Browsing by Author "Mansilha, H."
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- Alimentação em situações especiais - dislipidémiaPublication . Mansilha, H.Os pediatras têm vindo a tornar-se cada vez mais conscientes da necessidade de prevenir, rastrear, diagnosticar e tratar a dislipidemia na infância e adolescência. De facto, evidência científica significativa sustenta o conceito de que antecedentes determinantes de doença cardíaca no adulto podem ser identificados em idade pediátrica, e estudos prospectivos têm relacionado a presença de factores de risco cardiovascular nesta idade com o desenvolvimento de lesões ateroscleróticas prematuras. A dislipidemia raramente tem consequências adversas para a saúde na infância, mas os seus efeitos a longo prazo, tais como eventos clínicos cardiovasculares que se encontram entre as principais causas de morbilidade e mortalidades dos países industrializados, realçam o interesse do rastreio de anomalias lipídicas nas crianças. O objectivo desta revisão será analisar a recente evidência científica da associação das anomalias lipídicas com a arteriosclerose precoce, do impacto da obesidade de contornos epidémicos e outras patologias nos lípidos na infância, relações essas que poderão colocar as crianças e os adolescentes em risco acrescido para aterosclerose acelerada. De facto, o processo patofisiológico subjacente às lesões ateroscleróticas é complexo e resulta da interacção entre as lipoproteínas circulantes, mediadores inflamatórios, e uma variedade de células, que conduz à acumulação de partículas de lipoproteínas de colesterol de baixa densidade sob a camada endotelial arterial. É também revisto os aspectos clinicamente relevantes das vias metabólicas das lipoproteínas, no sentido de reconhecer e classificar os tipos mais importantes de dislipidemia, nomeadamente a hipercolesterolemia familiar. Embora a principal prevenção na infância do desenvolvimento dos factores de risco da doença cardiovascular prematura permaneça assente nas recomendações sobre o estilo de vida, um subgrupo de crianças pode necessitar de terapêutica para baixar os lípidos, antes da vida adulta. Assim, são apresentadas recomendações de orientação da decisão relativa à selecção de doentes de alto risco para aterosclerose acelerada, que possam necessitar de abordagem farmacológica das suas alterações lipídicas. Portanto, esta revisão sumariza a evidência actual sobre o impacto directo da dislipidemia no desenvolvimento do processo aterosclerótico facultando estratégias potenciais de prevenção e tratamento durante a infância e a adolescência. ABSTRACT Pediatricians have long been aware that dyslipidemia needs to be prevented, screened, diagnosed and treated in childhood and adolescence. In fact, a significant body of evidence supports the concept that the antecedents of adult heart disease can be identifi ed in childhood and the prospective studies have linked the presence of cardiovascular risk factors to early atherosclerotic lesions, in pediatric population. Dyslipidemia rarely leads to adverse health outcomes in childhood, but its long-term effects such as clinical cardiovascular events that are among the leading causes of morbidity and mortality in industrialized nations, raises interest in screening children for lipid abnormalities. The goal of this review is to examine new evidence on the association of lipid abnormalities with early atherosclerosis, the impact of the obesity epidemic and other medical conditions on lipids in childhood,that places children and adolescents at higher risk for accelerated atherosclerosis. Indeed, the pathophysiologic process behind atherosclerotic lesions is complex and results from the interplay between circulating lipoproteins, infl ammatory mediators, and a various cell types leading to accumulation of lowdensity lipoprotein cholesterol particles beneath the arterial endothelial layer. It is also reviewed the clinically relevant aspects of lipoprotein metabolism pathways in order to recognize and classify the most important types of dyslipidemia, namely familial hypercholesterolemia. Although the prevention of risk factors development of premature cardiovascular disease through lifestyle recommendations remains the mainstay of pediatric management, a subgroup of children may require lipid-lowering drug therapy before reaching adulthood. Thus, recommendations are provided to guide decision-making with regard to patient screening at high risk for accelerated atherosclerosis who might require pharmacological management of lipid abnormalities. Therefore, this review summarizes the current evidence for the direct impact of dyslipidemia on atherosclerotic process development and provides potencial prevention and treatment strategies during childhood and adolescence.
- Alimentação, exercício físicio e doença cardiovascular em adolescentes. Conclusões.Publication . Mansilha, H.; Santos, M.Eventos clínicos cardiovasculares decorrentes de Doença Cardiovascular (DCV) estão entre as principais causas de morbilidade e mortalidade nos países industrializados As origens patofisiológicas das lesões responsá- veis, as lesões ateroscleróticas, podem datar de décadas previamente aos eventos clínicos, ou seja, durante a infância e adolescência. A maioria destes eventos clínicos cardiovasculares são determinados por factores de risco sobejamente conhecidos e que incluem necessariamente desequilíbrios dietéticos e sedentarismo. Estes factores de risco podem até já não estar presentes na vida adulta, mas condicionarão sempre a saúde cardiovascular, pelo que é de primordial importância zelar pela saúde do indivíduo desde a sua infância e adolescência A . Estratégias de adopção de estilos de vida saudáveis, focados em alimentação saudável e exercício, têm vindo a demonstrar eficácia na prevenção das DCV B . Neste sentido, o Grupo de Estudos de Risco Cardiovascular em conjunto com o Núcleo de Nutrição em Cardiologia da Sociedade Portuguesa de Cardiologia promoveram, no âmbito da Segunda Reunião Conjunta, um workshop subordinado ao tema “Alimentação, actividade física e DCV em adolescentes”. Desta reunião de trabalho foram retiradas algumas conclusões que serão relatadas de forma objectiva, para que possam servir de recomendações práticas para todos os profissionais de saúde que intervém com adolescentes e suas famílias
- Bilirubin is independently associated with oxidized LDL levels in young obese patientsPublication . Nascimento, H.; Alves, A.; Coimbra, S.; Catarino, C.; Gomes, D.; Bronze-da-Rocha, E.; Costa, E.; Rocha-Pereira, P.; Aires, L.; Mota, J.; Mansilha, H.; Rêgo, C.; Santos-Silva, A.; Belo, L.BACKGROUND: Bilirubin can prevent lipid oxidation in vitro, but the association in vivo with oxidized low-density lipoprotein (Ox-LDL) levels has been poorly explored. Our aim is to the association of Ox-LDL with total bilirubin (TB) levels and with variables related with metabolic syndrome and inflammation, in young obese individuals. FINDINGS: 125 obese patients (13.4 years; 53.6% females) were studied. TB, lipid profile including Ox-LDL, markers of glucose metabolism, and levels of C-reactive protein (CRP) and adiponectin were determined. Anthropometric data was also collected. In all patients, Ox-LDL correlated positively with BMI, total cholesterol, LDLc, triglycerides (TG), CRP, glucose, insulin and HOMAIR; while inversely with TB and HDLc/Total cholesterol ratio (P < 0.05 for all). In multiple linear regression analysis, LDLc, TG, HDLc and TB levels were significantly associated with Ox-LDL (standardized Beta: 0.656, 0.293, -0.283, -0.164, respectively; P < 0.01 for all). After removing TG and HDLc from the analysis, HOMAIR was included in the regression model. In this new model, LDLc remained the best predictor of Ox-LDL levels (β = 0.665, P < 0.001), followed by TB (β = -0.202, P = 0.002) and HOMAIR (β = 0.163, P = 0.010). CONCLUSIONS: Lower bilirubin levels may contribute to increased LDL oxidation in obese children and adolescents, predisposing to increased cardiovascular risk.
- Body fat percentage is a major determinant of total bilirubin independently of UGT1A1*28 polymorphism in young obesePublication . Belo, L.; Nascimento, H.; Kohlova, M.; Bronze-da-Rocha, E.; Fernandes, J.; Costa, E.; Catarino, C.; Aires, L.; Mansilha, H.; Rocha-Pereira, P.; Quintanilha, A.; Rêgo, C.; Santos-Silva, A.OBJECTIVES: Bilirubin has potential antioxidant and anti-inflammatory properties. The UGT1A1*28 polymorphism (TA repeats in the promoter region) is a major determinant of bilirubin levels and recent evidence suggests that raised adiposity may also be a contributing factor. We aimed to study the interaction between UGT1A1 polymorphism, hematological and anthropometric variables with total bilirubin levels in young individuals. METHODS: 350 obese (mean age of 11.6 years; 52% females) and 79 controls (mean age of 10.5 years; 59% females) were included. Total bilirubin and C-reactive protein (CRP) plasma levels, hemogram, anthropometric data and UGT1A1 polymorphism were determined. In a subgroup of 74 obese and 40 controls body composition was analyzed by dual-energy X-ray absorptiometry. RESULTS: The UGT1A1 genotype frequencies were 49.9%, 42.7% and 7.5% for 6/6, 6/7 and 7/7 genotypes, respectively. Patients with 7/7 genotype presented the highest total bilirubin levels, followed by 6/7 and 6/6 genotypes. Compared to controls, obese patients presented higher erythrocyte count, hematocrit, hemoglobin and CRP levels, but no differences in bilirubin or in UGT1A1 genotype distribution. Body fat percentage was inversely correlated with bilirubin in obese patients but not in controls. This inverse association was observed either in 6/7 or 6/6 genotype obese patients. UGT1A1 polymorphism and body fat percentage were the main factors affecting bilirubin levels within obese patients (linear regression analysis). CONCLUSION: In obese children and adolescents, body fat composition and UGT1A1 polymorphism are independent determinants of total bilirubin levels. Obese individuals with 6/6 UGT1A1 genotype and higher body fat mass may benefit from a closer clinical follow-up.
- A case of Protracted Diarrhea in a Newborn: a Diagnostic ChallengePublication . Mendes, C.; Figueiredo, C.; Mansilha, H.; Proença, E.; Oliveira, D.; Lima, R.; Carvalho, C.Congenital diarrhea comprises a broad range of pathologies and often requires a thorough workup and immediate treatment. Although rare, microvillous inclusion disease (MVID) should be included in differential diagnosis of this presentation in the neonate. We report the case of a 36-week newborn who developed signs of severe dehydration and lethargy, requiring fluid resuscitation and total parenteral nutrition. MVID was diagnosed by recognition of profuse secretory diarrhea after an exhaustive etiological investigation, confirmed by DNA analysis.
- Tumefacção Parotídea Bilateral com Crepitações SubcutâneasPublication . Guerra, I.; Costa, E.; Mansilha, H.; Lima, R.ABSTRACT The presence of air within the parotid gland is called pneumoparotid. This is a rare cause of parotid swelling and is associated to many precipitant factors. A seven -year -old boy was seen at the Emergency Department due to a sudden bilateral parotid swelling. The physical examination revealed a crepitus in the parotid glands. Radiography and ultrasonography suggested the presence of air. This acute episode was self -limited with complete resolution within forty -eight hours. Clinical history revealed that the child had been fi lling up balloons before the symptoms began. An early diagnosis avoids recurrent attacks and is based on the identifi cation of air within the parotid gland associated with the existence of precipitant factors.