Protein metabolism in cystic fibrosis: responses to malnutrition and taurine supplementation

Increased protein breakdown has been cited as an important cause of nutrient loss in cystic fibrosis (CF). Taurine deficiency, which is common in CF, may contribute to the increased breakdown. The occurrence of and the benefit of taurine supplementation to abnormal protein metabolism in apparently optimally treated CF were assessed using a 12-mo double-blind crossover technique in 14 well-nourished and seven mildly-moderately malnourished infection-free preadolescent CF children. Muscle protein breakdown (urinary 3-methylhistidine technique) was significantly decreased in well-nourished (1.35% degraded/24 h +/- 0.15, p less than 0.05) and malnourished (1.24 +/- 0.11, p less than 0.001) CF children compared with controls (1.50 +/- 0.17, n = 13). Whole-body protein flux, synthesis, and catabolism ([15N]-glycine technique) were similar in all groups. Net protein gain was greater in CF children, particularly those who were well-nourished (0.55 g/(kg X 10 h) +/- 0.35, p less than 0.01) compared with controls (0.16 +/- 0.26). Taurine supplementation did not significantly affect any of the indices. In the absence of infection, protein metabolism in CF children responds appropriately to malnutrition.     

The acute-phase protein response to infection in edematous and nonedematous protein-energy malnutrition

BACKGROUND: Immune structure and function are more compromised in edematous protein-energy malnutrition (PEM) than in nonedematous PEM. Whether the positive acute-phase protein (APP) response to infection is affected remains unknown. OBJECTIVE: We assessed whether children with edematous PEM can mount a general APP response and compared the kinetic mechanisms of the response in children with edematous PEM with those in children with nonedematous PEM. DESIGN: Plasma C-reactive protein, alpha(1)-acid glycoprotein, alpha(1)-antitrypsin, haptoglobin, and fibrinogen concentrations and the fractional and absolute synthesis rates of alpha(1)-antitrypsin, haptoglobin, and fibrinogen were measured in 14 children with edematous PEM, aged 11.4 +/- 2 mo, and 9 children with nonedematous PEM, aged 10.1 +/- 1.4 mo, at 3 times: approximately 2 d after hospital admission (period 1), when they were malnourished and infected; approximately 8 d after admission (period 2), when they were malnourished but free of infection; and approximately 54 d after admission (period 3), when they had recovered. RESULTS: Children with edematous and nonedematous PEM had higher plasma concentrations of 4 of 5 APPs in period 1 than in period 3. The magnitude of the difference in concentration and in the rate of synthesis of the individual APPs was less in the children with edematous PEM than in those with nonedematous PEM. The kinetic data show that the characteristics of the APP response were different in the 2 groups. CONCLUSIONS: These results suggest that severely malnourished children can mount only a partial APP response to the stress of infection and that the magnitude of this response is less in those with edema.     

Serum proteinase inhibitors and other serum proteins in protein-energy malnutrition.

1. The concentrations of serum protein albumin, prealbumin and transferrin were determined in twenty-eight cases of protein-energy malnutrition (PEM) with infection, together with the levels of serum proteinase inhibitors (PI), alpha1-antitrypsin (AT), alpha1-antichymotrypsin (Ach), alpha2-macroglobulin (alpha2M) and inter-alpha-trypsin inhibitor (IalphaI). 2. Albumin, prealbumin and transferrin concentrations, as well as the levels of PI, IalphaI and alpha2M were found to be lower in cases of PEM associated with infection than the corresponding values for a group of healthy Thai preschool children and a group of newborn Thai children, but despite starvation AT and Ach values generally were increased. 3. The results provide support for the hypothesis that PI, especially AT and Ach might limit the synthesis of albumin, prealbumin and transferrin in PEM associated with infection, via the inhibition of the mobilization of body's own protein.     

Short-term nutritional supplementation during management of pulmonary exacerbations in cystic fibrosis: a controlled study, including effects of protein turnover

Effects of nutritional supplements on minimizing weight loss and abnormalities of protein turnover during pulmonary exacerbations in cystic fibrosis (CF) were studied by controlled trial. Patients received pulmonary therapy and either standard diet (n = 10) or adjunctive enteral supplements (n = 12). Initial protein turnover, measured by [15N]glycine kinetics, showed alterations of protein synthesis (P Syn) and catabolism (P Cat), which correlated with the degree of underweight, and negligible net protein deposition (P Dep). With treatment both groups had significant increases in mean body weight and forced expiratory volume in 1 s, expressed as percent predicted value for height (FEV1) by 3 wk, but a significant correlation between initial underweight and subsequent weight gain was observed only in supplemented patients. Mean P Syn and P Dep increased significantly (p less than 0.001) only in the supplemented group. Pulmonary exacerbations in CF have important adverse effects on body-protein metabolism, similar to changes in protein-energy malnutrition and infection. These effects are reversed by short-term nutritional support. Strategic nutritional intervention should thus be considered in management, especially in malnourished patients.     

Can a reward-based behavioural test be used to investigate the effect of protein-energy malnutrition on hippocampal function?

Our laboratory is investigating the effects of protein-energy malnutrition (PEM) on cognitive outcome following global ischemia. Here, we investigated whether PEM independently impairs working memory in the T-maze and if the associated food reward reverses PEM. Gerbils were fed 12.5% (control diet) or 2% protein. A loss of body weight (20.1%) in the 2% protein group and decreased food intake and serum albumin concentration compared to controls (17.5% and 18.2%, respectively) indicated that PEM was achieved. Based on T-maze criterion frequently used in ischemia studies, no difference was observed in the mean (+/- SEM) number of trials required (control 5.2 +/- 0.7; PEM 4.9 +/- 0.4; p = 0.758) or the number of animals reaching criterion (control 10/12; PEM 12/12; p = 0.140). Using more stringent criterion, PEM animals required fewer trials (control 7.3 +/- 0.7; PEM 5.4 +/- 0.4; p = 0.035), and more reached criterion (control 8/12; PEM 12/12; p = 0.028). PEM may increase motivation to obtain a food reward.     

Malnutrition in critically ill infants and children

The prevalences of acute and chronic protein-energy malnutrition (PEM) and deficiencies in stores of fat and somatic protein have not been previously examined in pediatric intensive care unit. One hundred eight nutritional assessments were performed using anthropometric techniques on infants and children in a multidisciplinary intensive care unit. Overall, the prevalence of acute PEM was 19% and chronic PEM was 18%. The prevalence of fat store depletion was 14% and somatic protein store depletion was 21%. In general, children less than 2 years had poorer nutritional status compared to children greater than 2 years. There was not a statistically significant difference between medical and surgical patients. It is concluded that PEM and deficiencies in the macronutrient stores of fat and somatic protein are common in critically ill infants and children.     

Short-term protein intake and stimulation of protein synthesis in stunted children with cystic fibrosis

BACKGROUND: Stunted children with cystic fibrosis (CF) have less net protein anabolism than do children without CF, and the result is retarded growth in the CF patients. It is not known whether protein intake above that recommended by the Cystic Fibrosis Foundation would further stimulate whole-body protein synthesis. OBJECTIVE: We studied the effects of 3 amounts of protein intake on whole-body protein synthesis and breakdown by using isotopic infusion of [1-(13)C]valine and [(15)N(2)]urea in children with stable CF who required tube feeding. DESIGN: In 8 pediatric CF patients, we administered 3 randomly allocated isocaloric diets with normal (NP), intermediate (IP), and high (HP) amounts of protein (1.5, 3, and 5 g . kg(-1) . d(-1), respectively) by continuous drip feeding during a 4-d period at 6-wk intervals. Each patient acted as his or her own control. On the fourth day of feeding, whole-body protein synthesis and breakdown were measured. RESULTS: Protein synthesis was significantly higher in the HP group (x +/- SEM: 1.78 +/- 0.07 micromol . kg(-1) . min(-1)) than in the IP (1.57 +/- 0.08 micromol . kg(-1) . min(-1); P=0.001) and NP (1.37 +/- 0.07 micromol . kg(-1) . min(-1); P < 0.001) groups. There were no significant differences in protein breakdown. Net retention of nitrogen was significantly higher in the HP group (12.93 +/- 1.42 micromol . kg(-1) . min(-1)) than in the IP (7.61 +/- 1.40 micromol . kg(-1) . min(-1); P=0.01) and HP (2.48 +/- 0.20 micromol . kg(-1) . min(-1); P < 0.001) groups. CONCLUSION: In stunted children with CF requiring tube feeding, the highest stimulation of whole-body protein synthesis was achieved with a short-term dietary protein intake of 5 g . kg(-1) . d(-1).     

Response of splanchnic and whole-body leucine kinetics to treatment of children with edematous protein-energy malnutrition accompanied by infection

BACKGROUND: Although the reduction in whole-body protein turnover and net protein loss induced by protein-energy malnutrition (PEM) has been well documented, it is unclear whether the protein-sparing mechanisms elicited by chronically inadequate intakes of dietary protein and energy are affected by the protein catabolic response to infection. OBJECTIVE: The objective of this study was to determine whether the presence of infection alters the PEM-induced reduction in whole-body protein metabolism. DESIGN: We determined whole-body leucine kinetics in 4 boys and 3 girls aged 6-15 mo with edematous PEM and infection approximately 3 d after admission (study 1), when they were both infected and malnourished; approximately 11 d after admission (study 2), when infection had resolved but they were still anthropometrically malnourished; and at recovery (study 3), when weight-for-length was at least 90% of that expected. RESULTS: The children had significantly less leucine flux in both study 1 and study 2 than they had in study 3. There were no significant differences in the amount of leucine released from protein breakdown or used for protein synthesis between study 1 and study 2. There were no significant differences in leucine balance or in either the amount or percentage of enteral leucine extracted by the splanchnic tissues among the 3 studies. CONCLUSIONS: When subjects are in the fed state, severe PEM induces a marked reduction in whole-body protein synthesis and breakdown rates, and the presence of infection does not alter this adaptation and hence the overall protein balance. A corollary is that children with severe PEM do not mount a protein catabolic response to infection.     

Energy expenditure and the body cell mass in cystic fibrosis

Poor nutritional status in patients with cystic fibrosis (CF) is associated with severe lung disease, and possible causative factors include inadequate intake, malabsorption, and increased energy requirements. Body cell mass (which can be quantified by measurement of total body potassium) provides an ideal standard for measurements of energy expenditure. The aim of this study was to compare resting energy expenditure (REE) in patients with CF with both predicted values and age-matched healthy children and to determine whether REE was related to either nutritional status or pulmonary function. REE was measured by indirect calorimetry and body cell mass by scanning with total body potassium in 30 patients with CF (12 male, mean age = 13.07 +/- 0.55 y) and 18 healthy children (six male, mean age = 12.56 +/- 1.25 y). Nutritional status was expressed as a percentage of predicted total body potassium. Lung function was measured in the CF group by spirometry and expressed as the percentage of predicted forced expiratory volume in 1 s. Mean REE was significantly increased in the patients with CF compared with healthy children (119.3 +/- 3.1% predicted versus 103.6 +/- 5% predicted, P < 0.001) and, using multiple regression techniques, REE for total body potassium was significantly increased in patients with CF (P = 0.0001). There was no relation between REE and nutritional status or pulmonary disease status in the CF group. In conclusion, REE is increased in children and adolescents with CF but is not directly related to nutritional status or pulmonary disease.     

小儿单纯性营养不良胃肠激素测定

【目的】　探讨单纯性营养不良 (protein energymalnutrition ,PEM )患儿胃肠内分泌功能 ,为PEM的治疗提供依据。　【方法】　采用放射免疫分析法测定空腹和摄入液体实验餐后 60min时外周血胃泌素 (gastrin ,Gas)、胃动素 (motilin ,MTL)、生长抑素 (somatostatin ,SS)水平。　【结果】　与对照组比较 ,观察组空腹和餐后 60min血中Gas和MTL水平明显降低 ,而SS水平则明显升高 (P均 <0 .0 1)。与对照组相似 ,观察组患儿餐后与餐前比较 ,Gas及MTL水平差异无显著性 (P >0 .0 5 ) ,SS水平则明显升高 (P <0 .0 1)。　【结论】　PEM患儿存在明显的胃肠内分泌功能紊乱。     

Malnutrition, nutritional indices, and early enteral feeding in critically ill children.

We measured the incidences of protein and fat depletions and the frequencies of acute and chronic protein-energy malnutrition during stress states in children and investigated the influence of early enteral feeding on nutrition indices and acute-phase proteins. Seventy-one, consecutively enrolled, critically ill children received early enteral feeding (energy intakes equal to 0.50, 1, 1.25, 1.5, and 1.5 of the predicted basal metabolic rates on days 1 through 5, respectively) through nasogastric tubes. On the first day of the study, 16.7% of the patients already were depleted of protein and 31% of fat stores. Overall, 16.9% were at risk for chronic protein-energy malnutrition and 21.1% for acute protein-energy malnutrition, whereas 4.2% and 5.6% already had chronic and acute, respectively protein-energy malnutrition. Only 22.7% of patients without protein deficiencies versus 37% of those at risk or already deficient developed multiple-organ system failure. Transferrin and prealbumin levels improved at the end of the period of early enteral feeding (187 +/- 6.6 versus 233 +/- 7 mg/dL, P < 0.0001; 15.1 +/- 2 versus 21.9 +/- 2.9 mg/dL, P < 0.0001; respectively); survivors had higher prealbumin levels than non-survivors (22.3 versus 15.5 mg/dL). With logistic regression analysis, only repleted energy, not anthropometric or nutrition indices, was independently associated with survival (P = 0.05). These results reinforce the observation that critically ill children are at risk for fat or protein depletion and development of malnutrition, which is associated with increased morbidity and mortality. We conclude that early enteral nutrition improves nutrition indices and outcomes.     

Anthropometry of the trunk and extremities in nutritional assessment of children with chronic renal failure.

OBJECTIVE: Comparison of anthropometric parameters of the trunk and extremities between 2 groups of children with chronic renal failure (CRF) with different levels of nutritional status and healthy controls. DESIGN: A prospective cross-sectional study. SETTINGS: Department of Physiology and Anthropology, University Ss. Cyril and Methodius, Skopje. PATIENTS: Twenty-one patients with a mean age of 10.5 +/- 3.2 years (10 boys and 11 girls) with mild to moderate CRF were divided into well-nourished (chronic renal failure children [CRFC] I) and undernourished (CRFC II) children according to their nutritional status. The control group was 22 healthy children with a mean age of 10.7 +/- 3.8 years (10 boys and 12 girls). Nutritional status of the children was assessed by comparing anthropometric parameters with National Center for Health Statistics references. INTERVENTIONS: Anthropometry. Main Outcome Measurements: Body weight, height, sitting height, arm and leg length, knee height, chest circumference, midarm circumference, triceps skin-fold thickness, and upper arm muscle and fat area. RESULTS: Mean sitting height, leg length, and chest circumference were significantly lower in both the CRFC I group (67.8 +/- 3.5, 57.5 +/- 4.2, and 53.0 +/- 3.9 cm) and the CRFC II group (65.6 +/- 2.2, 56.1 +/- 4.1, and 50.6 +/- 2.7 cm) compared with the healthy controls (72.5 +/- 2.9, 61.3 +/- 3.5, and 62.4 +/- 4.1 cm, respectively). The highest significant correlations to height were for sitting height (r = +0.82, P <.05) and knee height (r = +0.72, P <.05) in CRFC I and for leg length (r = +0.74, P <.05) in CRFC II. There was no correlation between anthropometric parameters of the trunk and extremities and upper muscle and fat area in both groups of CRF children. CONCLUSION: The sitting height, knee height, and leg length can be used in nutritional and growth assessment when it is impossible to make a reliable measurement of height in CRF children.     

Total free amino acids, ammonia, and protein in the sweat of children.

The concentrations of total free amino acids, ammonia and protein in sweat of 44 children, 20 males, and 24 females, 6 to 8 years old, were determined. The children were divided into three groups according to their height, weight, and body weight/height ratio; group I children had heights and weights below the 10th percentile for their age, group II had heights and weights between the 10th and 90th percentile, and group III had heights and weights above the 90th percentile. Sweating was induced by allowing the child to be moderatley active. The temperature at the time of the experiment was 31 to 33 C and the relative humidity 48%. The mean concentrations of total free amino acids (mg/100 ml) in sweat from groups I, II, and III children were 318.9 +/- 52.5, 293.9 +/- 82.9 and 265.1 +/- 57.0, respectively. Alanine, glycine, phenylalanine, threonine, and serine were the principal amino acids in sweat. The concentrations of ammonia in the sweat of group I, II, and III children were 6.90 +/- 2.97, 2.35 +/- 0.81, and 1.17 +/- 0.48 mg/100 ml, respectively. Protein concentrations were 44.15 +/- 14.07, 40.70 +/- 16.60, and 35.75 +/- 5.46 mg/100 ml, respectively.     

Effects of protein-energy malnutrition and human immunodeficiency virus-1 infection on essential fatty acid metabolism in children

This report summarizes data on the availability of essential fatty acids (EFAs) and their long-chain polyunsaturated fatty acid (LCPUFA) metabolites in protein-energy malnutrition (PEM), in human immunodeficiency virus-1 (HIV-1) infection for which less information is available, and the combination of both PEM and HIV-1. The contribution of different EFAs and LCPUFAs to the fatty-acid composition of plasma and erythrocyte membrane lipids was found to be reduced in children with PEM in comparison with well-nourished children. In addition to limited dietary EFA supply, reduced bioconversion of EFAs to their respective LCPUFA metabolites and/or peroxidative degradation of LCPUFAs may contribute to the reduction of LCPUFA status in malnourished children. Restoration of normal energy, protein, and EFA intakes does not appear to readily correct abnormalities of plasma and erythrocyte membrane LCPUFA values. Enhanced dietary supply of LCPUFAs and/or improved supply of antioxidant vitamins may represent novel therapeutic modalities in severe PEM. With and without PEM, HIV infection was related to altered availability of various EFAs and LCPUFAs in HIV-seropositive children. The plasma total lipid fatty-acid profiles seen in well-nourished children with HIV infection were compatible with an HIV infection-related enhancement of the metabolic activity of the conversion of EFAs to their respective LCPUFA metabolites. However, the plasma phospholipid EFA and LCPUFA profiles seen in severely malnourished children with HIV infection more closely resembled those seen in children with PEM but without HIV infection than in those in children with HIV infection but no PEM. Metabolic studies using stable isotope-labeled fatty acids may contribute to better understanding of the HIV-related changes in EFA metabolism and clearly are needed before therapeutic conclusions can be drawn.     

Assessment of whole body protein metabolism in critically ill children: can we use the [15N]glycine single oral dose method?

BACKGROUND & AIMS: Most stable-isotope methods to evaluate whole body protein metabolism in patients are invasive and difficult to use in children. In this study protein metabolism was evaluated with the non-invasive [15N]glycine single oral dose method in critically ill children and the value of the method is discussed. METHODS: [15N]glycine (100mg) was given orally to children (mean age 5.5 years; range 0.6-15.5 years) with meningococcal septic shock (MSS, n = 8), pneumonia (n = 5), and to healthy, fed and post-absorptive children (n = 10). Urine was collected during 9h, total amount of NH(3), labelled NH(3) and nitrogen were measured, and protein turnover, synthesis and breakdown were calculated using urinary NH(3) as end-product. RESULTS: Mean protein turnover in children with MSS, pneumonia and fed and post-absorptive healthy children was 0.63+/-0.13, 0.38+/-0.10, 0.28+/-0.03 and 0.28+/-0.02g N/kg/9h, respectively. Mean protein synthesis was 0.55+/-0.12, 0.29+/-0.09, 0.18+/-0.02, 0.20+/-0.02g N/kg/9h, respectively. Mean protein breakdown was 0.56+/-0.14, 0.28+/-0.12, 0.08+/-0.03, 0.28+/-0.02g N/kg/9h, respectively. Protein turnover, synthesis and breakdown were significantly increased in MSS patients compared to fed healthy children (P <0.01) and post-absorptive children (P <0.05). Protein turnover, protein synthesis, protein breakdown were significantly correlated with disease severity and body temperature (P <0.05). CONCLUSION: Results of whole body protein metabolism measured with the [15N]glycine single oral dose method in children with MSS and in healthy children were in line with expectations based on results obtained in earlier reports and with different methods.     

État nutritionnel des patients avec maladies du foie : épidémiologie et pronostic

Malnutrition is generally defined as protein-energy malnutrition (PEM) in patients with chronic liver disease, because the depletion of muscle mass and body fat is associated with protein depletion. Deficiencies of vitamins and minerals often coexist. PEM represents a common complication of advanced liver disease, both of alcoholic and nonalcoholic etiology. It is related to the severity of the liver disease more than to its etiology.Malnutrition negatively affects liver function, complications of the liver disease, and survival. Malnourished patients have an increased surgical risk and decreased survival after liver transplantation. The assessment of nutritional status in patients with chronic liver disease may be helpful in providing better prognostic information and more precise targeting of potential nutrition intervention.     

Energy expenditure in chronic alcoholics with and without liver disease

Chronic alcoholism activates metabolic pathways, resulting in wasteful expenditure of energy (Pirola and Lieber, J Nutr 1975;105:1544-8). To study this hypothesis, we measured oxygen consumption (VO2), carbon dioxide production (VCO2), and resting energy expenditure (MREE) utilizing indirect calorimetry in 8 chronic alcoholics with (group I) and 11 chronic alcoholics without (group II) clinical or biochemical evidence of alcoholic liver disease. Seven healthy volunteers served as controls. A statistically increased MREE was observed in group II subjects (p less than 0.05, MREE 999.7 +/- 111.4 kcal X day X m2) as compared to normals (MREE 842.3 +/- 42.1 kcal X day X m2) and group I subjects (MREE 813.4 +/- 101.4 kcal X day X m2). VO2 and VCO2 were also significantly higher (p less than 0.05) in group II than in group I and normals. The predicted resting energy expenditure as calculated by the Harris-Benedict equation was similar in both groups and normals. Theories to explain the increased MREE in group II subjects are presented.     

Dietary management of malnourished children with a new enteral feeding

Balance (nitrogen, fat, carbohydrate) and growth studies were carried out in nine children (6 to 26 months old) recovering from protein-energy malnutrition (PEM) using a concentrated (1 kcal/mL) formula designed specifically for 1- to 6-year-olds. The formula provided 12% of energy as protein (18% whey, 82% casein), 44% of energy as carbohydrate (69% corn syrup solids, 31% sucrose), and 44% of energy as fat (50% high-oleic safflower oil, 30% soy oil, 20% medium-chain triglycerides). Means +/- standard deviations of apparent nitrogen retention and absorption were 90 +/- 3% and 39 +/- 13%, respectively. Mean fecal fat excretion was 1.3 +/- 1.0 g/day. Length-age increased 3.0 +/- 0.8 months and weight-age increased 8.3 +/- 3.8 months during the 2.4 +/- 0.3 months of the study. (Length-age and weight-age were defined as the age (in months) to which the child's length or weight corresponded at the 50th percentile of the National Center for Health Statistics reference data). Thirteen additional 40- to 30-month-old children with kwashiorkor were fed the formula for 8 to 35 days as part of the initial management of PEM. The result was prompt weight gain and increases in serum proteins. Its formulation and the findings of our study indicate that this new formula offers a notable advantage over products designed for infants or adults in the enteral alimentation of young children.     

Relation between liver fat content and the rate of VLDL apolipoprotein B-100 synthesis in children with protein-energy malnutrition

BACKGROUND: Fatty infiltration of the liver is associated with an increased morbidity and mortality in children with severe protein-energy malnutrition (PEM), but its pathogenesis remains unclear. Although impaired synthesis of VLDL apolipoprotein B-100 (VLDL-apo B-100) is generally accepted as the pathogenetic mechanism, the rate of it synthesis has not been measured in children with PEM. OBJECTIVE: The objective of the study was to ascertain the relation between the degree of hepatic steatosis and the rate of VLDL-apo B-100 synthesis in children with PEM. DESIGN: The fractional and absolute rates of VLDL-apo B-100 synthesis were measured with a prime-constant intravenous infusion of [2H3]leucine in 13 severely malnourished children (8 boys and 5 girls) aged 7-18 mo. Hepatic fat content was estimated by computerized tomography scanning by using the ratio of liver to spleen (L:S) attenuation. The ratio is inversely related to hepatic fat content such that the lower the L:S, the greater the amount of fat in the liver. RESULTS: There were significant inverse relations between L:S attenuation and VLDL-apo B-100 concentration (P < 0.02), the absolute rate of VLDL-apo B-100 synthesis (P < 0.02), and plasma triacylglycerol (P < 0.02) and serum cholesterol (P < 0.05) concentrations. CONCLUSIONS: These results suggest that children with PEM synthesize VLDL-apo B-100 at a faster rate as the degree of hepatic fat infiltration increases. Thus, fatty infiltration of the liver in PEM is not due to a reduction in the synthesis of VLDL-apo B-100.     

A comparison of food group variety between toddlers with and without cystic fibrosis

OBJECTIVES: To compare the food group variety and nutritional adequacy of the diet between toddlers with cystic fibrosis (CF) and age-matched controls. Subjects A clinical sample of 22 toddlers with CF (mean age=21.3 +/- 7.2 months) matched to a community sample of 22 healthy peers. MAIN OUTCOME MEASURES: The variety index for toddlers (VIT) and the mean adequacy ratio (MAR). RESULTS: Fruit group scores were highest for children with CF (0.95 +/- 0.13; possible range 0.00-1.00), and dairy group scores were highest for controls (0.90 +/- 0.18). All children earned the lowest scores for vegetables (CF: 0.15 +/- 0.12; controls: 0.26 +/- 0.22). No significant differences were found when comparing VIT and MAR scores by sample (P >0.05). A moderate positive relationship was found between total VIT scores and MAR scores for all children (r=0.38, P <0.05). Toddlers with CF did not achieve the 120-150% of Recommended Dietary Allowance (RDA) for energy. CONCLUSIONS: Toddlers with CF are consuming diets that are varied and nutritionally adequate for a healthy child. To optimize nutritional status and growth, current recommendations for toddlers with CF to eat a well-balanced diet and to exceed daily RDA for energy requirements remain key dietary issues.