Objective: To assess the relationship between pancreatic enzyme therapy (PET) and the clinical outcomes of growth, abdominal pain, constipation, gassiness, and number of stools in cystic fibrosis (CF).Study design: Pa...Objective: To assess the relationship between pancreatic enzyme therapy (PET) and the clinical outcomes of growth, abdominal pain, constipation, gassiness, and number of stools in cystic fibrosis (CF).Study design: Patients (n = 1215) >4 weeks of age from 33 Cystic Fibrosis Foundation accredited sites who had a sweat chloride >60 mmol/L or two CF-causing mutations were enrolled using a proportionate sampling strategy in a nonblinded study.Patients submitted a stool sample and completed a questionnaire.The study coordinator also completed a questionnaire for each patient.Enzyme dosing and growth, abdominal pain, gassiness, constipation, and number of stools were compared.Results: Of the 1215 enrolled patients, 1131 (93.1%) were prescribed PET.Only 14.9%had pancreatic function assessed before enrolling in this study.Stool elastase-1 analysis identified 1074 (89%) patients as pancreatic insufficient (PI).There was no association between PET and the outcomes: growth, abdominal pain, gassiness, constipation, and number of stools.Conclusion: PET dose is not correlated with growth or gastrointestinal symptoms.More sensitive outcome measures of the effectiveness of PET in patients with CF are needed to guide treatment of PI.展开更多
Background: Ultraviolet B (UVB) radiation can be used in the prevention and treatment of vitamin D deficiency. Aim: To investigate, in a controlled study of patients with cystic fibrosis (CF), whether regular UVB radi...Background: Ultraviolet B (UVB) radiation can be used in the prevention and treatment of vitamin D deficiency. Aim: To investigate, in a controlled study of patients with cystic fibrosis (CF), whether regular UVB radiation would improve serum levels of calcidiol during the dark season (October-April). Methods: Thirty patients with mild to moderate disease were included (aged 9-40 y). All patients had cholecalciferol supplementation. One group (15 patients) was given UVB oneto three times a week for 6 mo and one group (15 sex-and age-matched patients) served as controls. The radiation source consisted of three TL 12/40W UVB fluorescent lamps. Initial treatment duration was 1 min, subsequently increased by 0.5-1 min/treatment to a maximum of 10 min. Results: The mean initial serum calcidiol levels were 21 ng/ml in the controls and 22 ng/ml in the intervention group. Serum calcidiol levels increased to 44 ng/ml after 8 wk and to 50 ng/ml after 24 wk of UVB radiation; the corresponding serum levels in the controls were 19 and 25 ng/ml, respectively. The mean serum calcitriol levels increased in the treated group and were unaltered in the control group. Conclusions: UVB radiation was effective in increasing vitamin D levels in patients with CF. The study resultsimply that UVB radiation is valuable in chronic conditions associated with vitamin D deficiency.展开更多
Objective:To evaluate risk factors for recurrent thrombosis in pediatric patients. Study design:This prospective observational cohort study enrolled 120 patients with acute venous thromboembolism from January 2003 to ...Objective:To evaluate risk factors for recurrent thrombosis in pediatric patients. Study design:This prospective observational cohort study enrolled 120 patients with acute venous thromboembolism from January 2003 to April 2005. Datacollection included medical and family history,radiologic and laboratory studies,therapy,and follow-up. Results:The overall prevalence of recurrent thrombosis in our cohort was 19/120(15.8%). Patients with recurrence were older,with a median age of 14.8 years (range 2 weeks-23.6 years),compared with 10.1years (range newborn 23.4 years) in patients without recurrence(P = 0.03). Six of the 19 patients with recurrent thrombosis had cystic fibrosis (CF),compared with 0/101without recurrence (P< 0.001). Five of these 6 patients were colonized with Burkholderia cepacia in their sputum. Central venous catheters were associated with most,but not all,of the thromboses in patients with CF. Conclusions:In this study,patients with CF had a high risk of recurrent venous thrombosis,as well as a high prevalence of colonization with B cepacia. The cause of this risk has not been defined. This observation may have important implications for thromboprophylaxis,particularly in the setting of central venous catheters.展开更多
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
文摘Objective: To assess the relationship between pancreatic enzyme therapy (PET) and the clinical outcomes of growth, abdominal pain, constipation, gassiness, and number of stools in cystic fibrosis (CF).Study design: Patients (n = 1215) >4 weeks of age from 33 Cystic Fibrosis Foundation accredited sites who had a sweat chloride >60 mmol/L or two CF-causing mutations were enrolled using a proportionate sampling strategy in a nonblinded study.Patients submitted a stool sample and completed a questionnaire.The study coordinator also completed a questionnaire for each patient.Enzyme dosing and growth, abdominal pain, gassiness, constipation, and number of stools were compared.Results: Of the 1215 enrolled patients, 1131 (93.1%) were prescribed PET.Only 14.9%had pancreatic function assessed before enrolling in this study.Stool elastase-1 analysis identified 1074 (89%) patients as pancreatic insufficient (PI).There was no association between PET and the outcomes: growth, abdominal pain, gassiness, constipation, and number of stools.Conclusion: PET dose is not correlated with growth or gastrointestinal symptoms.More sensitive outcome measures of the effectiveness of PET in patients with CF are needed to guide treatment of PI.
文摘Background: Ultraviolet B (UVB) radiation can be used in the prevention and treatment of vitamin D deficiency. Aim: To investigate, in a controlled study of patients with cystic fibrosis (CF), whether regular UVB radiation would improve serum levels of calcidiol during the dark season (October-April). Methods: Thirty patients with mild to moderate disease were included (aged 9-40 y). All patients had cholecalciferol supplementation. One group (15 patients) was given UVB oneto three times a week for 6 mo and one group (15 sex-and age-matched patients) served as controls. The radiation source consisted of three TL 12/40W UVB fluorescent lamps. Initial treatment duration was 1 min, subsequently increased by 0.5-1 min/treatment to a maximum of 10 min. Results: The mean initial serum calcidiol levels were 21 ng/ml in the controls and 22 ng/ml in the intervention group. Serum calcidiol levels increased to 44 ng/ml after 8 wk and to 50 ng/ml after 24 wk of UVB radiation; the corresponding serum levels in the controls were 19 and 25 ng/ml, respectively. The mean serum calcitriol levels increased in the treated group and were unaltered in the control group. Conclusions: UVB radiation was effective in increasing vitamin D levels in patients with CF. The study resultsimply that UVB radiation is valuable in chronic conditions associated with vitamin D deficiency.
文摘Objective:To evaluate risk factors for recurrent thrombosis in pediatric patients. Study design:This prospective observational cohort study enrolled 120 patients with acute venous thromboembolism from January 2003 to April 2005. Datacollection included medical and family history,radiologic and laboratory studies,therapy,and follow-up. Results:The overall prevalence of recurrent thrombosis in our cohort was 19/120(15.8%). Patients with recurrence were older,with a median age of 14.8 years (range 2 weeks-23.6 years),compared with 10.1years (range newborn 23.4 years) in patients without recurrence(P = 0.03). Six of the 19 patients with recurrent thrombosis had cystic fibrosis (CF),compared with 0/101without recurrence (P< 0.001). Five of these 6 patients were colonized with Burkholderia cepacia in their sputum. Central venous catheters were associated with most,but not all,of the thromboses in patients with CF. Conclusions:In this study,patients with CF had a high risk of recurrent venous thrombosis,as well as a high prevalence of colonization with B cepacia. The cause of this risk has not been defined. This observation may have important implications for thromboprophylaxis,particularly in the setting of central venous catheters.
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.
