A participants had normal endocrine status while receiving appropriate hormone replacement therapy, but had significantly decreased REE as a percent of predicted REE (using the modified Schofield equation).
CONCLUSION: Our results are consistent with reduced energy expenditure being the principle cause of obesity in PHP1A rather than it being caused by excessive energy intake or endocrine dysfunction.

PMID: 26709970 [PubMed - as supplied by publisher]

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