RESUMO
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
OBJECTIVES: The purpose of this study was to examine the zinc status of young infants with cystic fibrosis before and after the initiation of pancreatic enzyme therapy. STUDY DESIGN: Cross-sectional data were obtained for infants with cystic fibrosis identified by newborn screening. Plasma zinc concentrations were measured and analyzed according to enzyme use at the time of the blood draw. On a subgroup of infants, zinc concentrations were determined again after several weeks with enzyme therapy. RESULTS: Mean (+/-SD) plasma zinc concentration for the infants studied before the initiation of enzyme therapy was 10.4 +/- 2.2 micromol/L (68.3 +/- 14.7 microgram/dL) (n = 48), which was significantly lower than the mean for those receiving enzymes for >/=2 weeks, 11.8 +/- 2. 3 micromol/L (77.1 +/- 14.9 microgram/dL) (n = 15) (P =.03). For the group not yet receiving enzymes, 29% of infants had zinc concentrations in the deficient range. Data were available before and after enzyme therapy for 30 infants and indicated a mean increase of 1.64 +/- 3.0 micromol/L (10.7 +/- 19.3 microgram/dL) (P =. 005). CONCLUSIONS: These data suggest that many of the infants were zinc deficient at the time of diagnosis. We conclude that zinc should be included among the specific micronutrients given consideration in the management of cystic fibrosis, particularly in infants.
Assuntos
Fibrose Cística/sangue , Zinco/sangue , Estudos Transversais , Fibrose Cística/terapia , Humanos , Recém-Nascido , Análise dos Mínimos QuadradosRESUMO
OBJECTIVE: To determine whether differences in tolerance and absorption of fat and nitrogen exist between a semi-elemental nutritional formula without enzyme replacement and a nonelemental formula with enzyme replacement. STUDY DESIGN: Sixteen patients (eight girls and eight boys) with cystic fibrosis, 4 to 20 years of age (12+/-1.3, mean +/- SEM), who were pancreatic insufficient completed two 6-day regimens of nocturnal continuous enteral feedings offered in random order. Forty-four percent of the total daily energy was consumed as enteral tube feedings, and the remaining oral dietary intake remained constant throughout the study. Seventy-two-hour fecal collections from each study period were analyzed for total fat, long-chain fatty acids (LCFAs), medium-chain fatty acids (MCFAs), and nitrogen. RESULTS: Fat absorption was 80.2%+/-2.9% and 82.3%+/-3.1% (p = 0.58) for the semi-elemental and nonelemental formulas, respectively. Similarly, the coefficient of absorption of LCFAs was 69.5%+/-4.5% and 79.6%+/-3.4% (p = 0.30) for the semi-elemental and nonelemental formulas. Malabsorption of MCFAs contributed minimally to total fat malabsorption. There was no difference between formulas for MCFA or for nitrogen absorption, 83.7%+/-1.9% and 87.4%+/-1.4%, p = 0.48. All patients tolerated all feedings, and weight gained was 1.8+/-0.3 kg with no difference in weight gain between the trials. CONCLUSION: A nonelemental formula, with enzyme replacement, is absorbed as well as a predigested formula in patients with CF who are pancreatic insufficient.
Assuntos
Fibrose Cística/terapia , Nutrição Enteral , Alimentos Formulados , Adolescente , Adulto , Criança , Pré-Escolar , Gorduras na Dieta/metabolismo , Feminino , Humanos , Absorção Intestinal , Masculino , Nitrogênio/metabolismoRESUMO
OBJECTIVE: To determine whether there are clinical or physical factors that could be used to predict the duration of dependence on parenteral nutrition (PN) in infants who have undergone resection of small intestine in the neonatal period. STUDY DESIGN: Medical records of 44 patients who had small intestinal resection as neonates from 1985 to 1996 and who were dependent on PN for at least 3 months were reviewed. Statistical evaluation of patient variables and their impact on duration of dependence on PN were determined by using the Cox Proportional Hazard model. RESULTS: Twenty-seven patients became independent of PN before the age of 36 months. Seven patients between 40 and 129 months of age are permanently dependent on PN. Outcome could not be determined in 10 patients, four of whom died of hepatic failure while still receiving PN and six of whom are still receiving PN but are younger than 36 months of age. Small bowel length after initial surgery and the percent of daily energy intake received by the enteral route at 12 weeks' adjusted age were significantly related to the duration of dependence on PN. Gestational age, presence of the ileocecal valve, and development of cholestasis were not significantly related. With the use of the Cox Proportional Hazards survival model, a formula was generated to allow estimation of the duration of dependence on PN. CONCLUSIONS: The duration of dependence on PN can be predicted at an early age in neonatal short bowel syndrome by using two patient variables: the length of residual small bowel after initial surgery and the percent of daily energy intake tolerated through the enteral route.
Assuntos
Intestino Delgado/cirurgia , Nutrição Parenteral , Síndrome do Intestino Curto/terapia , Humanos , Lactente , Recém-Nascido , Modelos de Riscos Proporcionais , Síndrome do Intestino Curto/mortalidade , Análise de Sobrevida , Fatores de TempoRESUMO
The role of three-dimensional (3-D) treatment planning in the definitive treatment of carcinoma of the larynx with radiation was evaluated at four institutions as part of an NCI contract. A total of 30 different treatment approaches were devised for two patients with larynx cancer. CT scans were obtained for both patients and various treatment planning tools were employed to optimize beam arrangements and to evaluate the resulting dose distribution. The effect on dose distribution of a number of factors was also examined: 1) the use of dose calculation algorithms which correct for tissue inhomogeneities, 2) the variation of the CT numbers used for inhomogeneity corrections to simulate inaccuracies in the knowledge of the CT numbers, and 3) the modification of beam energy. A multitude of data was used in plan evaluation and a numerical score was given to each plan to estimate the tumor control probability and the normal tissue complication probability. We found 3-D treatment planning to be of potential value in optimizing treatment plans in larynx cancer. Improved target coverage was achieved when complete information describing 3-D geometry of the anatomy was utilized. In some cases, the treatment planning tools employed, such as the beam's eye view, helped devise novel beam arrangements which were useful alternatives to standard techniques. We found little effect of change in CT number on dose distributions. A comparison between dose distributions calculated with tissue inhomogeneity corrections to those calculated without this correction showed little difference. We did find some improvement in the dose to the primary tumor volume at lower beam energies, but with an increased larynx volume potentially receiving doses above tolerance.
Assuntos
Neoplasias Laríngeas/radioterapia , Planejamento da Radioterapia Assistida por Computador , Adulto , Humanos , Masculino , Pessoa de Meia-Idade , Dosagem Radioterapêutica , Tomografia Computadorizada por Raios XRESUMO
Three-dimensional treatment planning for the intact breast was performed on two patients who had undergone CT scanning. A total of 38 treatment plans were evaluated. Multiple plans were evaluated for each patient including plans with and without inhomogeneity corrections, plans using varying photon energies of 60Co, 4 MV, 6 MV, 10 MV, and 15 MV, and three-dimensionally unconstrained plans. Increased hot spots were appreciated in the central axis plane when lung inhomogeneity corrections were used. Additional hot spots were appreciated in off-axis planes towards the cephalad and caudad aspects of the target volume because of lung inhomogeneity corrections and changes in the breast contour. The use of 60Co was associated with an increase in the magnitude and volume of hot spots, whereas the use of higher energy photons such as 10 MV and 15 MV was associated with an unacceptable target coverage at shallow depths. Therefore, for the two patients studied, the use of a medium energy photon beam (such as from a 6 MV linear accelerator) appeared to be the energy of choice for treatment of the intact breast. The three-dimensionally unconstrained plans were able to improve slightly upon the standard plans, particularly with relationship of dose to normal tissue structures. Areas for future research were identified, including the use of tissue compensators.