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1.
Nutrients ; 15(13)2023 Jul 01.
Article in English | MEDLINE | ID: mdl-37447337

ABSTRACT

Our primary study objectives were to (i) determine the proportion of children admitted to the Pediatric Intensive Care Unit (PICU) with malnutrition diagnoses, (ii) compare healthcare utilization by malnourished and non-malnourished PICU patients, and (iii) examine the impact of implementing malnutrition screening and coding practices at a major academic urban tertiary care medical center. Using patient records, we conducted a retrospective analysis of 4106 children admitted to the PICU for severe illnesses between 2011 and 2019. Patients were identified as malnourished if records showed an ICD-9 or ICD-10 code for malnutrition. We compared malnourished and non-malnourished patients by age, admitting diagnoses, number of comorbid conditions, and clinical outcomes (length of stay, hospital readmission). About 1 of every 5 PICU-admitted patients (783/4106) had a malnutrition diagnosis. Patients with malnutrition were younger (mean age 6.2 vs. 6.9 years, p < 0.01) and had more comorbid conditions (14.3 vs. 7.9, p < 0.01) than those without. Malnourished patients had longer hospital stays (26.1 vs. 10.0 days, p < 0.01) and higher 30-day readmission rates (10% vs. 7%, p = 0.03). Implementation of malnutrition screening and coding practices was associated with an increase in malnutrition diagnosis. In this study of children admitted to the PICU, malnourished patients had more comorbid diagnoses and used more healthcare resources (prolonged hospitalizations and higher 30-day readmission rates), leading to higher healthcare costs. Such findings underscore the need for policies, training, and programs emphasizing identification and treatment of malnutrition at hospitals caring for critically ill children.


Subject(s)
Critical Illness , Malnutrition , Child , Humans , Child, Hospitalized , Retrospective Studies , Malnutrition/diagnosis , Malnutrition/epidemiology , Malnutrition/therapy , Length of Stay , Delivery of Health Care , Nutritional Status
2.
Nutrients ; 13(1)2021 Jan 09.
Article in English | MEDLINE | ID: mdl-33435326

ABSTRACT

BACKGROUND: The purpose of this study was to evaluate the growth, tolerance and compliance effects of an extensively hydrolyzed formula with added 2'-FL in an intended use population of infants. METHODS: A non-randomized, single-group, multicenter study was conducted. Infants (0-60 days of age) with suspected food protein allergy, persistent feeding intolerance, or presenting conditions where an extensively hydrolyzed formula (eHF) was deemed appropriate were enrolled in a 2-month feeding trial. The primary outcome was maintenance of weight for age z-score during the study. Weight, length, head circumference, formula intake, tolerance measures, clinical symptoms and questionnaires were collected. Forty-eight infants were enrolled and 36 completed the study. RESULTS: Weight for age z-scores of infants showed a statistically significant improvement from study day 1 to study day 60 (0.32 ± 0.11, p = 0.0078). CONCLUSIONS: Overall, the results of the study demonstrate that the study formula was well tolerated, safe and supported growth in the intended population.


Subject(s)
Immune Tolerance , Infant Formula , Milk Hypersensitivity/prevention & control , Milk, Human/chemistry , Oligosaccharides , Body Weight , Female , Food Hypersensitivity/prevention & control , Humans , Hydrolysis , Infant , Infant, Newborn , Male , Milk Hypersensitivity/epidemiology , Milk Hypersensitivity/physiopathology , Milk Proteins/adverse effects
3.
Diabetes Technol Ther ; 20(10): 672-680, 2018 10.
Article in English | MEDLINE | ID: mdl-30204483

ABSTRACT

BACKGROUND: Obesity is a risk factor for gestational diabetes (gestational diabetes). Low-glycemic index diets attenuate hyperglycemia. We designed a study to determine whether a slow-digesting, low-glycemic load (SD-LGL) beverage improves glucose tolerance in obese pregnant women without GDM. METHODS: This was a 3-arm comparison study comparing the effects of an SD-LGL nutritional beverage (glycemic load [GL] 730), an isocaloric control beverage (GL 1124), and habitual diet on glycemia in obese pregnant women. Sixteen women (mean body mass index 37 kg/m2) were recruited at 24-28 weeks to receive either the SD-LGL or eucaloric control beverage. This was consumed with breakfast and as a midafternoon snack over 2 days with a controlled diet. Following a 2-day washout period of habitual diet, women completed 2 days on the alternative beverage with controlled diet. A 10-h fast preceded each intervention phase. Twenty-four hour glucose was measured using continuous glucose monitoring. RESULTS: Consumption of the lower GL beverage was associated with improved measures of glycemia, compared with the control beverage and habitual diet at different time periods. Glucose estimates for control versus SD-LDL at 24 h (0.23 mmol/L [0.16 to 0.31], P < 0.001), daytime (0.26 mmol/L [0.18 to 0.34], P < 0.001), and nighttime (0.05 mmol/L [-0.01 to 0.11], P = 0.09). Postprandial glucose was lower after breakfast but not after dinner, compared with the control beverage (0.09 mmol/L [0.01 to 0.18], P = 0.03). CONCLUSION: A slow-digesting, low-glycemic nutritional beverage may facilitate improved glucose control in obese pregnant women. To address potential benefit for clinical outcomes, a randomized controlled trial is warranted.


Subject(s)
Beverages , Diabetes, Gestational/prevention & control , Glucose Intolerance/drug therapy , Glycemic Load , Obesity/drug therapy , Adult , Blood Glucose/analysis , Body Mass Index , Diet , Dietary Carbohydrates , Female , Glucose Tolerance Test , Humans , Pregnancy
4.
Eur J Clin Nutr ; 72(4): 548-556, 2018 04.
Article in English | MEDLINE | ID: mdl-29367733

ABSTRACT

BACKGROUND/OBJECTIVES: Breakfast consumption has been shown to impact children's growth and development, but the influence of breakfast skipping on total daily intakes is not known. The purpose of this study was to examine the differences in nutritional intakes and food consumption between children who consume breakfast versus those who do not. SUBJECTS/METHODS: Dietary recall data were assessed for 2-year-old to 5-year-old children (n = 3443) and 6-year-old to 12-year-old children (n = 5147) from NHANES 2005-2012. Dietary intakes and diet quality scores were compared across breakfast consumption and skipping with means and 95% confidence interval. RESULTS: Children who skipped breakfast on the day of intake had significantly lower energy intakes for the total day (5911 vs 6723 kJ) but had greater energy intakes from non-breakfast meals and snacks. Children who skipped breakfast consumed nearly 40% of the day's intake (2332 kJ of 5911 kJ) from snacks, with 586 kJ from snacks as added sugars. Breakfast skipping was also related to significantly lower intakes of fiber, folate, iron, and calcium intakes. Overall diet quality scores, as well as fruit, whole fruit, whole grains, dairy and empty calorie subscale scores were significantly better in children who ate breakfast. CONCLUSIONS: Children who skipped breakfast presented poorer overall diet quality and lower total intakes commonly obtained at breakfast. To address missed opportunities, nutrition professionals should encourage children's consumption of a nutritious breakfast to support overall diet quality.


Subject(s)
Breakfast , Diet/statistics & numerical data , Nutritive Value , Child , Child, Preschool , Cross-Sectional Studies , Energy Intake , Humans , Nutrition Surveys , United States/epidemiology
5.
Matern Child Nutr ; 14(1)2018 01.
Article in English | MEDLINE | ID: mdl-28337856

ABSTRACT

Nutritional quality of children's diets is a public health priority in the fight against childhood obesity and chronic diseases. The main purpose of this study was to determine contribution of snacks to energy and nutrient intakes and to identify leading snack food sources of energy, total fat, and added sugars amongst young children in the United States. Using the 2005-2012 NHANES data, dietary intakes of 2- to 5-year-old children were analysed from a parent-reported 24-hour dietary recall (n = 3,429). Snacking occasions were aggregated to determine the proportion of total food/beverage intake obtained from snacks, estimate energy, and nutrient intakes, and identify the leading snack food sources of energy, added sugars, and total fat. Nearly all children consumed a snack on the reported day (62% morning, 84% afternoon, and 72% evening). Snacks accounted for 28% of total energy intake, 32% of carbohydrates, 39% of added sugars, and 26% of total fat and dietary fiber intakes for the day. Snacking occasions accounted for 46.6% of all beverages consumed on the reported day. Snacks and sweets food category (i.e., cookies and pastries) were the leading sources of energy (44%), total fat (52%), and added sugars (53%) consumed during snacking occasions. Sweetened beverages (e.g., fruit and sport drinks) contributed 1-quarter of all added sugars obtained from snacks. Snacks contribute considerable amount of energy and nutrients to young children's diets, with a heavy reliance on energy-dense foods and beverages. Targeted interventions are needed to improve the nutritional quality of snacks consumed by young children.


Subject(s)
Diet , Snacks , Beverages/analysis , Beverages/statistics & numerical data , Child Nutritional Physiological Phenomena , Child, Preschool , Dietary Fats/administration & dosage , Dietary Fiber/administration & dosage , Dietary Sugars/administration & dosage , Energy Intake , Humans , Nutrition Surveys , Nutritive Value , United States
6.
Br J Nutr ; 118(10): 813-821, 2017 Nov.
Article in English | MEDLINE | ID: mdl-29151374

ABSTRACT

With the association between increased carotenoid intake and lower risk of chronic diseases, the absorption of lutein from the diet becomes an important factor in its delivery and physiological action. The primary objective of this study was to gain an understanding of how a new formulation technology (mixture of mono- and diglycerides (MDG)), affected lutein absorption. Subjects (n 24) were randomised in a cross-over, double-blind study to receive a single dose of 6 mg lutein (FloraGLO 20 %) provided as capsules containing either high-oleic safflower (SAF) oil or a MDG oil. Subjects receiving a single dose of lutein in MDG showed a significantly greater change from baseline (0 h) to 4, 6, 8, 12, 24, 48 and 336 h (P<0·05) and baseline adjusted AUC for plasma lutein at 48 and 336 h (P<0·001) as compared with subjects given lutein in SAF. Analysis of the 48 h absorption kinetics of lutein showed that the time to peak level of lutein (12 h) was the same for SAF and MDG groups, but the change in plasma lutein at 12 and 48 h were 129 and 320 % higher, respectively, for MDG compared with SAF. This difference continued as the adjusted AUC 0-48 and 0-336 h for the MDG group was 232 and 900 % higher, respectively, v. SAF. The study data show that by changing the lipid that is combined with a lutein supplement results in significant increases in lutein absorption in healthy adults.


Subject(s)
Dietary Supplements , Diglycerides/pharmacology , Intestinal Absorption , Lutein/pharmacokinetics , Monoglycerides/pharmacology , Adult , Area Under Curve , Cross-Over Studies , Diet , Double-Blind Method , Female , Humans , Lutein/blood , Male , Oleic Acid/pharmacology , Reference Values , Safflower Oil , Triglycerides/pharmacology
7.
J Nutr ; 146(12): 2559-2566, 2016 Dec.
Article in English | MEDLINE | ID: mdl-27798337

ABSTRACT

BACKGROUND: Evidence suggests that human milk oligosaccharides (HMOs) provide multiple benefits to infants, including prebiotic effects, gut maturation, antimicrobial activities, and immune modulation. Clinical intervention studies with HMOs are required to confirm these benefits in infants. OBJECTIVE: Our objective was to investigate the effects of feeding formulas supplemented with the HMO 2'-fucosyllactose (2'-FL) on biomarkers of immune function in healthy term infants. METHODS: We performed a substudy nested within a randomized, double-blind, controlled growth and tolerance study in healthy singleton infants (birth weight ≥2490 g) who were enrolled by 5 d of life and exclusively formula-fed (n = 317) or breastfed (n = 107) from enrollment to 4 mo of age. Formula-fed infants were randomly assigned to receive 1 of 3 formulas, all containing 2.4 g total oligosaccharides/L [control: galacto-oligosaccharides (GOS) only; experimental formulas: GOS + 0.2 or 1.0 g 2'-FL/L], and compared with a breastfed reference group. For this substudy, blood samples were drawn from infants at 6 wk of age (n = 31-42/group). Peripheral blood mononuclear cells (PBMCs) were isolated for cellular phenotyping and stimulated ex vivo with phytohemagglutinin for proliferation and cell cycle progression or respiratory syncytial virus (RSV). Cytokine concentrations were measured in plasma and in ex vivo-stimulated culture supernatants. RESULTS: Breastfed infants and infants fed either of the experimental formulas with 2'-FL were not different but had 29-83% lower concentrations of plasma inflammatory cytokines than did infants fed the control formula [interleukin (IL) receptor antagonist (IL-1ra), IL-1α, IL-1ß, IL-6, and tumor necrosis factor α (TNF-α)] (P ≤ 0.05). In ex vivo RSV-stimulated PBMC cultures, breastfed infants were not different than either of the groups fed formula with 2'-FL, but they had lower concentrations of TNF-α (31%) and interferon γ (IFN-γ 54%) (P ≤ 0.05) and tended to have lower IL-1ra (25%) and IL-6 (38%) (unadjusted P ≤ 0.05) and IL-1ß (30%) (unadjusted P = 0.06) than did infants fed the control formula. CONCLUSIONS: Our data indicate that infants fed formula supplemented with 2'-FL exhibit lower plasma and ex vivo inflammatory cytokine profiles, similar to those of a breastfed reference group. This trial was registered at clinicaltrials.gov as NCT01808105.


Subject(s)
Breast Feeding , Cytokines/blood , Infant Formula/chemistry , Trisaccharides/pharmacology , Cell Proliferation , Cytokines/metabolism , Double-Blind Method , Gene Expression Regulation , Humans , Infant , Infant, Newborn , Inflammation/blood , Inflammation/metabolism , Leukocytes, Mononuclear/metabolism , Respiratory Syncytial Viruses/isolation & purification , Trisaccharides/administration & dosage , Trisaccharides/chemistry , Viral Load
9.
J Pediatr Gastroenterol Nutr ; 61(6): 649-58, 2015 Dec.
Article in English | MEDLINE | ID: mdl-26154029

ABSTRACT

OBJECTIVES: The aim of the present study was to examine the growth and tolerance of infants fed infant formulas with a caloric density closer to human milk (HM) supplemented with human milk oligosaccharides (HMOs) and to study uptake of the HMOs. METHODS: A prospective, randomized, controlled, growth and tolerance study was conducted in healthy, singleton infants (birth weight ≥2490 g), who were enrolled by day of life (DOL) 5. Formula-fed infants were randomized to 1 of 3 formulas with a caloric density of 64.3 kcal/dL. Each formula contained galactooligosaccharides, and the 2 experimental formulas contained varying levels (0.2 and 1.0 g/L) of the HMO 2'-fucosyllactose (2'FL). The 3 formula groups were compared with an HM-fed reference group. Infants were exclusively fed either formula (n = 189) or HM (n = 65) from enrollment to 119 DOL. 2'FL was measured in the blood and urine collected from a subset of infants at DOL 42 and 119, and in HM collected from breast-feeding mothers at DOL 42. RESULTS: There were no significant differences among any groups for weight, length, or head circumference growth during the 4-month study period. All of the formulas were well tolerated and comparable for average stool consistency, number of stools per day, and percent of feedings associated with spitting up or vomit. 2'FL was present in the plasma and urine of infants fed 2'FL, and there were no significant differences in 2'FL uptake relative to the concentration fed. CONCLUSIONS: This is the first report of infants fed 2'FL-fortified formulas with a caloric density similar to HM. Growth and 2'FL uptake were similar to those of HM-fed infants.


Subject(s)
Bottle Feeding , Dietary Supplements , Energy Intake , Growth , Infant Formula/chemistry , Milk, Human/chemistry , Trisaccharides/pharmacology , Breast Feeding , Feces , Female , Humans , Infant, Newborn , Male , Prospective Studies , Trisaccharides/metabolism , Trisaccharides/pharmacokinetics
10.
Mol Genet Metab ; 112(3): 210-7, 2014 Jul.
Article in English | MEDLINE | ID: mdl-24881969

ABSTRACT

In an effort to increase harmonization of care and enable outcome studies, the Genetic Metabolic Dietitians International (GMDI) and the Southeast Regional Newborn Screening and Genetics Collaborative (SERC) are partnering to develop nutrition management guidelines for inherited metabolic disorders (IMD) using a model combining both evidence- and consensus-based methodology. The first guideline to be completed is for maple syrup urine disease (MSUD). This report describes the methodology used in its development: formulation of five research questions; review, critical appraisal and abstraction of peer-reviewed studies and unpublished practice literature; and expert input through Delphi surveys and a nominal group process. This report includes the summary statements for each research question and the nutrition management recommendations they generated. Each recommendation is followed by a standardized rating based on the strength of the evidence and consensus used. The application of technology to build the infrastructure for this project allowed transparency during development of this guideline and will be a foundation for future guidelines. Online open access of the full, published guideline allows utilization by health care providers, researchers, and collaborators who advise, advocate and care for individuals with MSUD and their families. There will be future updates as warranted by developments in research and clinical practice.


Subject(s)
Maple Syrup Urine Disease/diet therapy , Maple Syrup Urine Disease/surgery , Age Factors , Diet , Dietary Supplements , Disease Management , Evidence-Based Medicine , Female , Health Care Surveys , Humans , Liver Transplantation , Male , Maple Syrup Urine Disease/blood , Practice Guidelines as Topic , Pregnancy
11.
J Nutr ; 144(8): 1256-63, 2014 Aug.
Article in English | MEDLINE | ID: mdl-24899160

ABSTRACT

Lutein is a carotenoid that varies in breast milk depending on maternal intake. Data are lacking with regard to the effect of dietary lutein supplementation on breast milk lutein concentration during lactation and subsequent plasma lutein concentration in breast-fed infants. This study was conducted to determine the impact of lutein supplementation in the breast milk and plasma of lactating women and in the plasma of breast-fed infants 2-3 mo postpartum. Lutein is the dominant carotenoid in the infant brain and the major carotenoid found in the retina of the eye. Eighty-nine lactating women 4-6 wk postpartum were randomly assigned to be administered either 0 mg/d of lutein (placebo), 6 mg/d of lutein (low-dose), or 12 mg/d of lutein (high-dose). The supplements were consumed for 6 wk while mothers followed their usual diets. Breast milk carotenoids were measured weekly by HPLC, and maternal plasma carotenoid concentrations were measured at the beginning and end of the study. Infant plasma carotenoid concentrations were assessed at the end of the study. No significant differences were found between dietary lutein + zeaxanthin intake and carotenoid concentrations in breast milk and plasma or body mass index at baseline. Total lutein + zeaxanthin concentrations were greater in the low- and high-dose-supplemented groups than in the placebo group in breast milk (140% and 250%, respectively; P < 0.0001), maternal plasma (170% and 250%, respectively; P < 0.0001), and infant plasma (180% and 330%, respectively; P < 0.05). Lutein supplementation did not affect other carotenoids in lactating women or their infants. Lactating women are highly responsive to lutein supplementation, which affects plasma lutein concentrations in the infant. This trial was registered at clinicaltrials.gov as NCT01747668.


Subject(s)
Carotenoids/blood , Dietary Supplements , Lactation/drug effects , Lutein/administration & dosage , Lutein/blood , Milk, Human/chemistry , Adult , Breast Feeding , Diet , Dose-Response Relationship, Drug , Female , Humans , Infant , Male , Postpartum Period
12.
J Nutr Metab ; 2012: 891201, 2012.
Article in English | MEDLINE | ID: mdl-23056929

ABSTRACT

Faster weight gain early in infancy may contribute to a greater risk of later obesity in formula-fed compared to breast-fed infants. One potential explanation for the difference in weight gain is higher macronutrient intake in formula-fed infants during the first weeks of life. A systematic review was conducted using Medline to assess the macronutrient and energy content plus volume of intake in breast-fed and formula-fed infants in early infancy. All studies from healthy, term, singleton infants reporting values for the composition of breast milk during the first month of life were included. The energy content of colostrum (mean, SEM: 53.6 ± 2.5 kcal/100 mL), transitional milk (57.7 ± 4.2 kcal/100 mL), and mature milk (65.2 ± 1.1 kcal/100 mL) was lower than conventional infant formula (67 kcal/100 mL) on all days analyzed. The protein concentration of colostrum (2.5 ± 0.2 g/100 mL) and transitional milk (1.7 ± 0.1 g/100 mL) was higher than formula (1.4 g/100 mL), while the protein content of mature milk (1.3 ± 0.1 g/100 mL) was slightly lower. Formula-fed infants consume a higher volume and more energy dense milk in early life leading to faster growth which could potentially program a greater risk of long-term obesity.

13.
Mol Genet Metab ; 86(4): 448-55, 2005 Dec.
Article in English | MEDLINE | ID: mdl-16260164

ABSTRACT

BACKGROUND: Poor growth has been described in patients with urea cycle enzyme defects treated with protein-restricted diets, while protein status is seldom reported. OBJECTIVE: To assess the effects of nutritional therapy with a medical food on growth and protein status of patients with a urea cycle enzyme defect. METHODS: A 6-mo multicenter outpatient study was conducted with infants and toddlers managed by nutrition therapy with Cyclinex-1 Amino Acid-Modified Medical Food with Iron (Ross Products Division, Abbott Laboratories, Columbus, OH). Main outcome variables were anthropometrics and plasma amino acids (selected), albumin, and transthyretin concentrations. RESULTS: Seventeen patients completed the study. Mean (+/-SE) baseline age was 11.30+/-3.20 months (median 4.40 months; range 0.22-38.84 months). Length and weight z-scores increased significantly during the 6-month study. Head circumference increased, but not significantly. Three patients were stunted and two were wasted (-2.0 z-score) at baseline while at study end, only one patient was both stunted and wasted. The majority of patients increased in length, head circumference, and weight z-scores during study. Mean (+/-SE) plasma albumin concentration increased from 34+/-2g/L at baseline to 38+/-1g/L at study end. Plasma transthyretin increased from a mean (+/-SE) of 177+/-13 mg/L at baseline to 231+/-15 mg/L at study end. No correlation was found between plasma NH(3) concentrations and medical food intake. Plasma NH(3) concentration was positively correlated with the percentage of Food and Agriculture Organization/World Health Organization/United Nations recommended protein ingested. CONCLUSIONS: Intakes of adequate protein and energy for age result in anabolism and linear growth without increasing plasma NH(3) concentrations. Medical food intakes did not correlate with plasma NH(3) concentrations.


Subject(s)
Metabolism, Inborn Errors/diet therapy , Urea/metabolism , Amino Acids/administration & dosage , Amino Acids/blood , Blood Proteins/metabolism , Body Height , Child, Preschool , Dietary Proteins/administration & dosage , Energy Intake , Female , Growth , Humans , Infant , Infant, Newborn , Male , Metabolism, Inborn Errors/genetics , Metabolism, Inborn Errors/metabolism , Metabolism, Inborn Errors/pathology , Nutritional Status , Prealbumin/metabolism , Serum Albumin/metabolism , Weight Gain
14.
Mol Genet Metab ; 81(4): 263-72, 2004 Apr.
Article in English | MEDLINE | ID: mdl-15059613

ABSTRACT

Marked progress has been made over the past 15 years in defining the specific biochemical defects and underlying molecular mechanisms of oxidative phosphorylation disorders, but limited information is currently available on the development and evaluation of effective treatment approaches. Metabolic therapies that have been reported to produce a positive effect include coenzyme Q(10) (ubiquinone), other antioxidants such as ascorbic acid and vitamin E, riboflavin, thiamine, niacin, vitamin K (phylloquinone and menadione), and carnitine. The goal of these therapies is to increase mitochondrial ATP production, and to slow or arrest the progression of clinical symptoms. In the present study, we demonstrate for the first time that there is a significant increase in ATP synthetic capacity in lymphocytes from patients undergoing cofactor treatment. We also examined in vitro cofactor supplementation in control lymphocytes in order to determine the effect of the individual components of the cofactor treatment on ATP synthesis. A dose-dependent increase in ATP synthesis with CoQ(10) incubation was demonstrated, which supports the proposal that CoQ(10) may have a beneficial effect in the treatment of oxidative phosphorylation (OXPHOS) disorders.


Subject(s)
Adenosine Triphosphate/biosynthesis , Mitochondrial Diseases/drug therapy , Ubiquinone/analogs & derivatives , Ubiquinone/therapeutic use , Adolescent , Adult , Antioxidants/therapeutic use , Child , Coenzymes , Dose-Response Relationship, Drug , Female , Humans , Lymphocytes/drug effects , Lymphocytes/metabolism , Male , Middle Aged , Ubiquinone/administration & dosage
15.
Mol Genet Metab ; 80(1-2): 181-8, 2003.
Article in English | MEDLINE | ID: mdl-14567967

ABSTRACT

BACKGROUND: Failure-to-thrive (FTT) has been described in patients with organic acidemias treated with low protein diets. OBJECTIVE: To determine if patients with methylmalonic (MMA) or propionic acidemia (PA) can achieve normal growth and nutrition status. METHODS: A 6-month multicenter outpatient study was conducted with infants and toddlers treated with Propimex-1 Amino Acid-Modified Medical Food With Iron (Ross Products Division, Abbott Laboratories, Columbus, OH). Main outcome measures were anthropometrics, protein status indices, plasma retinol, and alpha-tocopherol. RESULTS: Sixteen patients completed the study. Mean baseline age was 0.54 +/- 0.02 years (range 0.03-3.00 years). By study end, mean National Center for Health Statistics (NCHS) weight centile increased from 26 to 49%; mean crown-heel length centile from 25 to 33%; and mean head circumference centile from 43 to 54%. Mean (+/- SE) protein and energy intakes by <6-month-old, 6<12-month-old, and 1<4-year-old patients were 15.3 +/- 0.9 g and 645 +/- 10 kcal; 18.3 +/- 1.1 g and 741 +/- 92 kcal; and 25.1 +/- 2.46 g and 1062 +/- 100 kcal, respectively. Plasma glycine concentrations were significantly and negatively correlated with energy intake (r=-0.77, p<0.0005). No correlation was found between dietary protein intakes and plasma ammonia concentrations. Protein status indices, retinol and alpha-tocopherol concentrations were within reference ranges at study end. CONCLUSIONS: Propimex-1 improved growth and nutrition status in patients with MMA or PA in just 6 months when fed in sufficient amounts. Providing energy and protein for patients with FTT at intakes recommended for catch-up growth may have resulted in even better growth.


Subject(s)
Body Weight/physiology , Methylmalonic Acid/blood , Nutritional Status/physiology , Peroxisomal Disorders/diet therapy , Propionates/blood , Amino Acids/administration & dosage , Ammonia/blood , Child, Preschool , Female , Humans , Infant , Iron/administration & dosage , Male , Nutritional Status/genetics , Nutritional Support , Peroxisomal Disorders/genetics , Vitamin A/blood , alpha-Tocopherol/blood
16.
J Am Diet Assoc ; 103(8): 1029-38, 2003 Aug.
Article in English | MEDLINE | ID: mdl-12891154

ABSTRACT

Mitochondrial disorders are degenerative diseases characterized by a decrease in the ability of mitochondria to supply cellular energy requirements. Substantial progress has been made in defining the specific biochemical defects and underlying molecular mechanisms, but limited information is available about the development and evaluation of effective treatment approaches. The goal of nutritional cofactor therapy is to increase mitochondrial adenosine 5'-triphosphate production and slow or arrest the progression of clinical symptoms. Accumulation of toxic metabolites and reduction of electron transfer activity have prompted the use of antioxidants, electron transfer mediators (which bypass the defective site), and enzyme cofactors. Metabolic therapies that have been reported to produce a positive effect include Coenzyme Q(10) (ubiquinone); other antioxidants such as ascorbic acid, vitamin E, and lipoic acid; riboflavin; thiamin; niacin; vitamin K (phylloquinone and menadione); creatine; and carnitine. A literature review of the use of these supplements in mitochondrial disorders is presented.


Subject(s)
Antioxidants/therapeutic use , Coenzymes/therapeutic use , Mitochondrial Diseases/diet therapy , Adenosine Triphosphate/metabolism , Antioxidants/metabolism , Coenzymes/metabolism , Electron Transport/drug effects , Humans , Mitochondrial Diseases/metabolism , Oxidative Phosphorylation/drug effects
17.
Anal Biochem ; 313(1): 137-44, 2003 Feb 01.
Article in English | MEDLINE | ID: mdl-12576069

ABSTRACT

Biochemical analysis of oxidative phosphorylation (OXPHOS) disorders is traditionally carried out on muscle biopsies, cultured fibroblasts, and transformed lymphocytes. Here we present a new screening technique using lymphocytes to identify OXPHOS dysfunction and initially avoid an invasive diagnostic procedure. Lymphocytes represent an easily obtainable source of tissue that presents advantages over the use of fibroblasts or lymphoblast cell lines. The time delay in culturing skin fibroblasts and the interactions between cell transformation and mitochondrial activity are avoided in this methodology. The method requires a small amount of blood (<5 mL); can be completed in a few hours, and allows for repeated measurements. Our assay has been adapted from published methods utilizing cultured fibroblasts and transformed lymphocytes, and our data suggest that measurement of ATP synthesis in lymphocytes is an effective screening tool for diagnosing OXPHOS disorders. This method may also provide an objective tool for monitoring response to treatment and evaluating progression of disease.


Subject(s)
Biological Assay , Genetic Testing , Lymphocytes/metabolism , Mitochondrial Diseases/diagnosis , Adult , Child , Female , Humans , Male , Middle Aged
18.
Can J Diet Pract Res ; 60(3): 166-169, 1999.
Article in English | MEDLINE | ID: mdl-11551328

ABSTRACT

Athletes often have inadequate nutrition knowledge and poor nutritional habits, which can have a negative impact on athletic performance. This study assessed the nutrition knowledge of competitive adolescent and young adult male hockey players, and examined the impact of a nutrition intervention program, Sport Nutrition for the Athletes of Canada (SNAC). Before the intervention, nutrition knowledge was tested in 175 adolescent and young adult male hockey players. The intervention was provided as part of a hockey camp curriculum and was based on the SNAC workbook, which emphasizes achieving a balanced diet with adequate energy and fluid intake. After the intervention, nutrition knowledge was assessed in a subgroup of 33 hockey players. The pre-intervention nutrition knowledge score was 45% (n = 175), which suggests this population had little sport nutrition knowledge. Nutrition knowledge scores two weeks after the intervention showed no meaningful improvement in the subgroup. The results of this study suggest that the SNAC nutrition intervention program offered under the conditions of this study, did not effectively improve nutrition knowledge in adolescent and young adult male hockey players.

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