keyword
MENU ▼
Read by QxMD icon Read
search

protein metabolism children

keyword
https://www.readbyqxmd.com/read/28392417/pharmacological-inhibition-of-carnitine-palmitoyltransferase-1-restores-mitochondrial-oxidative-phosphorylation-in-human-trifunctional-protein-deficient-fibroblasts
#1
Bruno Lefort, Elodie Gouache, Cécile Acquaviva, Marine Tardieu, Jean François Benoist, Jean-François Dumas, Stéphane Servais, Stéphan Chevalier, Christine Vianey-Saban, François Labartheh
BACKGROUND: Mitochondrial Trifunctional Protein deficiency (TFPD) is a severe genetic disease characterized by altered energy metabolism and accumulation of long-chain (LC) acylcarnitines in blood and tissues. This accumulation could impair the mitochondrial oxidative phosphorylation (OxPhos), contributing to the non-optimal outcome despite conventional diet therapy with medium-chain triglycerides (MCT). METHOD: Acylcarnitine and OxPhos parameters were measured in TFPD-fibroblasts obtained from 8 children and cultured in medium mimicking fasting (LCFA) or conventional treatment (MCT), with or without Etomoxir (ETX) an inhibitor of carnitine palmitoyltransferase 1 (CPT1) activity, and were compared to results obtained with fibroblasts from 5 healthy-control children...
April 6, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28388381/protein-requirements-of-the-critically-ill-pediatric-patient
#2
Jorge A Coss-Bu, Jill Hamilton-Reeves, Jayshil J Patel, Claudia R Morris, Ryan T Hurt
This article includes a review of protein needs in children during health and illness, as well as a detailed discussion of protein metabolism, including nitrogen balance during critical illness, and assessment and prescription/delivery of protein to critically ill children. The determination of protein requirements in children has been difficult and challenging. The protein needs in healthy children should be based on the amount needed to ensure adequate growth during infancy and childhood. Compared with adults, children require a continuous supply of nutrients to maintain growth...
April 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/28386663/a-child-with-phenylketonuria-and-focal-segmental-glomerulosclerosis-the-bright-side-of-proteinuria
#3
Fatma Rabah, Khalid Al-Thihli, Mohamed El-Naggari, Ibtisam B Elnour
Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism. Phenylalanine hydroxylase is the underlying deficient enzyme. If left untreated, growth failure, microcephaly, global developmental delay, seizures and severe intellectual impairment would characterize the clinical picture of PKU. On the other side of protein homeostasis lies nephrotic syndrome. It is a well-known quantitative defect due to significant proteinuria. Focal segmental glomerulosclerosis (FSGS) is a special congenital variant affecting children and adults...
April 7, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28385328/adiponectin-profile-and-irisin-expression-in-italian-obese-children-association-with-insulin-resistance
#4
Ersilia Nigro, Olga Scudiero, Maria Ludovica Monaco, Rita Polito, Pietro Schettino, Anna Grandone, Laura Perrone, Emanuele Miraglia Del Giudice, Aurora Daniele
Adiponectin (Acrp30), its high molecular weight (HMW) oligomers, and Irisin are molecules involved in several metabolic processes. To investigate if these cytokines could represent new metabolic markers, we evaluated the expression of Acrp30 and Irisin in serum of obese children from South Italy affected by different degrees of insulin resistance (IR). The anthropometric and metabolic features were evaluated in 27 obese children versus 13 age-matched controls. The expression of Acrp30, its pattern and Irisin were investigated by ELISA, western blotting and fast protein liquid chromatography...
April 3, 2017: Cytokine
https://www.readbyqxmd.com/read/28382600/treatment-adherence-and-psychological-wellbeing-in-maternal-carers-of-children-with-phenylketonuria-pku
#5
Emma Medford, Dougal Julian Hare, Katie Carpenter, Stewart Rust, Simon Jones, Anja Wittkowski
Phenylketonuria (PKU), a rare metabolic disorder, causes cognitive impairment unless treated with a strict, protein-restricted diet, but few studies have examined the relationship between treatment compliance and parental wellbeing. In the present study, 46 primary caregivers of children with PKU completed measures of psychological distress, parenting stress (related to caring for a child with an illness), resilience, perceived social support and child dependency. Treatment adherence was assessed using the proportion of blood phenylalanine concentrations within target range in the preceding year...
April 6, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28379580/association-of-tsh-with-cardiovascular-disease-risk-in-overweight-and-obese-children-during-lifestyle-intervention
#6
J M Rijks, J Plat, E Dorenbos, B Penders, Wjm Gerver, Ace Vreugdenhil
Context: Overweight and obese children have an increased risk to develop cardiovascular diseases(CVD) in which thyroid stimulating hormone(TSH) has been suggested as an intermediary factor. However, results of cross-sectional studies are inconclusive and intervention studies investigating changes in TSH concentrations in association with changes in cardiovascular risk parameters in overweight and obese children are scarce. Objective: To gain insight in associations of circulating TSH concentrations and cardiovascular risk parameters in overweight and obese children...
March 31, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28356294/exocytosis-proteins-as-novel-targets-for-diabetes-prevention-and-or-remediation
#7
Arianne Aslamy, Debbie C Thurmond
Diabetes remains one of the leading causes of morbidity and mortality worldwide, affecting an estimated 422 million adults. In the U.S. it is predicted that 1 in every 3 children born as of 2000 will suffer from diabetes in their lifetime. Type 2 diabetes results from combinatorial defects in pancreatic beta cell glucose-stimulated insulin secretion and in peripheral glucose uptake. Both processes, insulin secretion and glucose uptake, are mediated by exocytosis proteins, SNARE (soluble N-ethylmaleimide-sensitive factor attachment protein receptor) complexes, Sec1/Munc18 (SM), and Double C2-domain protein B (DOC2B)...
March 29, 2017: American Journal of Physiology. Regulatory, Integrative and Comparative Physiology
https://www.readbyqxmd.com/read/28338217/fasting-up-regulates-ferroportin-1-expression-via-a-ghrelin-ghsr-mapk-signaling-pathway
#8
Qian-Qian Lou, Yu-Fu Zhou, Mesona Yung-Jin Chen, Li Liu, Juan Ma, Meng-Wan Zhang, Fa-Li Zhang, Ya Ke, Zhong-Ming Qian
The significant positive correlation between ghrelin and iron and hepcidin levels in the plasma of children with iron deficiency anemia prompted us to hypothesize that ghrelin may affect iron metabolism. Here, we investigated the effects of fasting or ghrelin on the expression of hepcidin, ferroportin 1 (Fpn1), transferrin receptor 1 (TfR1), ferritin light chain (Ft-L) proteins and ghrelin, and also hormone secretagogue receptor 1 alpha (GHSR1α) and ghrelin O-acyltransferase (GOAT) mRNAs in the spleen and/or macrophage...
March 24, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28333398/dietary-cholesterol-interacts-with-srebf1-to-modulate-obesity-in-chinese-children
#9
Shixiu Zhang, Xinying Lin, Henry Lynn, Guifa Xu, Jun Li, Changfeng Zhao, Mingmei Li
SCOPE: Sterol Regulatory Element Binding Protein 1 Gene (SREBF1) is an important candidate gene for obesity that could be affected by cholesterol. Different SREBF1 gene variants may have distinct responses to cholesterol, leading to different risks for obesity and obesity-related metabolic traits. Thus, we performed a gene-by-diet correlation analysis to test whether SREBF1 gene variation modulate the relationship between cholesterol and obesity. METHODS AND RESULTS: A total of 642 school-aged children in Jinan, China, were selected by stratified cluster nested sampling...
March 23, 2017: Molecular Nutrition & Food Research
https://www.readbyqxmd.com/read/28329079/sleep-duration-and-cardiometabolic-risk-among-chinese-school-aged-children-do-adipokines-play-a-mediating-role
#10
Lujiao Li, Junling Fu, Xin Ting Yu, Ge Li, Lu Xu, Jinghua Yin, Hong Cheng, Dongqing Hou, Xiaoyuan Zhao, Shan Gao, Wenhui Li, Changhong Li, Struan F A Grant, Mingyao Li, Yi Xiao, Jie Mi, Ming Li
Objective: To assess the associations between sleep duration and cardiometabolic risk factors in Chinese school-aged children and to explore the possible mediating role of adipokines. Methods: Sleep duration was collected in 3,166 children from the Beijing Child and Adolescent Metabolic Syndrome study. Glucose homeostasis and other cardiometabolic risk factors were assessed. Serum adipokines including leptin, total and high-molecular-weight (HMW) adiponectin, resistin, fibroblast growth factor 21 (FGF21) and retinol binding protein 4 (RBP4) were determined...
March 9, 2017: Sleep
https://www.readbyqxmd.com/read/28324661/improved-risk-stratification-in-pediatric-septic-shock-using-both-protein-and-mrna-biomarkers-persevere-xp
#11
Hector R Wong, Natalie Z Cvijanovich, Nick Anas, Geoffrey L Allen, Neal J Thomas, Michael T Bigham, Scott L Weiss, Julie C Fitzgerald, Paul A Checchia, Keith Meyer, Michael Quasney, Mark Hall, Rainer Gedeit, Robert J Freishtat, Jeffrey Nowak, Shekhar S Raj, Shira Gertz, Jocelyn R Grunwell, Christopher J Lindsell
RATIONALE: We previously derived and validated the Pediatric Sepsis Biomarker Risk Model (PERSEVERE) to estimate baseline mortality risk in children with septic shock. The PERSEVERE biomarkers are serum proteins, selected from among the proteins directly related to 80 mortality risk assessment genes. The initial approach to selecting the PERSEVERE biomarkers left 68 genes unconsidered. OBJECTIVE: Determine if the 68 previously unconsidered genes can improve upon the performance of PERSEVERE and provide biological information regarding the pathophysiology of septic shock...
March 21, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28315884/complementary-feeding-micronutrients-and-developmental-outcomes-of-children
#12
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
#13
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. 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 and epileptic spasms. Epileptic spasms were observed in patients with ALG1-, ALG6, ALG11-CDG and CDG-Ix, and occurred at an early age, before 6 months in all cases, except one who had spasms that started at 18 months...
March 1, 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
#14
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
#15
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
#16
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...
April 3, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28278160/hyperleptinemia-in-children-with-autosomal-recessive-spinal-muscular-atrophy-type-i-iii
#17
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
#18
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
#19
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
#20
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
keyword
keyword
47362
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"