title: Energy expenditure in obese children with pseudohypoparathyroidism type 1a.
abstract: BACKGROUND
  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.
  
  
  METHODS
  We studied 6 obese females with PHP-1a and 17 obese female controls. Patients were recruited from a single academic center.
  
  
  METHODS
  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.
authors.affiliation: Division of Pediatric Endocrinology, Vanderbilt Children's Hospital, Nashville, TN 37232, USA. ashley.h.shoemaker@vanderbilt.edu
authors.forename: A H
authors.initials: AH
authors.lastname: Shoemaker
authors.forename: J P
authors.initials: JP
authors.lastname: Lomenick
authors.forename: B R
authors.initials: BR
authors.lastname: Saville
authors.forename: W
authors.initials: W
authors.lastname: Wang
authors.forename: M S
authors.initials: MS
authors.lastname: Buchowski
authors.forename: R D
authors.initials: RD
authors.lastname: Cone
mesh_terms: D015502:Absorptiometry, Photon; D000293:Adolescent; D017668:Age of Onset; D001481:Basal Metabolism; D001786:Blood Glucose / Q000378:metabolism*; D001823:Body Composition; D002153:Calorimetry, Indirect; D002648:Child; D004198:Disease Susceptibility; D004734:Energy Metabolism / Q000235:genetics; D005260:Female; D006442:Glycated Hemoglobin / Q000378:metabolism*; D006801:Humans; D007328:Insulin / Q000097:blood*; D063766:Pediatric Obesity / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism*; D010641:Phenotype; D020641:Polymorphism, Single Nucleotide; D019518:Postprandial Period; D011547:Pseudohypoparathyroidism / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism*; D012146:Rest; D022722:Thermogenesis; D014481:United States / Q000453:epidemiology; D015430:Weight Gain
chemical_list: D001786:Blood Glucose; D006442:Glycated Hemoglobin; D007328:Insulin; C517652:hemoglobin A1c protein, human