Objective: To assess hormonal status and morphology of ovaries in cystic fibrosis (CF) adolescents. Design: Prospective study. Setting: University teaching hospital. Patient(s): Female adolescents: 18 with CF and 18 n...Objective: To assess hormonal status and morphology of ovaries in cystic fibrosis (CF) adolescents. Design: Prospective study. Setting: University teaching hospital. Patient(s): Female adolescents: 18 with CF and 18 normal. Intervention(s): Transabdominal pelvic ultrasonography and venipuncture. Main Outcome Measure(s): Hormone profile and ultrasound examination of ovaries and uterus. Result(s): Levels of LH, LH/FSH, androstenedione, and PRL were significantly higher in the CF adolescents. Levels of sex hormone- binding globulin (SHBG) were significantly lower and had negative correlation with percentage of body fat. Percentage of body fat and body mass index were significantly lower in CF and had significant correlation. Levels of E2, FSH, T, and DHEAS were comparable in the two groups. Ultrasound revealed cysts in eight (44% ) of the CF subjects; six of these had LH/FSH > 3, and three had been operated for ovarian torsion. Nine out of all of the CF subjects (50% ) had DM. No obesity, hirsutism, or acne was observed. The Shwachman score was 87.44 ± 4.83 and correlated significantly with the percentage of body fat. Conclusion(s): Multifollicular ovaries were frequent in CF adolescents. Hormone changes characteristic of polycystic ovary syndrome were detected. The low T levels, despite low SHBG, and the absence of hirsutism or acne may be a result of a lower percentage of body fat, disturbances at the pilosebaceous-adipocyte endocrine unit, or mechanical or other causes.展开更多
Objective: Our purpose was to determine glucose tolerance in pregnant women with cystic fibrosis (CF) and to relate glucose tolerance to insulin sensitivity, hepatic glucose production, and protein turnover. Methods: ...Objective: Our purpose was to determine glucose tolerance in pregnant women with cystic fibrosis (CF) and to relate glucose tolerance to insulin sensitivity, hepatic glucose production, and protein turnover. Methods: We studied 8 CF women during pregnancy (CFPreg). Results were compared with those from 9 pregnant controls (PregCont) and 8 nonpregnant CF women (CFCont). The following metabolic studies were conducted: oral glucose tolerance test (OGTT), hyperinsulinemic euglycemic clamp, stable isotope infusion of [1- 13C]leucine and [6,6- 2H2]glucose for measurement of whole body protein turnover and hepatic glucose production (HGP), respectively. Indirect calorimetry was used to measure resting energy expenditure (REE), and food intake was measured by 3- day food journals. Fat-free mass was measured by total body potassium 40K scan. Results: All but one CFPreg developed diabetes by the end of the second trimester and had significantly lower insulin secretion and more insulin resistance than PregCont. Hepatic glucose production was significantly higher and suppression by insulin was less in CF subjects, and protein breakdown was significantly igher. Insulin resistance and HGP increased during pregnancy similarly in CFPreg and PregCont groups. Conclusion: Pregnancy in CF is associated with decreased nsulin sensitivity and high HGP, in addition to inherent decreased insulin secretion. Pregnancy in CF is also associated with increased protein turnover and less response to insulin’ s anticatabolic effect. These changes appear to predispose the pregnant CF women to early development of diabetes and poor weight gain.展开更多
文摘Objective: To assess hormonal status and morphology of ovaries in cystic fibrosis (CF) adolescents. Design: Prospective study. Setting: University teaching hospital. Patient(s): Female adolescents: 18 with CF and 18 normal. Intervention(s): Transabdominal pelvic ultrasonography and venipuncture. Main Outcome Measure(s): Hormone profile and ultrasound examination of ovaries and uterus. Result(s): Levels of LH, LH/FSH, androstenedione, and PRL were significantly higher in the CF adolescents. Levels of sex hormone- binding globulin (SHBG) were significantly lower and had negative correlation with percentage of body fat. Percentage of body fat and body mass index were significantly lower in CF and had significant correlation. Levels of E2, FSH, T, and DHEAS were comparable in the two groups. Ultrasound revealed cysts in eight (44% ) of the CF subjects; six of these had LH/FSH > 3, and three had been operated for ovarian torsion. Nine out of all of the CF subjects (50% ) had DM. No obesity, hirsutism, or acne was observed. The Shwachman score was 87.44 ± 4.83 and correlated significantly with the percentage of body fat. Conclusion(s): Multifollicular ovaries were frequent in CF adolescents. Hormone changes characteristic of polycystic ovary syndrome were detected. The low T levels, despite low SHBG, and the absence of hirsutism or acne may be a result of a lower percentage of body fat, disturbances at the pilosebaceous-adipocyte endocrine unit, or mechanical or other causes.
文摘Objective: Our purpose was to determine glucose tolerance in pregnant women with cystic fibrosis (CF) and to relate glucose tolerance to insulin sensitivity, hepatic glucose production, and protein turnover. Methods: We studied 8 CF women during pregnancy (CFPreg). Results were compared with those from 9 pregnant controls (PregCont) and 8 nonpregnant CF women (CFCont). The following metabolic studies were conducted: oral glucose tolerance test (OGTT), hyperinsulinemic euglycemic clamp, stable isotope infusion of [1- 13C]leucine and [6,6- 2H2]glucose for measurement of whole body protein turnover and hepatic glucose production (HGP), respectively. Indirect calorimetry was used to measure resting energy expenditure (REE), and food intake was measured by 3- day food journals. Fat-free mass was measured by total body potassium 40K scan. Results: All but one CFPreg developed diabetes by the end of the second trimester and had significantly lower insulin secretion and more insulin resistance than PregCont. Hepatic glucose production was significantly higher and suppression by insulin was less in CF subjects, and protein breakdown was significantly igher. Insulin resistance and HGP increased during pregnancy similarly in CFPreg and PregCont groups. Conclusion: Pregnancy in CF is associated with decreased nsulin sensitivity and high HGP, in addition to inherent decreased insulin secretion. Pregnancy in CF is also associated with increased protein turnover and less response to insulin’ s anticatabolic effect. These changes appear to predispose the pregnant CF women to early development of diabetes and poor weight gain.
