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https://www.readbyqxmd.com/read/28315884/complementary-feeding-micronutrients-and-developmental-outcomes-of-children
#1
Jacqueline F Gould
The period of complementary feeding (6-24 months of age) can be a challenging and vulnerable time for infant nutrition due to disproportionately high requirements for metabolic processes, rapid developmental processes, and limited gastric capacity. This is a period of crucial brain development where high caloric intake is necessary to allow synaptogenesis (creation of channels between neurons for communication), and maintenance of established synapses, myelination (laying the myelin sheath around neuronal axons) and everyday psychological functioning...
2017: Nestlé Nutrition Institute Workshop Series
https://www.readbyqxmd.com/read/28300031/epileptic-spasms-in-congenital-disorders-of-glycosylation
#2
Andreia G Pereira, Nadia Bahi-Buisson, Christine Barnerias, Nathalie Boddaert, Rima Nabbout, Pascale de Lonlay, Anna Kaminska, Monika Eisermann
Congenital disorders of glycosylation (CDG) are a group of rare metabolic diseases, characterized by impaired glycosylation of proteins and lipids. Multisystemic involvement is common and neurological impairment is notably severe and disabling, concerning the central and peripheral nervous system. Epilepsy is frequent, but detailed electroclinical description is rare. We describe, retrospectively, the electroclinical features in five children with CDG syndrome and epileptic spasms. Epileptic spasms were observed in patients with CDG Ik, Ic, Ix, and Ip subtypes, and occurred at an early age, before 6 months in all cases, except one who had spasms that started at 18 months...
March 14, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/28294445/the-challenge-of-nutritional-profiling-of-a-protein-free-feed-module-for-children-on-low-protein-tube-feeds-with-organic-acidaemias
#3
A Daly, S Evans, C Ashmore, S Chahal, S Santra, A MacDonald
BACKGROUND: Enteral tube feeding for children with organic acidaemias (OA) is recommended. Protein restriction, providing minimum safe levels of protein intake, is advocated. Standard paediatric tube feeding formulae provide more than the minimum safe protein requirements and are unsuitable in OA without modification. Modified paediatric enteral feeds consist of several modular ingredients. The aim of this prospective longitudinal interventional study was to assess the efficacy of a premeasured novel protein-free module developed for children aged over 12 months compared to conventional practice...
March 10, 2017: Journal of Human Nutrition and Dietetics: the Official Journal of the British Dietetic Association
https://www.readbyqxmd.com/read/28293101/new-developments-in-the-management-of-partial-onset-epilepsy-role-of-brivaracetam
#4
REVIEW
Giangennaro Coppola, Giulia Iapadre, Francesca Felicia Operto, Alberto Verrotti
Currently, a number of novel anticonvulsant drugs, the so-called third generation, are in various stages of development. Several of them are already available or in ongoing clinical trials. These new compounds should take advantage of new insights into the basic pathophysiology of epileptogenesis, drug metabolism and drug interactions. Many of them still need to be further evaluated mainly in real-world observational trials and registries. Among newer anticonvulsant drugs for partial-onset seizures (POSs), rufinamide, lacosamide, eslicarbazepine and perampanel are those new treatment options for which more substantial clinical evidence is currently available, both in adults and, to some extent, in children...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/28287408/nutrient-sensing-nuclear-receptors-ppar%C3%AE-and-fxr-control-liver-energy-balance
#5
Geoffrey A Preidis, Kang Ho Kim, David D Moore
The nuclear receptors PPARα (encoded by NR1C1) and farnesoid X receptor (FXR, encoded by NR1H4) are activated in the liver in the fasted and fed state, respectively. PPARα activation induces fatty acid oxidation, while FXR controls bile acid homeostasis, but both nuclear receptors also regulate numerous other metabolic pathways relevant to liver energy balance. Here we review evidence that they function coordinately to control key nutrient pathways, including fatty acid oxidation and gluconeogenesis in the fasted state and lipogenesis and glycolysis in the fed state...
March 13, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28278160/hyperleptinemia-in-children-with-autosomal-recessive-spinal-muscular-atrophy-type-i-iii
#6
Heike Kölbel, Berthold P Hauffa, Stefan A Wudy, Anastasios Bouikidis, Adela Della Marina, Ulrike Schara
BACKGROUND: Autosomal-recessive proximal spinal muscular atrophies (SMA) are disorders characterized by a ubiquitous deficiency of the survival of motor neuron protein that leads to a multisystemic disorder, which mostly affects alpha motor neurons. Disease progression is clinically associated with failure to thrive or weight loss, mainly caused by chewing and swallowing difficulties. Although pancreatic involvement has been described in animal models, systematic endocrinological evaluation of the energy metabolism in humans is lacking...
2017: PloS One
https://www.readbyqxmd.com/read/28271036/functional-and-clinical-relevance-of-novel-and-known-pcsk1-variants-for-childhood-obesity-and-glucose-metabolism
#7
Dennis Löffler, Susanne Behrendt, John W M Creemers, Jürgen Klammt, Gabriela Aust, Juraj Stanik, Wieland Kiess, Peter Kovacs, Antje Körner
OBJECTIVE: Variants in Proprotein Convertase Subtilisin/Kexin Type 1 (PCSK1) may be causative for obesity as suggested by monogenic cases and association studies. Here we assessed the functional relevance in experimental studies and the clinical relevance through detailed metabolic phenotyping of newly identified and known PCSK1 variants in children. RESULTS: In 52 obese children selected for elevated proinsulin levels and/or impaired glucose tolerance, we found eight known variants and two novel heterozygous variants (c...
March 2017: Molecular Metabolism
https://www.readbyqxmd.com/read/28263087/protein-losing-enteropathy-in-an-infant-with-rotavirus-infection
#8
Adriana Parisi, Alessandro Cafarotti, Roberta Salvatore, Piernicola Pelliccia, Luciana Breda, Francesco Chiarelli
Protein-losing enteropathy (PLE) is a rare gastro-intestinal complication characterised by intestinal loss of proteins with consequent hypoproteinaemia and generalised oedema. Rotavirus infection associated with PLE in children has rarely been reported. A 6-month-old girl presented with diarrhoea, fever and generalised oedema. Total serum proteins were 34 g/L (61-79) and plasma albumin 16.8 g/L (40-50), serum sodium was 126 mmol/L and there was mild metabolic alkalosis (pH 7.46). Stool for alpha-1 antitrypsin was >1...
March 6, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28236852/the-promising-anticancer-drug-3-bromopyruvate-is-metabolized-through-glutathione-conjugation-which-affects-chemoresistance-and-clinical-practice-an-evidence-based-view
#9
Salah Mohamed El Sayed, Hussam Baghdadi, Mohammed Zolaly, Hamdi H Almaramhy, Mongi Ayat, Jagadish G Donki
3-Bromopyruvate (3BP) is a promising effective anticancer drug against many different tumors in children and adults. 3BP exhibited strong anticancer effects in both preclinical and human studies e.g. energy depletion, oxidative stress, anti-angiogenesis, anti-metastatic effects, targeting cancer stem cells and antagonizing the Warburg effect. There is no report about 3BP metabolism to guide researchers and oncologists to improve clinical practice and prevent drug resistance. In this article, we provide evidences that 3BP is metabolized through glutathione (GSH) conjugation as a novel report where 3BP was confirmed to be attached to GSH followed by permanent loss of pharmacological effects in a picture similar to cisplatin...
March 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28216642/high-fat-meal-systemic-inflammation-and-glucose-homeostasis-in-obese-children-and-adolescents
#10
A Morandi, E Fornari, F Opri, M Corradi, M Tommasi, R Bonadonna, C Maffeis
We aimed to assess in obese youths the relationships between interleukin-6 (IL-6), fat meal-induced endotoxemia and glucose homeostasis. Twenty obese children/adolescents (9-17 years old, 11 boys) underwent a standard oral glucose tolerance test and, 7-14 days later, a 5-h fat meal test (fat=69% of energy, saturated/monounsaturated/polyunsaturated fatty acids=31.5%/35%/33.5%), with serial measures of IL-6 and two markers of lipopolysaccharide (LPS) exposure and translocation, LPS-binding protein (LBP) and soluble CD14 (sCD14)...
March 14, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28215425/outcomes-of-lung-transplantation-for-infants-and-children-with-genetic-disorders-of-surfactant-metabolism
#11
Whitney B Eldridge, Qunyuan Zhang, Albert Faro, Stuart C Sweet, Pirooz Eghtesady, Aaron Hamvas, F Sessions Cole, Jennifer A Wambach
OBJECTIVE: To compare outcomes of infants and children who underwent lung transplantation for genetic disorders of surfactant metabolism (SFTPB, SFTPC, ABCA3, and NKX2-1) over 2 epochs (1993-2003 and 2004-2015) at St Louis Children's Hospital. STUDY DESIGN: We retrospectively reviewed clinical characteristics, mortality, and short- and long-term morbidities of infants (transplanted at <1 year; n = 28) and children (transplanted >1 year; n = 16) and compared outcomes by age at transplantation (infants vs children) and by epoch of transplantation...
February 16, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28208596/impact-of-early-nutrition-on-body-composition-in-children-aged-9-5-years-born-with-extremely-low-birth-weight
#12
Sonja Stutte, Bettina Gohlke, Annika Peiler, Felix Schreiner, Mark Born, Peter Bartmann, Joachim Woelfle
To evaluate body composition, metabolism and growth as well as their interaction with early nutrition in former extremely low birth weight infants (ELBW), we assessed qualitative and quantitative nutritional intake during initial hospitalization and infantile growth parameters in 61 former ELBW infants with a birth weight <1000 g. In two follow-up exams, physical and biochemical development were measured at 5.7 and at 9.5 years. At the second follow-up, in addition to biochemical reassessment, body composition was analyzed by dual-energy x-ray absorptiometry (DEXA)...
February 10, 2017: Nutrients
https://www.readbyqxmd.com/read/28191503/differences-in-gut-metabolites-and-microbial-composition-and-functions-between-egyptian-and-u-s-children-are-consistent-with-their-diets
#13
V Shankar, M Gouda, J Moncivaiz, A Gordon, N V Reo, L Hussein, O Paliy
Previous studies indicated that populations consuming a Mediterranean diet rich in fiber, vegetables, and fruits have a significantly lower risk of cardiovascular and metabolic diseases than populations of industrialized societies consuming diets enriched in processed carbohydrates, animal proteins, and fats. To explore the potential contributions of gut microbiota to the observed diet-related metabolic effects, we conducted an integrative analysis of distal gut microbiota composition and functions and intestinal metabolites in Egyptian and U...
January 2017: MSystems
https://www.readbyqxmd.com/read/28188435/marked-increase-in-urinary-excretion-of-apolipoproteins-in-children-with-nephrolithiasis-associated-with-hypercalciuria
#14
Larisa Kovacevic, Hong Lu, Joseph A Caruso, Tuhina Govil-Dalela, Ronald Thomas, Yegappan Lakshmanan
BACKGROUND: Using a proteomic approach, we aimed to identify and compare the urinary excretion of proteins involved in lipid transport and metabolism in children with kidney stones and hypercalciuria (CAL), hypocitraturia (CIT), and normal metabolic work-up (NM), and in healthy controls (HCs). Additionally, we aimed to confirm these results using ELISA, and to examine the relationship between the urinary excretion of selected proteins with demographic, dietary, blood, and urinary parameters...
February 10, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28183786/extracellular-rnas-are-associated-with-insulin-resistance-and-metabolic-phenotypes
#15
Ravi Shah, Venkatesh Murthy, Michael Pacold, Kirsty Danielson, Kahraman Tanriverdi, Martin G Larson, Kristina Hanspers, Alexander Pico, Eric Mick, Jared Reis, Sarah de Ferranti, Elizaveta Freinkman, Daniel Levy, Udo Hoffmann, Stavroula Osganian, Saumya Das, Jane E Freedman
OBJECTIVE: Insulin resistance (IR) is a hallmark of obesity and metabolic disease. Circulating extracellular RNAs (ex-RNAs), stable RNA molecules in plasma, may play a role in IR, though most studies on ex-RNAs in IR are small. We sought to characterize the relationship between ex-RNAs and metabolic phenotypes in a large community-based human cohort. RESEARCH DESIGN AND METHODS: We measured circulating plasma ex-RNAs in 2,317 participants without diabetes in the Framingham Heart Study (FHS) Offspring Cohort at cycle 8 and defined associations between ex-RNAs and IR (measured by circulating insulin level)...
February 9, 2017: Diabetes Care
https://www.readbyqxmd.com/read/28167244/impact-of-risperidone-on-leptin-and-insulin-in-children-and-adolescents-with-autistic-spectrum-disorders
#16
Pornpen Srisawasdi, Natchaya Vanwong, Yaowaluck Hongkaew, Apichaya Puangpetch, Somlak Vanavanan, Boontarika Intachak, Nattawat Ngamsamut, Penkhae Limsila, Chonlaphat Sukasem, Martin H Kroll
OBJECTIVE: To evaluate the influence of dose and duration of risperidone treatment on cardiovascular and diabetes risk biomarkers in children and adolescents with autistic spectrum disorders (ASDs). DESIGN AND METHODS: In this cross-sectional analysis, a total of 168 ASDs patients (89% male) treated with a risperidone-based regimen for ≥12months were included. Blood samples were analyzed for glucose and lipid metabolic markers, adiponectin, leptin, prolactin, cortisol and high sensitive C-reactive protein...
February 3, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/28129817/-dalbavancin-pharmacokinetic-and-pharmacodynamic-parameters
#17
José Ramón Azanza, Belén Sádaba, Joana Reis
Dalbavancin is a new lipoglycopeptide antibiotic whose structure influences its pharmacokinetic profile. It is not absorbed after oral administration and is therefore administered intravenously. It is distributed through intracellular fluid, reaching adequate concentrations in the skin, bone, blister fluid and synovial fluid. Plasma protein binding is very high. Concentrations in brain tissue and cerebrospinal fluid (CSF) are inadequate. Excretion is through non-microsomal metabolism with inactive metabolites and through the kidneys by glomerular filtration...
January 2017: Enfermedades Infecciosas y Microbiología Clínica
https://www.readbyqxmd.com/read/28099128/bone-health-assessment-of-food-allergic-children-on-restrictive-diets-a-practical-guide
#18
Artemis E Doulgeraki, Emmanouel M Manousakis, Nikolaos G Papadopoulos
BACKGROUND: Food allergy in childhood is on the rise globally and is managed with avoidance diets; recent case reports of food allergic children with nutritional rickets in the literature highlight the importance of close monitoring of bone health in this population. METHODS: There is no consensus as yet with regard to bone health evaluation in food allergic children; therefore, extensive literature search was performed and the existing evidence is presented, along with a relevant algorithm...
February 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28089618/24-hour-protein-arginine-and-citrulline-metabolism-in-fed-critically-ill-children-a-stable-isotope-tracer-study
#19
Carlijn T I de Betue, Xiomara C Garcia Casal, Dick A van Waardenburg, Stephen M Schexnayder, Koen F M Joosten, Nicolaas E P Deutz, Marielle P K J Engelen
BACKGROUND & AIMS: The reference method to study protein and arginine metabolism in critically ill children is measuring plasma amino acid appearances with stable isotopes during a short (4-8 h) time period and extrapolate results to 24-h. However, 24-h measurements may be variable due to critical illness related factors and a circadian rhythm could be present. Since only short duration stable isotope studies in critically ill children have been conducted before, the aim of this study was to investigate 24-h appearance of specific amino acids representing protein and arginine metabolism, with stable isotope techniques in continuously fed critically ill children...
January 4, 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28067901/targeting-samhd1-with-the-vpx-protein-to-improve-cytarabine-therapy-for-hematological-malignancies
#20
Nikolas Herold, Sean G Rudd, Linda Ljungblad, Kumar Sanjiv, Ida Hed Myrberg, Cynthia B J Paulin, Yaser Heshmati, Anna Hagenkort, Juliane Kutzner, Brent D G Page, José M Calderón-Montaño, Olga Loseva, Ann-Sofie Jemth, Lorenzo Bulli, Hanna Axelsson, Bianca Tesi, Nicholas C K Valerie, Andreas Höglund, Julia Bladh, Elisée Wiita, Mikael Sundin, Michael Uhlin, Georgios Rassidakis, Mats Heyman, Katja Pokrovskaja Tamm, Ulrika Warpman-Berglund, Julian Walfridsson, Sören Lehmann, Dan Grandér, Thomas Lundbäck, Per Kogner, Jan-Inge Henter, Thomas Helleday, Torsten Schaller
The cytostatic deoxycytidine analog cytarabine (ara-C) is the most active agent available against acute myelogenous leukemia (AML). Together with anthracyclines, ara-C forms the backbone of AML treatment for children and adults. In AML, both the cytotoxicity of ara-C in vitro and the clinical response to ara-C therapy are correlated with the ability of AML blasts to accumulate the active metabolite ara-C triphosphate (ara-CTP), which causes DNA damage through perturbation of DNA synthesis. Differences in expression levels of known transporters or metabolic enzymes relevant to ara-C only partially account for patient-specific differential ara-CTP accumulation in AML blasts and response to ara-C treatment...
February 2017: Nature Medicine
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