44 results match your criteria: "Pediatric University Clinic[Affiliation]"

Article Synopsis
  • S-adenosylhomocysteine hydrolase deficiency is an autosomal recessive metabolic disorder disrupting the methionine cycle, which can range from severe symptoms to asymptomatic cases.
  • Two clinically asymptomatic siblings from Pakistan were diagnosed with mild chronic liver failure and other mild symptoms, with one sibling showing improvements after dietary changes.
  • The research highlights a specific genetic variant common in South Asia and suggests this milder form of the disease may be underdiagnosed, emphasizing the need for better therapeutic management to prevent future health complications.
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Objective: This study aimed to explore the utility of a high-intensity interval training (HIIT) app as a remote exercise support strategy in children with obesity through assessing adherence, possible effects on obesity-related outcomes, and perceptions of its use to support counseling and improve the treatment of children with obesity.

Methods: Thirty-seven children (94.6% with obesity; 45.

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Palliative Care in the Ageing European Population: A Cross-Country Comparison.

Int J Environ Res Public Health

January 2024

CINTESIS@RISE, Biochemistry Lab, Faculty of Pharmacy, University of Porto, 4050-313 Porto, Portugal.

With Europe's ageing population and rising demand for palliative care, it is crucial to examine the use of palliative care among older adults during their last years of life and understand the factors influencing their access and end-of-life circumstances. This study employed a cohort of SHARE participants aged 65 years or older who had passed away between Wave 6 (2015) and Wave 7 (2017). Information on death circumstances, palliative care utilization, and associated variables were analysed.

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Glutaric Acidemia Type 1: Diagnosis, Clinical features, and Outcome in a Portuguese Cohort.

Endocr Metab Immune Disord Drug Targets

September 2023

Hereditary Metabolic Disease Reference Center, Metabolic Unit, Pediatric Department, Santa Maria's Hospital-Lisbon North University Hospital Center, EPE, Pediatric University Clinic, Faculty of Medicine, University of Lisbon, Lisbon, Portugal.

Introduction: Glutaric acidemia type 1 (GA1) is a rare autosomal recessive disorder characterized by a deficiency of glutaryl-CoA dehydrogenase, resulting in the accumulation of glutaric acid (GA), 3-hydroxyglutaric acid, and glutarylcarnitine, especially in the brain. GA1-affected children are clinically characterized by macrocephaly. Neurological abnormalities usually appear between 6 and 18 months of age, often triggered by a catabolic event.

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Background: Recognizing the need for palliative care (PC) and referral to PC teams improves patients' quality of life. However, in patients with moderate/severe clinical complexity, early recognition of the need for PC may not correspond to referral to specialized PC services. The definition for clinical complexity is still underexplored, as well as the instruments available to assess complexity.

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Atherosclerosis Prevention in Adolescents with Obesity: The Role of Moderate-Vigorous Physical Activity.

Int J Environ Res Public Health

November 2022

Pediatric University Clinic, Faculty of Medicine, Universidade de Lisboa, 1649-035 Lisbon, Portugal.

Carotid intima-media thickness (cIMT) is a subclinical marker of atherosclerotic development, which is impaired in adolescents with obesity. This study aimed to analyze the impact of physical activity (PA), cardiorespiratory fitness (CRF), body mass index (BMI), and body composition changes on the cIMT of adolescents with obesity. Longitudinal data (6 months) from adolescents aged 12-18 years, with a BMI ≥97th percentile, previously recruited for the non-randomized controlled trial PAC-MAnO (Clinicaltrials.

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Food portion sizes and their relationship with energy, and nutrient intakes in adolescents: The HELENA study.

Nutrition

February 2023

Growth, Exercise, Nutrition and Development Research Group, Faculty of Health Sciences, University of Zaragoza, Zaragoza, Spain; CIBER Fisiopatología de la Obesidad y Nutrición, Instituto de Salud Carlos III, Madrid, Spain; Instituto de Investigación Sanitaria Aragón, Zaragoza, Spain.

Objectives: This study aimed to investigate the associations between portion sizes (PSs) from different food groups and energy, as well as nutrient intakes in European adolescents.

Methods: A sample of 1631 adolescents (54.2 % girls) were included from the Healthy Lifestyle in Europe by Nutrition in Adolescence Cross-Sectional (HELENA) study.

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Acquired Vitamin B12 Deficiency in Newborns: Positive Impact on Newborn Health through Early Detection.

Nutrients

October 2022

Hereditary Metabolic Disease Reference Center, Metabolic Unit, Pediatric Department, Santa Maria's Hospital-Lisbon North University Hospital Center, EPE, Pediatric University Clinic, Faculty of Medicine, University of Lisbon, 1600-190 Lisbon, Portugal.

The early diagnosis of and intervention in vitamin B12 deficiency in exclusively breastfed infants by mothers with low vitamin B12 is crucial in preventing possible irreversible neurologic damage, megaloblastic anemia, and failure to thrive. We assess the usefulness of the early detection of asymptomatic B12 deficiency related to acquired conditions and highlight the importance of monitoring serum vitamin B12 levels during pregnancy. We describe demographic, clinical, dietary, and biochemical data, including the evolution of a vitamin B12 deficiency's functional biomarkers.

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Background: The family environment influences food consumption and behaviours, which impact adolescent's eating habits, diet and health. Young individuals who frequently eat family meals are less likely to develop risk- and behaviour-related outcomes as obesity.

Aim: To assess the relationship between the family meal environment and food and macronutrient consumption in European adolescents.

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Vigorous physical activity: A potential ally in adolescent obesity management.

Eur J Sport Sci

April 2023

Rheumatology Research Unit, Molecular Medicine Institute, Faculty of Medicine, University of Lisbon; Pediatric Obesity Clinic, Department of Pediatrics, Hospital de Santa Maria, Lisbon, Portugal.

Individual variability may contribute to the modest and inconsistent results reported in obesity-management interventions. This study aimed to investigate the impact of non-modifiable as well as modifiable factors on body mass index (BMI) and body fat variance in adolescents with obesity followed in a clinical obesity-management programme, in order to better understand individual variability. Non-modifiable factors (i.

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Birth weight and breastfeeding are differentially associated with physical fitness components.

Eur J Clin Nutr

June 2022

Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, and CIC 1403 - Clinical Investigation Center, F-59000, Lille, France.

Background/objectives: The study purpose was to assess the impact of birth weight and breastfeeding duration on physical fitness components.

Subjects/methods: Study participants were 985 adolescents boys and 1246 girls (12.5-17.

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Background: The association between high fructose consumption and elevated blood pressure continues to be controversial, especially in adolescence. The aim of this study was to assess the association between fructose consumption and elevated blood pressure in an European adolescent population.

Methods: A total of 1733 adolescents (mean ± SD age: 14.

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Effective and safe exercise protocols for obesity management in adolescents are imperative. This study aimed to analyse compliance, efficacy, and safety of combined high-intensity interval training circuit (HIIT) in the management of obesity (including overweight) in adolescents, compared to traditional training (TT). Data from 55 adolescents (47.

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The value of physical activity (PA) counselling and its impact on PA behaviour and weight management have been in question. The main aim of this study was to analyse 6 and 12-month effects of a PA consultation (PAC-a structured form of PA counselling) with and without the inclusion of structured exercise, on body mass index (BMI) z-score, body composition and PA levels of adolescents with excess weight (BMI ≥p85), as part of a clinical multicomponent weight management program. Participants were allocated at baseline into a control (CG-standard care, including paediatric and nutrition consultations) and two experimental groups (EGI and EGII).

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A recent study concerning the "Impact of COVID-19 on the Interrelation of Physical Activity, Screen Time and Health-Related Quality of Life in Children and Adolescents in Germany" was investigated by Wunsch et al. [..

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Adolescents' dietary polyphenol intake in relation to serum total antioxidant capacity: the HELENA study.

Int J Food Sci Nutr

February 2022

Department of Public Health and Primary Care, Faculty of Medicine and Health Sciences, Ghent University, Ghent, Belgium.

We evaluated the association between intake of total polyphenols, polyphenol classes and the 10 most consumed individual polyphenols with serum total antioxidant capacity (TAC) in 749 European adolescents (53% girls; 15% overweight; 12.5-17.5 years-old) from the cross-sectional HELENA study of 2006-2007.

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Background: Dietary misreporting is the main limitation of dietary assessments and has been associated with BMI during youth. However there are no prior studies assessing misreporting and cardiometabolic risks (CMRs) in adolescence.

Objectives: To examine the associations between dietary misreporting and CMR factors in adolescents and to assess the potential bias in the association between CMR and energy intake (EI) driven by dietary misreporting.

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Metabolically healthy overweight adolescents: definition and components.

Ann Pediatr Endocrinol Metab

December 2020

Pediatric Obesity Clinic, Department of Pediatrics, Hospital de Santa Maria, Lisbon; Rheumatology Research Unit, Molecular Medicine Institute, Faculty of Medicine, University of Lisbon, Lisbon, Portugal.

Purpose: In adolescents, the definition and clinical implications of metabolically healthy overweight (MHO) status have not been established. This study aimed to investigate the prevalence of MHO according to its most widespread definition, which is based on metabolic syndrome (MS), and to explore further metabolic indicators such as Homeostatic Model Assessment of Insulin Resistance, total cholesterol, low-density lipoprotein cholesterol, alanine aminotransferase, and C-reactive protein levels, together with metabolic health predictors in a sample of adolescents attending a pediatric obesity clinic.

Methods: Data from 487 adolescents categorized as overweight (52.

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Background: Investigating life-stage factors associated with overweight may be useful in the prevention of excessive BMI increase. The main aim of this study was to investigate the influence of the route of delivery, birth weight and overweight onset on overweight severity in a sample of overweight adolescents followed at a Pediatric Obesity Clinic.

Methods: Clinical data from 412 adolescents with overweight (BMI ≥ p85), aged 10-18 were retrospectively collected and analyzed.

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Inborn Errors of Metabolism in a Tertiary Pediatric Intensive Care Unit.

J Pediatr Intensive Care

September 2022

Pediatric Intensive Care Unit, Department of Pediatric, Santa Maria's Hospital - Lisbon North University Hospital Center, EPE, Pediatric University Clinic, Faculty of Medicine, University of Lisbon, Lisbon, Portugal.

Few studies exist describing resources and care of pediatric patients with inborn errors of metabolism (IEM) admitted to pediatric intensive care unit (PICU). This study aims to characterize the PICU admissions of these patients to provide better diagnostic and therapeutic care in the future. Retrospective analysis of pediatric patients with IEM admitted to the PICU of a tertiary care center at a metabolic referral university hospital from 2009 to 2019 was included.

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A new measure of health motivation influencing food choices and its association with food intakes and nutritional biomarkers in European adolescents.

Public Health Nutr

April 2020

Growth, Exercise, Nutrition and Development (GENUD) Research Group, Universidad de Zaragoza, Instituto Agroalimentario de Aragón (IA2), Instituto de Investigación Sanitaria de Aragón (IIS Aragón) and Centro de Investigación Biomédica en Red de Fisiopatología de la Nutrición y la Obesidad (CIBEROBN), Zaragoza50009, Spain.

Article Synopsis
  • The goal of the study was to create a new way to measure how health motivation affects food choices and see how it connects to what people eat and their health indicators.
  • Researchers used surveys and health tests from 2954 teens across ten cities in Europe to gather information.
  • The new measurement tool, called SOFIE, shows that people who score higher are likely to eat healthier foods like cereals, dairy, meats, and fish, and also have better vitamins in their blood, while lower scores are linked to drinking alcohol.
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Objectives: European dietary regulations affect national dietary guidelines for child nutrition. The update of the German Dietary Scheme for the first year of life is used to examine the translation of European nutrient references into food-based guidelines while maintaining traditional habits.

Methods: Within the Dietary Scheme, intake of energy and nutrients was calculated in the complementary feeding period for each of the 3 daily recommended complementary meals (a vegetable-potato-meat meal, a milk-cereal meal, a cereal-fruit meal) in addition to the daily liquid-milk servings (breast milk or follow-on formula).

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In a pilot study, we wanted to influence the food selection of employees in a pediatric clinic bistro aiming to increase the sale of "healthy" grain buns (number and proportion of all sold buns). During basic assessment, the mean weekly sale of grain buns was 98 (52.3%) and in the second week of highlighting them on a green napkin under a transparent hood (intervention 1) reached 124 (54.

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NGLY1 deficiency-A rare congenital disorder of deglycosylation.

JIMD Rep

May 2020

Metabolic Diseases Unit, Pediatric Department, Santa Maria's Hospital - Lisbon North University Hospital Center EPE, Pediatric University Clinic, Faculty of Medicine, University of Lisbon Lisbon Portugal.

Pathogenic variants in the NGLY1 gene are associated with a Congenital Disorder of Deglycosylation (CDDG) characterized by delays in reaching developmental milestones, complex hyperkinetic movement disorder, transient elevation of transaminases, and alacrima or hypolacrima. To date, only few cases of NGLY1 deficiency have been identified and reported in the literature. This report highlights a first child of non-consanguineous parents with no relevant family history who presented with hypotonia and poor weight gain since birth.

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Background: Although high dietary polyphenol intake is negatively associated with risk of certain inflammation-associated chronic diseases, the underlying mechanisms are not fully understood and few studies have explored this in adolescents.

Objective: This study aimed to evaluate the association between intakes of total polyphenols, polyphenol classes, and the 10 most commonly consumed individual polyphenols with inflammatory biomarkers in the blood of European adolescents.

Methods: In the Healthy Lifestyle in Europe by Nutrition in Adolescence (HELENA) Study, 526 adolescents (54% girls; 12.

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