Objectives: Impaired longitudinal growth and poor weight gain are common and important problems in children with cystic fibrosis. This study evaluates the hy pothesis that adjunctive growth hormone (GH) therapy augmen...Objectives: Impaired longitudinal growth and poor weight gain are common and important problems in children with cystic fibrosis. This study evaluates the hy pothesis that adjunctive growth hormone (GH) therapy augments the growth respons e to nutritional supplementation. Study design: We recruited 18 prepubertal chil dren who received enteral nutritional supplementation for at least 2 years befor e enrollment. Nine were randomly assigned to receive no GH for 1 year, followed by 1 year of GH. Nine were randomly assigned to receive 1 year of GH followed by a second year of GH. Measurements included height, weight, pulmonary function, lean tissue mass, bone mineral content, hospitalizations, outpatient antibiotic use, and caloric intake. Results: Growth hormone resulted in significant improve ment in height, weight, bone mineral content, lean tissue mass, and number of ho spitalizations. Pulmonary function was similar at baseline. Absolute forced vita l capacity and forced expiratory volume in 1 minute significantly increased in G H treatment, but there was no significant change in percent predicted pulmonary function. Caloric intake was similar in both groups during both years. Conclusio ns: These results suggest that GH is a useful for enhancing growth in children w ith cystic fibrosis receiving enteral nutritional supplementation.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Objectives: Impaired longitudinal growth and poor weight gain are common and important problems in children with cystic fibrosis. This study evaluates the hy pothesis that adjunctive growth hormone (GH) therapy augments the growth respons e to nutritional supplementation. Study design: We recruited 18 prepubertal chil dren who received enteral nutritional supplementation for at least 2 years befor e enrollment. Nine were randomly assigned to receive no GH for 1 year, followed by 1 year of GH. Nine were randomly assigned to receive 1 year of GH followed by a second year of GH. Measurements included height, weight, pulmonary function, lean tissue mass, bone mineral content, hospitalizations, outpatient antibiotic use, and caloric intake. Results: Growth hormone resulted in significant improve ment in height, weight, bone mineral content, lean tissue mass, and number of ho spitalizations. Pulmonary function was similar at baseline. Absolute forced vita l capacity and forced expiratory volume in 1 minute significantly increased in G H treatment, but there was no significant change in percent predicted pulmonary function. Caloric intake was similar in both groups during both years. Conclusio ns: These results suggest that GH is a useful for enhancing growth in children w ith cystic fibrosis receiving enteral nutritional supplementation.
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.
