Clinical Trial: Energy Expenditure and Body Composition in Pseudohypoparathyroidism 1a

Study Status: Active, not recruiting
Recruit Status: Active, not recruiting
Study Type: Observational

Official Title: Altered Resting Energy Expenditure as a Cause of Obesity in Pseudohypoparathyroidism 1a: A Pilot Study

Brief Summary: The investigators would like to learn more about the metabolic consequences of pseudohypoparathyroidism type 1a in children, adolescents and adults with this condition. People with pseudohypoparathyroidism 1a are at risk for development of obesity. To better understand the cause of overweight or obesity, investigators are measuring body composition and resting energy expenditure (REE), which is the amount of calories burned while completely at rest. The investigators also want to determine the amount of body fat.

Detailed Summary:

Pseudohypoparathyroidism 1a (PHP1a) is a disorder that is associated with many endocrine problems. People with PHP1a are at risk for the development of obesity. The objective of the study will help determine if obesity is related to abnormalities energy expenditure, meaning that people with PHP1a may not burn as many calories while at rest as those without the disorder.

In order to further evaluate obesity in PHP1a, investigators are planning to measure resting energy expenditure (REE), which is the amount of calories burned while completely at rest. Investigators will also evaluate body composition by looking at measures of growth and development and determining the amount of body fat using dual energy x-ray absorptiometry (DXA) as well as blood and urine biologic markers of obesity. The investigators plan to evaluate people with PHP1a at all weights.


Sponsor: Children's Hospital of Philadelphia

Current Primary Outcome: Resting Energy Expenditure [ Time Frame: 30 minutes ]

The subject will rest for 30 minutes in the lab prior to the start of the test. A 60-minute resting energy expenditure (REE) test will be performed between 7:00 and 10:00 A.M. with the subject resting quietly under a clear, plastic hood watching a videotape.


Original Primary Outcome: Resting Energy Expenditure [ Time Frame: 30 minutes ]

The subject will rest for 30 minutes in the lab prior to the start of the test. A 60-minute REE test will be performed between 7:00 and 10:00 AM with the subject resting quietly under a clear, plastic hood watching a videotape.


Current Secondary Outcome: Characterize body composition in patients with PHP1a. [ Time Frame: 12 months ]

Body composition outcomes will be characterized by whole body lean mass and fat mass sex- and race-specific z-scores relative to height.


Original Secondary Outcome: Same as current

Information By: Children's Hospital of Philadelphia

Dates:
Date Received: July 19, 2011
Date Started: June 2011
Date Completion: April 2018
Last Updated: May 1, 2017
Last Verified: May 2017