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Metabolics syndrome pediatrics

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https://www.readbyqxmd.com/read/28439217/pediatric-metabolic-syndrome-pathophysiology-and-laboratory-assessment
#1
Victoria Higgins, Khosrow Adeli
Pediatric overweight and obesity is an emerging public health priority as rates have rapidly increased worldwide. Obesity is often clustered with other metabolic abnormalities including hypertension, dyslipidemia, and insulin resistance, leading to increased risk of cardiovascular disease. This cluster of risk factors, termed the metabolic syndrome, has traditionally been reported in adults. However, with the increased prevalence of pediatric obesity, the metabolic syndrome is now evident in children and adolescents...
March 2017: EJIFCC
https://www.readbyqxmd.com/read/28437620/american-association-of-clinical-endocrinologists-and-american-college-of-endocrinology-guidelines-for-management-of-dyslipidemia-and-prevention-of-cardiovascular-disease
#2
Paul S Jellinger, Yehuda Handelsman, Paul D Rosenblit, Zachary T Bloomgarden, Vivian A Fonseca, Alan J Garber, George Grunberger, Chris K Guerin, David S H Bell, Jeffrey I Mechanick, Rachel Pessah-Pollack, Kathleen Wyne, Donald Smith, Eliot A Brinton, Sergio Fazio, Michael Davidson
OBJECTIVE: The development of these guidelines is mandated by the American Association of Clinical Endocrinologists (AACE) Board of Directors and American College of Endocrinology (ACE) Board of Trustees and adheres with published AACE protocols for the standardized production of clinical practice guidelines (CPGs). METHODS: Recommendations are based on diligent reviews of the clinical evidence with transparent incorporation of subjective factors, according to established AACE/ACE guidelines for guidelines protocols...
April 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28425133/fecal-microbiota-signatures-of-adult-patients-with-different-types-of-short-bowel-syndrome
#3
Yuhua Huang, Feilong Guo, Yousheng Li, Jian Wang, Jieshou Li
BACKGROUND AND AIM: Short bowel syndrome (SBS) is a common cause of intestinal failure and can be divided into three types depending on intestinal anatomy. Gut dysbiosis has been observed in pediatric SBS patients and is associated with impaired outcome. Little is known about the changes in gut microbiota of adult SBS patients. Therefore, we aim to characterize the fecal microbiota of adult patients with different types of SBS. METHODS: Fifteen fecal samples from healthy controls and adult patients with type II or type III SBS were collected (five in each group)...
April 19, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28414188/a-case-of-splenomegaly-in-cbl-syndrome
#4
Rachel R Coe, Margaret L McKinnon, Maja Tarailo-Graovac, Colin J Ross, Wyeth W Wasserman, Jan M Friedman, Paul C Rogers, Clara D M van Karnebeek
INTRODUCTION: We present a child with unexplained splenomegaly to highlight this feature as a presenting sign of the RASopathy CBL syndrome and to draw attention to the power and utility of next generation genomic sequencing for providing rapid diagnosis and critical information to guide care in the pediatric clinical setting. CLINICAL REPORT: A 7-year-old boy presented with unexplained splenomegaly, attention deficit hyperactivity disorder, mild learning difficulties, easy bruising, mild thrombocytopenia, and subtle dysmorphic features...
April 13, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28411097/-acute-liver-failure-related-to-inherited-metabolic-diseases-in-young-children
#5
Filipa Dias Costa, Rita Moinho, Sandra Ferreira, Paula Garcia, Luísa Diogo, Isabel Gonçalves, Carla Pinto
INTRODUCTION: Pediatric acute liver failure (ALF) due to inherited metabolic diseases (IMD) is a rare life-threatening condition with a poor prognosis. Early intervention may be lifesaving. OBJECTIVE: To describe clinical presentation, investigation and outcomes of ALF related to IMD in young children. MATERIAL AND METHODS: Retrospective review of the medical records of children aged up to 24 months, admitted to a tertiary pediatric and neonatal Intensive Care Unit during a 27-year period, fulfilling the ALF criteria, with documented metabolic etiology...
April 11, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28392825/a-compound-heterozygous-mutation-in-the-fmo3-gene-the-first-pediatric-case-causes-fish-odor-syndrome-in-korea
#6
Ji Hyun Kim, Sung Min Cho, Jong-Hee Chae
Trimethylaminuria (TMAuria), known as "fish odor syndrome," is a congenital metabolic disorder characterized by an odor resembling that of rotting fish. This odor is caused by the secretion of trimethylamine (TMA) in the breath, sweat, and body secretions and the excretion of TMA along with urine. TMAuria is an autosomal recessive disorder caused by mutations in flavin-containing monooxygenase 3 (FMO3). Most TMAuria cases are caused by missense mutations, but nonsense mutations have also been reported in these cases...
March 2017: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28378140/management-of-non-hodgkin-lymphoma-icmr-consensus-document
#7
Nirav Thacker, Sameer Bakhshi, Girish Chinnaswamy, Tushar Vora, Maya Prasad, Deepak Bansal, Sandeep Agarwala, Gauri Kapoor, Venkatraman Radhakrishnan, Siddharth Laskar, Tanvir Kaur, G K Rath, Rupinder Singh Dhaliwal, Brijesh Arora
Hitherto poor outcomes, paucity of data and heterogeneity in International approach to Pediatric NHL (Non-Hodgkin Lymphoma) prompted the need for guidelines for Indian population with vast variability in access, affordability and infrastructure across the country. These guidelines are based on consensus among the experts and best available evidence applicable to Indian setting. Evaluation of NHL should consist of easily doable and rapid tissue diagnosis (biopsy or flow cytometry of peripheral blood/malignant effusions), St Jude/IPNHLSS (International Pediatric Non-Hodgkin Lymphoma Staging System) and risk grouping with CSF (Cerebro-spinal fluid), bone marrow, whole body imaging [CECT (Contrast enhanced computerized tomography) ± MRI (Magnetic resonance imaging)] and blood investigations for LDH (Lactate dehydrogenase), TLS (Tumor lysis syndrome) and organ functions...
April 5, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28336101/metabolic-syndrome-hepatic-steatosis-and-cardiovascular-risk-in-children
#8
Irene Rutigliano, Roberta Vinci, Gianpaolo De Filippo, Monica Mancini, Luca Stoppino, Maria d'Apolito, Ida Giardino, Luca Macarini, Massimo Pettoello Mantovani, Angelo Campanozzi
OBJECTIVES: Pediatric metabolic syndrome (MetS) is a well-recognized entity; however, there is no consensus on its exact value in predicting long-term cardiovascular (CV) risk. Hepatic steatosis (HS) is another emerging condition associated with pediatric obesity, and data have been reported suggesting a possible role of HS in CV risk linked to MetS. The aim of the present study was to evaluate the usefulness of HS and MetS cluster in predicting CV risk linked to pediatric obesity. METHODS: We studied 803 overweight and obese children (395 girls and 408 boys, mean age 9...
April 2017: Nutrition
https://www.readbyqxmd.com/read/28333140/metabolic-and-molecular-insights-into-an-essential-role-of-nicotinamide-phosphoribosyltransferase
#9
Li Q Zhang, Leon Van Haandel, Min Xiong, Peixin Huang, Daniel P Heruth, Charlie Bi, Roger Gaedigk, Xun Jiang, Ding-You Li, Gerald Wyckoff, Dmitry N Grigoryev, Li Gao, Linheng Li, Min Wu, J Steven Leeder, Shui Qing Ye
Nicotinamide phosphoribosyltransferase (NAMPT) is a pleiotropic protein implicated in the pathogenesis of acute respiratory distress syndrome, aging, cancer, coronary heart diseases, diabetes, nonalcoholic fatty liver disease, obesity, rheumatoid arthritis, and sepsis. However, the underlying molecular mechanisms of NAMPT in these physiological and pathological processes are not fully understood. Here, we provide experimental evidence that a Nampt gene homozygous knockout (Nampt(-/-)) resulted in lethality at an early stage of mouse embryonic development and death within 5-10 days in adult mice accompanied by a 25...
March 23, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28324110/effects-of-metreleptin-in-pediatric-patients-with-lipodystrophy
#10
Rebecca J Brown, Cristina Adelia Meehan, Elaine Cochran, Kristina I Rother, David E Kleiner, Mary Walter, Phillip Gorden
Context: Lipodystrophy syndromes are rare disorders of deficient adipose tissue. Metreleptin, a human analog of leptin, improved metabolic abnormalities in mixed cohorts of leptin-deficient children and adults with lipodystrophy. Objective: Determine effects of metreleptin on diabetes, hyperlipidemia, non-alcoholic fatty liver disease (NAFLD), growth, and puberty in leptin-deficient pediatric patients with lipodystrophy. Design: Prospective, single-arm, open-label studies with continuous enrollment since 2000...
January 23, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28296430/successful-use-of-sertindole-for-severe-behavioral-dyscontrol-in-a-pediatric-case-of-syndromic-autism-spectrum-disorder
#11
Ahmed Naguy
Autism spectrum disorder (ASD) is commonly associated with a host of challenging behaviors. Pharmacotherapy is indicated if psychosocial and educational interventions fail. Atypical antipsychotics have the strongest evidence base so far, with both risperidone and aripiprazole being FDA approved. Unfortunately, their use is fraught with metabolic and neurohormonal side effects. In this study, the author is reporting on a case of syndromic ASD/moderate intellectual disability with severe behavioral component that failed multiple psychotropic trials and ultimately responded dramatically to sertindole...
March 15, 2017: Journal of Child and Adolescent Psychopharmacology
https://www.readbyqxmd.com/read/28284396/pyridoxal-phosphate-supplementation-in-neuropediatric-disorders
#12
Elisenda Cortès-Saladelafont, Marta Molero-Luis, Hsjd Working Group, Rafael Artuch, Àngels García-Cazorla
Pyridoxal phosphate (PLP) is the active form of vitamin B6 and a cofactor in many enzyme reactions including neurotransmitter metabolism. PLP metabolism disturbances may mostly lead to refractory seizures. In this report, we review the main pathophysiological factors related with PLP deficiency and our experience in PLP treatment in pediatric patients with low-normal cerebrospinal fluid PLP values who presented epilepsy. Only one case had a definite diagnosis (Phelan-McDermid syndrome). The results of extensive metabolic workups and targeted genetic studies were normal for all patients...
November 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28279074/oxidized-derivatives-of-linoleic-acid-in-pediatric-metabolic-syndrome-is-their-pathogenic-role-modulated-by-the-genetic-background-and-the-gut-microbiota
#13
Domenico Tricò, Anna Di Sessa, Sonia Caprio, Naga Chalasani, Wanqing Liu, Tiebing Liang, Joerg Graf, Raimund I Herzog, Casey D Johnson, Giuseppina Rosaria Umano, Ariel E Feldstein, Nicola Santoro
We tested whether oxidized linoleic acid metabolites (OXLAM) are associated with pediatric metabolic syndrome (MetS) and a proatherogenic lipoprotein profile in 122 obese adolescents. Furthermore, we examined whether genetic and metagenomic factors can modulate plasma OXLAM concentrations by genotyping the fatty acid desaturase 1/2 (FADS) gene and by characterizing the gut microbiota. Subjects with MetS (n = 50) showed higher concentrations of 9- and 13-oxo-octadecadienoic acid (9- and 13-oxo-ODE) than subjects without MetS (n = 72)...
April 7, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/28272108/looking-for-new-diagnostic-tools-and-biomarkers-of-hypertension-in-obese-pediatric-patients
#14
Wojciech Strojny, Dorota Drozdz, Kamil Fijorek, Michał Korostynski, Marcin Piechota, Walentyna Balwierz, Jacek A Pietrzyk, Przemko Kwinta, Maciej Siedlar, Szymon Skoczen
INTRODUCTION: Development of obesity in childhood may be linked to an increased risk of hypertension. OBJECTIVES: This study aimed (a) to analyze the expression of genes associated with blood pressure (BP) in obese children, (b) to evaluate ambulatory blood pressure monitoring (ABPM) as a diagnostic tool in hypertension in children, and (c) to assess the prevalence of metabolic syndrome in children with obesity. PATIENTS AND METHODS: Office BP measurements and ABPM were performed in 49 children with obesity and 25 age-matched healthy children...
March 7, 2017: Blood Pressure Monitoring
https://www.readbyqxmd.com/read/28258649/the-cardiac-manifestations-of-inherited-metabolic-diseases-in-children
#15
REVIEW
David F A Lloyd, Roshni Vara, Sujeev Mathur
Inborn errors of metabolism (IEMs) are responsible for around 5% of all cases of cardiomyopathy (CM) and 15% of non-idiopathic cases. Storage disorders such as Pompe disease (glycogen storage disease type II) typically cause hypertrophic cardiomyopathy, whereas the accumulation of toxic metabolites, as seen in the organic acidurias, is associated with dilated cardiomyopathy (DCM). Mixed pathology is also possible, particularly in late presentations. IEMs such as Barth syndrome, a disorder of cardiolipin stability usually associated with DCM, have been associated with rarer types of CM such as endocardial fibroelastosis (EFE) and left ventricular non-compaction...
March 4, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28248832/pathophysiology-of-pediatric-multiple-organ-dysfunction-syndrome
#16
Joseph A Carcillo, Bradley Podd, Rajesh Aneja, Scott L Weiss, Mark W Hall, Timothy T Cornell, Thomas P Shanley, Lesley A Doughty, Trung C Nguyen
OBJECTIVE: To describe the pathophysiology associated with multiple organ dysfunction syndrome in children. DATA SOURCES: Literature review, research data, and expert opinion. STUDY SELECTION: Not applicable. DATA EXTRACTION: Moderated by an experienced expert from the field, pathophysiologic processes associated with multiple organ dysfunction syndrome in children were described, discussed, and debated with a focus on identifying knowledge gaps and research priorities...
March 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28242438/heritability-parental-transmission-and-environment-correlation-of-pediatric-onset-type-2-diabetes-mellitus-and-metabolic-syndrome-related-traits
#17
América L Miranda-Lora, Jenny Vilchis-Gil, Mario Molina-Díaz, Samuel Flores-Huerta, Miguel Klünder-Klünder
AIM: To estimate the heritability, parental transmission and environmental contributions to the phenotypic variation in type 2 diabetes mellitus and metabolic syndrome-related traits in families of Mexican children and adolescents. METHODS: We performed a cross-sectional study of 184 tri-generational pedigrees with a total of 1160 individuals (99 families with a type 2 diabetes mellitus proband before age 19). The family history of type 2 diabetes mellitus in three generations was obtained by interview...
February 10, 2017: Diabetes Research and Clinical Practice
https://www.readbyqxmd.com/read/28222035/nutritional-status-and-metabolic-profile-in-neurologically-impaired-pediatric-surgical-patients
#18
Gloria Pelizzo, Valeria Calcaterra, Veronica Carlini, Mario Fusillo, Matteo Manuelli, Catherine Klersy, Noemi Pasqua, Elona Luka, Riccardo Albertini, Mara De Amici, Hellas Cena
BACKGROUND: Malnutrition is reported in pediatric neuromotor disability and impacts the child's health. We described the nutritional and metabolic status in neurologically impaired (NI) children undergoing surgery. METHODS: Anthropometry, body composition, hormonal and nutritional evaluations were performed in 44 NI subjects (13.7±8.0 years). Energy needs were calculated by Krick's formula. Metabolic syndrome (MS) was defined applying the following criteria (≥3 defined MS): fasting blood glucose >100 mg/dL and/or homeostasis model assessment for insulin resistance (HOMA-IR) >97...
March 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28215263/sleep-disordered-breathing-systemic-adipokine-secretion-and-metabolic-dysregulation-in-overweight-and-obese-children-and-adolescents
#19
Annelies Van Eyck, Kim Van Hoorenbeeck, Benedicte Y De Winter, Luc Van Gaal, Wilfried De Backer, Stijn L Verhulst
OBJECTIVE: Obstructive sleep apnea (OSA) is common among overweight and obese children, and it is an independent risk factor for developing metabolic syndrome. However, the mechanisms linking OSA and metabolic syndrome are still unclear, but a role for adipose tissue dysfunction caused by intermittent hypoxia has been suggested. Therefore, the goal of this study was to investigate the relationship between OSA and systemic adipokine concentrations in overweight and obese children. METHODS: We included 164 overweight and obese children in a tertiary center and distributed them in groups based on their obstructive apnea-hypopnea index (111 controls, 28 mild OSA, 25 moderate-to-severe OSA)...
February 2017: Sleep Medicine
https://www.readbyqxmd.com/read/28193268/adipokines-insulin-resistance-and-adiposity-as-a-predictors-of-metabolic-syndrome-in-child-survivors-of-lymphoma-and-acute-lymphoblastic-leukemia-of-a-developing-country
#20
Lourdes Barbosa-Cortés, Mardia López-Alarcón, Juan Manuel Mejía-Aranguré, Miguel Klünder-Klünder, María Del Carmen Rodríguez-Zepeda, Hugo Rivera-Márquez, Alan de la Vega-Martínez, Jorge Martin-Trejo, Juan Shum-Luis, Karina Solis-Labastida, Enrique López-Aguilar, Guadalupe Matute-González, Roberto Bernaldez-Rios
BACKGROUND: There is a growing body of evidence indicating that pediatric survivors of cancer are at a greater risk of developing metabolic syndrome. This study evaluated some probable predictors of metabolic syndrome (MS), such as leptin and adiponectin concentrations, the leptin/adiponectin ratio, insulin resistance, and adiposity, in a sample of child survivors of lymphoma and leukemia in Mexico City. METHODS: Fifty two children (leukemia n = 26, lymphoma n = 26), who were within the first 5 years after cessation of therapy, were considered as eligible to participate in the study...
February 13, 2017: BMC Cancer
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