Assuntos
Asma/tratamento farmacológico , Dexametasona/administração & dosagem , Glucocorticoides/administração & dosagem , Prednisona/administração & dosagem , Doença Aguda , Administração Oral , Criança , Dexametasona/uso terapêutico , Glucocorticoides/uso terapêutico , Humanos , Prednisona/uso terapêutico , Fatores de TempoRESUMO
OBJECTIVE: To prospectively evaluate the biochemical status of vitamins A, D, and E in children with cystic fibrosis (CF). SUBJECTS: A total of 127 infants identified by the Colorado CF newborn screening program. DESIGN: Vitamin status (serum retinol, 25-hydroxy vitamin D, ratio of alpha-tocopherol/total lipids) and serum albumin were assessed at diagnosis (4 to 8 weeks), ages 6 months, 12 months, and yearly thereafter, to age 10 years. RESULTS: Deficiency of 1 or more vitamins was present in 44 (45.8%) of 96 patients at age 4 to 8 weeks as follows: vitamin A 29.0%, vitamin D 22.5%, and vitamin E 22.8%. Of these patients with initial deficiency, the percent that was deficient at 1 or more subsequent time points, despite supplementation, was vitamin A 11.1%, vitamin D 12.5%, and vitamin E 57.1%. Of the initial patients with vitamin sufficiency, the percent who became deficient at any time during the 10-year period was as follows: vitamin A 4.5%, vitamin D 14.4%, and vitamin E 11.8%. The percent of patients deficient for 1 or more vitamins ranged from 4% to 45% for any given year. CONCLUSIONS: Despite supplementation with standard multivitamins and pancreatic enzymes, the sporadic occurrence of fat-soluble vitamin deficiency and persistent deficiency is relatively common. Frequent and serial monitoring of the serum concentrations of these vitamins is therefore essential in children with CF.
Assuntos
Fibrose Cística/metabolismo , Deficiência de Vitamina A/epidemiologia , Deficiência de Vitamina D/epidemiologia , Deficiência de Vitamina E/epidemiologia , Criança , Pré-Escolar , Fibrose Cística/diagnóstico , Fibrose Cística/tratamento farmacológico , Seguimentos , Fármacos Gastrointestinais/uso terapêutico , Humanos , Lactente , Recém-Nascido , Triagem Neonatal , Pancrelipase/uso terapêutico , Estudos Prospectivos , Fatores de Tempo , Vitamina A/sangue , Deficiência de Vitamina A/diagnóstico , Vitamina D/sangue , Deficiência de Vitamina D/diagnóstico , Vitamina E/sangue , Deficiência de Vitamina E/diagnóstico , Vitaminas/uso terapêuticoRESUMO
The purpose of this study was to assess the delivery to the lungs and the short-term safety of recombinant human deoxyribonuclease (rhDNase, Pulmozyme) in children with cystic fibrosis younger than 5 years of age compared with older children. Patients between the ages of 3 months and 10 years had bronchoscopic examination with bronchoalveolar lavage (BAL) after administration of an aerosol dose of 2.5 mg of rhDNase. After recovery from the procedure, patients were discharged home for an additional 13 days of rhDNase therapy. During this time adverse events were recorded to assess short-term safety. A total of 98 patients were enrolled, 65 (66%) aged 3 months to 5 years and 33 (34%) aged 5 years to 10 years. Deoxyribonuclease concentrations in BAL fluid were variable (interquartile range, 752 to 3943 micrograms/mL epithelial lining fluid [ELF]) and did not depend on patient age, weight, or height or differ when delivered through a mouthpiece or mask. The median value for the BAL DNA concentration in the younger group was 432 micrograms/mL ELF compared with 703 micrograms/mL ELF in the older patients. This study demonstrates the value of bronchoscopy and BAL for assessing nebulized medication delivery in young children and shows that aerosolized medications can be delivered to and are present in comparable amounts in the lower airways of younger and older children. Exposure to rhDNase appears to be safe over 2 weeks in infants and young children with cystic fibrosis.
Assuntos
Broncoscopia/métodos , Fibrose Cística/diagnóstico , Fibrose Cística/tratamento farmacológico , Desoxirribonucleases/efeitos adversos , Aerossóis , Fatores Etários , Anticorpos Catalíticos , Formação de Anticorpos/imunologia , Lavagem Broncoalveolar/métodos , Criança , Pré-Escolar , Desoxirribonucleases/imunologia , Feminino , Seguimentos , Humanos , Lactente , Masculino , Nebulizadores e VaporizadoresRESUMO
To determine whether pulmonary function in infants with asymptomatic cystic fibrosis is related to genotype, we studied 18 infants with cystic fibrosis identified through neonatal screening and 18 healthy control infants. Infants with cystic fibrosis (mean age, 2.0 months; range, 1.0 to 4.6 months) were identified from June 1990 to September 1991 through a statewide screening program based on elevated immunoreactive trypsinogen concentrations. Pulmonary function testing was done before and after inhalation of albuterol. There were no differences in pulmonary function between the cystic fibrosis group and the control infants (mean age, 2.7 months; range, 0.9 to 4.5 months). However, infants homozygous for the delta F508 deletion (n = 10) differed from infants with other genotypic variants of cystic fibrosis and control infants with respect to respiratory system resistance (79.4 +/- 11.5 vs 52.0 +/- 3.8 vs 55.5 +/- 5.0 cm H2O/L per second, respectively; p = 0.04) and specific conductance (0.15 +/- 0.02 vs 0.21 +/- 0.02 vs 0.21 +/- 0.02 cm H2O-1 sec-1, respectively; p = 0.02). Infants homozygous for the delta F508 deletion, but not other infants, responded to albuterol with a decrease in respiratory system resistance. We conclude that infants with asymptomatic cystic fibrosis homozygous for the delta F508 deletion have early evidence of airways obstruction and may need early respiratory treatment.
Assuntos
Fibrose Cística/genética , Pulmão/fisiopatologia , Triagem Neonatal , Fibrose Cística/epidemiologia , Fibrose Cística/fisiopatologia , Feminino , Genótipo , Humanos , Lactente , Recém-Nascido , Masculino , Testes de Função Respiratória , Fatores de RiscoRESUMO
Using a commercially available computer-based analysis of ventilation and esophageal pressure (PeDs), we studied 11 healthy term neonates with serial pulmonary mechanics measurements during the first 3 days of life to determine the intrasubject variability of repeated measurements. Three consecutive pulmonary function tests were obtained before and after repositioning of the esophageal catheter, for a total of six measurements per day in each subject. The daily coefficient of variation for these 11 subjects ranged from 6% to 32% for respiratory rate; 6% to 27% for tidal volume; 3% to 28% for specific dynamic compliance, and 11% to 69% for pulmonary resistance. Repositioning the esophageal catheter did not produce significant differences in measurements of pulmonary mechanics (p greater than 0.05). We conclude that within a given subject, the maximum variability (mean + 2 SD) was 28% for specific dynamic compliance and 56% for pulmonary resistance. These intrasubject variability limits are important when one is interpreting pulmonary mechanics measurements in neonates before and after specific treatment, such as bronchodilators, diuretics, surfactant, or steroids.