Metabolic syndrome in children and adolescents with phenylketonuria

Kanufre, Viviane C.; Soares, Rosângelis D. L.; Alves, Michelle Rosa Andrade; Aguiar, Marcos José Burle de; Starling, Ana Lúcia Pimenta; Norton, Rocksane de Carvalho

doi:10.1016/j.jped.2014.06.006

Cited by 22 publications

(10 citation statements)

References 26 publications

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“…It also helps prevent the onset of chronic noncommunicable diseases, considering that being overweight is a primary risk factor for the onset of metabolic disorders. 33,34 Kanufre et al 35 found higher blood concentrations of triglycerides and basal insulin, a higher total cholesterol/a high density lipoprotein ratio (HDL-c) and index homeostatic model…”

Section: Discussionmentioning

confidence: 99%

Overweight and Associated Factors in Children and Adolescents With Phenylketonuria: A Systematic Review

Sena

Andrade

Silva

et al. 2020

Rev. paul. pediatr.

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Objective: To verify the occurrence of overweight in children and adolescents with phenylketonuria and to identify possible causal factors.Data sources: A systematic review was performed in the SciELO, PubMed and VHL databases using the descriptors "Phenylketonurias", "Overweight", "Child" and "Adolescent". Original articles conducted with children and adolescents, published between 2008 and 2018 in Portuguese, English or Spanish languages were included.Data synthesis: A total of 16 articles were identified and, after screening procedures, 6 studies were selected for the review.Overweight in children and adolescents with phenylketonuria was a frequent occurence in the studies included in this review, ranging from 7.8 to 32.6%. The female sex was the most affected by the nutritional disorder. Furthermore, a high caloric intake combined with a lack of stimuli to practice physical activities were main factors associated with the excessive weight in the population of interest.Conclusions: Excess weight can be considered a common outcome in children and adolescents with phenylketonuria. It is mainly caused by inadequate food consumption and sedentary lifestyle.The importance of early identification of nutritional disturbances in children and adolescents with phenylketonuria should be emphasized, in order to prevent associated chronic diseases and to promote health by encouraging continued healthy eating habits and the regular practice of physical exercises.Objetivo: Verificar a ocorrência de excesso de peso em crianças e adolescentes com fenilcetonúria e identificar possíveis fatores causais. Fontes de dados: Revisão sistemática realizada nas bases de dados Scientific Eletronic Library Online (SciELO), Publisher Medline (PubMed) e Biblioteca Virtual em Saúde (BVS) com os descritores "Phenylketonurias", "Overweight", "Child" e "Adolescent". Foram incluídos artigos originais sobre crianças e adolescentes, publicados entre 2008 e 2018 nos idiomas português, inglês ou espanhol. Síntese dos dados: Foram identificados 16 artigos e, após aplicação dos procedimentos de seleção, 6 estudos foram selecionados para compor a revisão. O excesso de peso em crianças e adolescentes com fenilcetonúria foi evento frequente nos estudos incluídos na presente revisão, variando de 7,8 a 32,6%. Aponta-se o sexo feminino como o grupo mais acometido pelo distúrbio nutricional.O principal fator associado ao excesso de peso na população de interesse na população de interesse foi o consumo calórico elevado aliado à falta de estímulos para a prática de atividades físicas.Conclusões: O excesso de peso pode ser considerado um desfecho comum em crianças e adolescentes com fenilcetonúria, sendo ocasionado principalmente pelo consumo alimentar inadequado e pelo sedentarismo. Salienta-se a importância da identificação precoce de agravos nutricionais em crianças e adolescentes fenilcetonúricos, a fim de prevenir doenças crônicas associadas e promover a saúde, com incentivo à manutenção de hábitos alimentares saudáveis e à prática regular de exercícios fí...

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Section: Discussionmentioning

confidence: 99%

Overweight and Associated Factors in Children and Adolescents With Phenylketonuria: A Systematic Review

Sena

Andrade

Silva

et al. 2020

Rev. paul. pediatr.

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“…It is interesting to note that several of the conditions identified among PKU patients in this study (diabetes mellitus, dyslipidemia, obesity) are constituents of the metabolic syndrome. Kanufre et al [24] found that overweight PKU patients may be vulnerable to the development of the metabolic syndrome.…”

Section: Discussionmentioning

confidence: 99%

Clinical burden of illness in patients with phenylketonuria (PKU) and associated comorbidities - a retrospective study of German health insurance claims data

Trefz

Muntau

Kohlscheen³

et al. 2019

Orphanet J Rare Dis

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Background Phenylketonuria (PKU) is an inherited deficiency in the enzyme phenylalanine hydroxylase (PAH), which, when poorly-managed, is associated with clinical features including deficient growth, microcephaly, seizures, and intellectual impairment. The management of PKU should start as soon as possible after diagnosis to prevent irreversible damage and be maintained throughout life. The aim of this study was to assess the burden of illness in PKU patients in general and in PKU patients born before and after the introduction of newborn screening in Germany. Methods This retrospective matched cohort analysis used the Institut für angewandte Gesundheitsforschung Berlin (InGef) research database containing anonymized healthcare claims of approximately 4 million covered lives. PKU patients were compared with matched controls from the general population within the same database (1:10 ratio via direct, exact matching on age and gender without replacement). PKU patients were included if they were aged ≥18 years on 01/01/15 and were continuously enrolled from 01/01/10 to 31/12/15. The 50 most commonly reported comorbidities and 50 most commonly prescribed medications in the PKU population were analyzed. Differences between groups were tested using 95% confidence interval (CI) of prevalence ratio (PR) values. Results The analysis included 377 adult PKU patients (< 5 of which were receiving sapropterin dihydrochloride) and 3,770 matched controls. Of the 50 most common comorbidities in the PKU population, those with a statistically significant PR > 1.5 vs controls included major depressive disorders (PR = 2.3), chronic ischemic heart disease (PR = 1.7), asthma (PR = 1.7), dizziness and giddiness (PR = 1.8), unspecified diabetes mellitus (PR = 1.7), infectious gastroenteritis and colitis (PR = 1.7), and reaction to severe stress and adjustment disorders (PR = 1.6). The most commonly prescribed Anatomical Therapeutic Chemical (ATC) subcodes among PKU patients (vs the control population) are for systemic antibacterials (34.7% vs 32.8%), anti-inflammatory and antirheumatic (29.4% vs 27.5%), renin-angiotensin agents (30.0% vs 27.0%), acid-related disorders (29.4% vs 20.2%), and beta-blockers (24.9% vs 19.9%). Conclusion The overall clinical burden on patients with PKU is exacerbated by a significantly higher risk of numerous comorbidities and hence, prescribing of the requisite medication, both for recognized (e.g. major depressive disorders) and more unexpected comorbidities (e.g. ischemic heart disease). Electronic supplementary material The online version of this article (10.1186/s13023-019-1153-y) contains supplementary material, which is available to authorized users.

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“…A restrição proteica favorece e estimula o consumo de alimentos ricos em carboidratos e em lipídios, e aumenta o risco de sobrepeso e obesidade (KANUFRE et al, 2015). Há, portanto a necessidade de fazer um trabalho de prevenção e conscientização a respeito ao excesso de peso e alteração metabólica, relacionados aos hábitos alimentares.…”

Section: Resultsunclassified

Fenilcetonúria: Aspectos Gerais, de Saúde Pública e Situação no Estado de Goiás

Goulart

Silva

Santos

et al. 2017

Ens Cien C Biol Agra Saúde

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ResumoO objetivo deste trabalho foi estudar a fenilcetonúria e realizar um diagnóstico sobre a situação atual dos fenilcetonúricos no Estado de Goiás, a fim de se conhecer melhor os pacientes, bem como divulgar a doença, a importância do tratamento e a necessidade de investimentos em nível nacional. Os dados dos fenilcetonúricos foram obtidos por meio de seus prontuários organizados pelo Ambulatório Multidisciplinar Especializado da Associação de Pais e Amigos dos Excepcionais de Anápolis. Os dados agrupados em quadros, sem as identidades dos pacientes, foram organizados e discutidos nesse trabalho. Segundo o levantamento realizado neste trabalho, atualmente, o Estado de Goiás possui 76 pessoas com fenilcetonúria em tratamento sendo que destes 39,5% são do sexo masculino e 60,5% feminino. A maioria dos pacientes são menores de 18 anos (77,63%) e possuem renda familiar entre 1 a 3 salários mínimos (55%). Os fenilcetonúricos residem em trinta e duas diferentes cidades no Estado de Goiás. Este trabalho servirá de base para maior divulgação e conscientização da fenilcetonúria e da necessidade de investimentos em pesquisas na área da saúde e processos tecnológicos, a fim de desenvolver alimentos de baixo custo destinados a pessoas com a doença, uma vez que a dieta é cara e a maioria dos pacientes possui baixa renda.Palavras-chave: Fenilcetonúria. Diagnóstico. Goiás. Abstract The objective of this research was to study phenylketonuria and to performa diagnosis of the current situation of phenylketonuria in the state of Goiás, in order to better understand patients and divulgate the disease, the importance of treatment and the need for investments nationwide. The information onphenylketonuria was obtained through medical records organized by the Specialized Multidisciplinary Clinic Association of Parents and Friends of Exceptional Children from Anápolis. The information grouped in tables without

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Metabolic syndrome in children and adolescents with phenylketonuria

Cited by 22 publications

References 26 publications

Overweight and Associated Factors in Children and Adolescents With Phenylketonuria: A Systematic Review

Overweight and Associated Factors in Children and Adolescents With Phenylketonuria: A Systematic Review

Clinical burden of illness in patients with phenylketonuria (PKU) and associated comorbidities - a retrospective study of German health insurance claims data

Fenilcetonúria: Aspectos Gerais, de Saúde Pública e Situação no Estado de Goiás

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