Study of Metabolic Effects of Pregnancy in Women With Cystic Fibrosis

Cystic Fibrosis

cardiovascular and respiratory diseases, cystic fibrosis, genetic diseases and dysmorphic syndromes, rare disease

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.

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.

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