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Study of Metabolic Effects of Pregnancy in Women With Cystic Fibrosis

2014-07-23 21:56:00 | BioPortfolio

Summary

OBJECTIVES: I. Compare the clinical status of pregnant vs non-pregnant women with cystic fibrosis.

II. Determine glucose tolerance during each trimester of pregnancy in these women.

III. Evaluate peripheral insulin sensitivity in these women. IV. Evaluate whole body protein turnover and hepatic glucose production in these women.

V. Determine resting energy expenditure in these women.

Description

PROTOCOL OUTLINE: Patients undergo a glucose tolerance test over 3 hours and a potassium body scan on day 1. Patients undergo an indirect calorimetry over 20 minutes on day 2. Patients receive stable-labeled leucine and stable-labeled glucose IV over 3 hours followed by insulin and glucose IV over 4-4.5 hours on day 2 using the hyperinsulinemic euglycemic clamp technique.

Blood and breath samples are collected to measure glucose tolerance, peripheral insulin sensitivity, and whole body protein turnover. Hepatic glucose production is measured by mass spectrophotometry.

Patients maintain a 3-day food journal before pregnancy, during each trimester, and after pregnancy.

Patients undergo each study during the final 2 weeks of each trimester of pregnancy and then at 6 months post-partum.

Study Design

Observational Model: Natural History

Conditions

Cystic Fibrosis

Intervention

glucose, insulin, leucine

Location

University of Utah School of Medicine
Salt Lake City
Utah
United States
84132

Status

Terminated

Source

National Center for Research Resources (NCRR)

Results (where available)

View Results

Links

Published on BioPortfolio: 2014-07-23T21:56:00-0400

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Medical and Biotech [MESH] Definitions

An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.

A chloride channel that regulates secretion in many exocrine tissues. Abnormalities in the CFTR gene have been shown to cause cystic fibrosis. (Hum Genet 1994;93(4):364-8)

A strain of mice widely studied as a model for cystic fibrosis. These mice are generated from embryonic stem cells in which the CFTR (cystic fibrosis transmembrane conductance regulator) gene is inactivated by gene targeting. As a result, all mice have one copy of this altered gene in all their tissues. Mice homozygous for the disrupted gene exhibit many features common to young cystic fibrosis patients, including failure to thrive, meconium ileus, and alteration of mucous and serous glands.

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Maintenance of a constant blood glucose level by perfusion or infusion with glucose or insulin. It is used for the study of metabolic rates (e.g., in glucose, lipid, amino acid metabolism) at constant glucose concentration.

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