Pediatric palliative care for metabolic diseases: 20-year epidemiological survey of outpatients at a Brazilian quaternary hospital.

The interface between pediatric palliative care (PPC) and inborn metabolic diseases (IMD) remains incipient, though these conditions fill the state of art of complex chronic diseases, eligible to this health approach. We analyzed the medical records of PPC clinic during the years 2001 to 2021 and the IMD outpatients. We established a parallel with the world scientific literature concerning the epidemiology of PPC and IMD. Among outpatients, 14% were diagnosed with IMD, which were referred to the PPC service earlier compared to Non-IMD cases. The Group 3 (complex molecules) was the most frequent (64.7%), following by Group 1 representing by small molecules (21.6%), the latter having a lower median age at diagnosis when compared to the former (0.7 vs. 5.2 years, p = 0.001). The sphingolipidoses were the pathologies most frequent in our cohort, in line with what was observed in the literature. There were no differences between IMD groups in terms of diagnosis and PPC referral age, however in Non-IMD conditions, the age of diagnosis were earlier than IMD. Nevertheless, IMD group showed lower age of referral to PPC. The IMD comprises large fraction of outpatients in the PPC setting, thus further studies are needed in this field.

Body Composition and Phase Angle: How to Improve Nutritional Evaluation in Juvenile Dermatomyositis Patients.

(1) Background: This study aimed to assess body composition (BC) using bioelectrical impedance and food intake in juvenile dermatomyositis (JDM) patients. Associations between BC and physical activity, disease activity/cumulative damage and health-related quality of life parameters were also evaluated; (2) Methods: This was a cross-sectional study with 30 consecutive JDM patients (18 female and 12 male) and 24 healthy volunteers (14 female and 10 male) of both sexes followed at our pediatric rheumatology unit. The gathering of anthropometric and dietary data, and the performance of physical activity and bioelectrical impedance were undertaken in face-to-face meetings and through questionnaires. Clinical and therapeutic data were collected from medical records according to information from routine medical appointments; (3) Results: The frequency of high/very high body fat was significantly higher in controls compared with JDM patients (66.7% vs. 91.7%; p = 0.046). The median phase angle was significantly lower in patients compared with controls (5.2 ± 1.3 vs. 6.1 ± 1.0; p = 0.016). Body fat and lean mass were positively correlated with disease duration (rs = +0.629, p < 0.001 and rs = +0.716, p < 0.001, respectively) and phase angle (PhA) (rs = +0.400, p = 0.029 and rs = +0.619, p < 0.001, respectively). JDM patients with PhA ≥ 5.5 presented higher lean mass when compared with patients with PhA < 5.5 (p = 0.001); (4) Conclusions: Bioelectrical impedance can be a useful auxiliary exam in the medical and nutritional follow-up of JDM patients, because it seems to impact functional ability. These findings may assist professionals when advising JDM patients about the importance of physical activity and healthy eating in the preservation of lean mass.

Phytosterol supplementation in the treatment of dyslipidemia in children and adolescents: a systematic review.

OBJECTIVE: To carry out a systematic review on the effects of phytosterol supplementation on the treatment of dyslipidemia in children and adolescents. DATA SOURCES: Review in the SciELO, Lilacs, Bireme, PubMed and Web of Science databases, with no time limit. Descriptors: phytosterols or plant sterols and dyslipidemias, hypercholesterolemia, cholesterol, children, adolescent, in English and Portuguese. The articles included were published in Portuguese, English or Spanish and evaluated the effect of phytosterol supplementation in pediatric patients with dyslipidemia. Documents that involved adults or animals, review papers, case studies and abstracts were excluded. Two authors performed independent extraction of articles. Of 113 abstracts, 19 were read in full and 12 were used in this manuscript. DATA SYNTHESIS: Phytosterol supplementation to reduce cholesterol levels has been shown to be effective in reducing LDL-cholesterol levels by approximately 10%, with reductions above 10% in LDL-cholesterol levels observed after 8 to 12 weeks of intervention. Studies have not shown significant changes in HDL-cholesterol and triglyceride levels. Based on the absence of adverse effects, its use seems to be safe and of good tolerance in children and adolescents. CONCLUSIONS: Phytosterol supplementation seems to be of great therapeutic aid for the treatment of hypercholesterolemia in children and adolescents. Further studies assessing the long-term effect of phytosterol supplementation are necessary.

Phytosterol supplementation in the treatment of dyslipidemia in children and adolescents: a systematic review / Suplementação de fitoesteróis no tratamento da dislipidemia em crianças e adolescentes: uma revisão sistemática

ABSTRACT Objective: To carry out a systematic review on the effects of phytosterol supplementation on the treatment of dyslipidemia in children and adolescents. Data sources: Review in the SciELO, Lilacs, Bireme, PubMed and Web of Science databases, with no time limit. Descriptors: phytosterols or plant sterols and dyslipidemias, hypercholesterolemia, cholesterol, children, adolescent, in English and Portuguese. The articles included were published in Portuguese, English or Spanish and evaluated the effect of phytosterol supplementation in pediatric patients with dyslipidemia. Documents that involved adults or animals, review papers, case studies and abstracts were excluded. Two authors performed independent extraction of articles. Of 113 abstracts, 19 were read in full and 12 were used in this manuscript. Data synthesis: Phytosterol supplementation to reduce cholesterol levels has been shown to be effective in reducing LDL-cholesterol levels by approximately 10%, with reductions above 10% in LDL-cholesterol levels observed after 8 to 12 weeks of intervention. Studies have not shown significant changes in HDL-cholesterol and triglyceride levels. Based on the absence of adverse effects, its use seems to be safe and of good tolerance in children and adolescents. Conclusions: Phytosterol supplementation seems to be of great therapeutic aid for the treatment of hypercholesterolemia in children and adolescents. Further studies assessing the long-term effect of phytosterol supplementation are necessary.

RESUMO Objetivo: Realizar uma revisão sistemática sobre os efeitos da suplementação de fitoesteróis no tratamento da dislipidemia em crianças e adolescentes. Fontes de dados: Revisão nos bancos SciELO, Lilacs, Bireme, Pubmed e Web of Science, sem limite de tempo. Descritores: phytosterols or plant sterols, dyslipidemias, hypercholesterolemia, cholesterol, children, adolescent, nas línguas inglesa e portuguesa. Os artigos incluídos foram publicados nos idiomas português, inglês ou espanhol e avaliaram o efeito da suplementação de fitoesteróis em pacientes pediátricos com dislipidemia. Estudos que envolviam adultos ou animais, trabalhos de revisão, estudos de caso e resumos foram excluídos. A extração independente de artigos foi realizada por dois autores. Do total de 113 resumos, 19 foram lidos na íntegra, e 12 utilizados neste manuscrito. Síntese de dados: A suplementação de fitoesteróis para a redução dos níveis de colesterol mostrou-se eficiente, de forma a promover a redução de aproximadamente 10% dos níveis de LDL-colesterol, sendo observadas reduções acima de 10% em 8 a 12 semanas de intervenção. Os estudos não mostraram alterações significantes nos níveis de HDL-colesterol e triglicérides. Com base na ausência de efeitos adversos, seu uso parece ser seguro e de boa tolerância em crianças e adolescentes. Conclusões: A suplementação com fitoesteróis parece ser de grande auxílio terapêutico para o tratamento da hipercolesterolemia em crianças e adolescentes. São necessários mais estudos que avaliem o efeito em longo prazo da suplementação de fitoesteróis.

Alterações alimentares e hábitos de vida de pacientes com fibrose cística na pandemia de COVID-19 / Dietary changes and life habits of patients with cystic fibrosis in the COVID-19 pandemic / Cambios en la dieta y hábitos de vida de pacientes con fibrosis quística en la pandemia COVID-19

Objetivo: verificar alterações no consumo alimentar e hábitos de vida em pacientes com fibrose cística brasileiros durante o período de isolamento social devido a pandemia de COVID-19. Método: pesquisa transversal com levantamento de dados por meio de questionário elaborado pela equipe de nutricionistas especialistas no tratamento de pacientes com fibrose cística, com questões referentes às manifestações respiratórias recentes de pacientes ou familiares, alterações nos hábitos de aquisição e compras de alimentos e alterações quanto ao consumo de grupos alimentares e suplementos. Os dados foram tabulados e foi realizada uma análise descritiva. Resultados: 40,34% das famílias de pacientes com fibrose cística mudaram os hábitos de compras de alimentos, cerca de 40% dos pacientes diminuíram a prática de atividade física e aumentaram o tempo de uso de telas em mais de 50% durante o período da pandemia de COVID-19. Conclusão: Apesar de algumas dificuldades relacionadas à aquisição dos alimentos e alterações em hábitos de vida, houve manutenção do consumo alimentar da maioria dos grupos alimentares e suplementos pelos pacientes pediátricos com fibrose cística brasileiros durante o isolamento social devido a pandemia de COVID-19.

Objective: to verify changes in food consumption and lifestyle in Brazilian cystic fibrosis patients during the period of social isolation due to the COVID-19 pandemic. Method: cross-sectional survey with data collection through a questionnaire prepared by the team of nutritionists specialized in the treatment of patients with cystic fibrosis, with questions regarding the recent respiratory manifestations of patients or family members, changes in the habits of purchasing food and changes regarding the consumption of food groups and supplements. The data were tabulated and a descriptive analysis was performed. Results: 40.34% of the families of patients with cystic fibrosis changed their food shopping habits, about 40% of the patients decreased their physical activity and increased the time spent using screens by more than 50% during the pandemic COVID-19. Conclusion: despite some difficulties related to the acquisition of food and changes in lifestyle, there was maintenance of the food consumption of most food groups and supplements by Brazilian pediatric patients with cystic fibrosis during social isolation due to the COVID-19 pandemic.

Objetivo: verificar cambios en el consumo de alimentos y estilo de vida en pacientes brasileños con fibrosis quística durante el período de aislamiento social por la pandemia de COVID-19. Método: encuesta transversal con recolección de datos por medio de un cuestionario elaborado por un equipo de nutricionistas especializados en el tratamiento de pacientes con fibrosis quística, con preguntas sobre las manifestaciones respiratorias recientes de pacientes o familiares, cambios en los hábitos de compra de alimentos y cambios en el consumo de grupos de alimentos y suplementos. Los datos se tabularon y se realizó un análisis descriptivo. Resultados: el 40,34% de los familiares de pacientes con fibrosis quística cambiaron sus hábitos de compra de alimentos, alrededor del 40% de los pacientes disminuyó la práctica de actividad física y aumentó el tiempo de uso de pantallas en más del 50% durante el período pandémico. COVID-19. Conclusión: A pesar de algunas dificultades relacionadas con la adquisición de alimentos y cambios en el estilo de vida, hubo mantenimiento del consumo de alimentos de la mayoría de los grupos de alimentos y suplementos por parte de pacientes pediátricos brasileños con fibrosis quística durante el aislamiento social debido a la pandemia COVID-19.

Juvenile idiopathic arthritis activity and function ability: deleterious effects in periodontal disease?

The impact of juvenile idiopathic arthritis (JIA) in periodontal diseases is controversial probably due to gender and age heterogeneity. We therefore evaluated a homogeneous female post-pubertal JIA population for these conditions. Thirty-five JIA patients and 35 gender/age comparable healthy controls were evaluated according to demographic data, complete periodontal evaluation, fasting lipoproteins, and anti-lipoprotein lipase antibodies. JIA scores, laboratorial tests, X-rays, and treatment were also assessed. Current age was similar in JIA patients and controls (11.90 ± 2.0 vs. 12.50 ± 3.0 years, p = 0.289). Complete periodontal assessments revealed that gingival index, dental plaque, gingival bleeding, and clinical dental attachment indices were alike in JIA patients and controls (p > 0.05), except for gingival enlargement in former group (p < 0.0001). Further analysis of patients with and without gingivitis revealed that cyclosporine use was more often observed in JIA patients with gingivitis (37 vs. 0%, p = 0.01), whereas no differences were evidenced in demographic, JIA scores, inflammatory markers, and lipid profile in both groups. Of note, two parameters of periodontal assessment were correlated with JIA scores [gingival index (GI) and Childhood Health Assessment Questionnaire (CHAQ) (r s = +0.402, p = 0.020)] and plaque index (PI) and visual analog scale (VAS) physician (r s = +0.430, p = 0.013). In addition, evaluation of dental assessment demonstrated that JIA activity scores had positive correlation with decayed, missing, and filled teeth (DMF-T) and junvenile athritis disease activity score (JADAS) (r s = +0.364,p = 0.037), VAS physician (r s = +0.401,p = 0.021) and VAS patient (r s = +0.364,p = 0.037). We demonstrated, using rigorous criteria, that periodontal and dental condition in JIA is similar to controls. In spite of that, the finding of a correlation with disease parameters provides additional evidence that increased activity and reduced functional ability underlies the deleterious effect of JIA in oral health.

Assessment of antioxidants status and superoxide dismutase activity in HIV-infected children.

OBJECTIVE: This study aims to assess the nutritional status of selenium, copper and zinc; and also the erythrocyte superoxide dismutase activity of HIV-infected children compared to a control group. METHODS: A cross-sectional study was carried out with prepubertal HIV-infected children (n=51) and their healthy siblings (n=32). All biochemical measurements including plasma selenium, serum copper levels, serum and erythrocyte zinc levels and erythrocyte superoxide dismutase activity were evaluated according to dietary, clinical and biochemical parameters. RESULTS: Compared to the control group, the HIV-infected children had lower z-score values for height-for-age (p=0.0006), higher prevalence of stunting (11.8%) (p=0.047), lower selenium levels (p=0.0006) and higher copper levels (p=0.019). No difference was found concerning superoxide dismutase activity (p>0.05). The HIV-infected group presented a higher proportion (45.1%) of children with zinc intakes below the estimated average requirement (p=0.014); however, no association with zinc biochemical parameters was found. CONCLUSION: HIV-infected children have an inadequate selenium and copper nutritional status, which could influence the progression to AIDS. An adequate micronutrient status could improve the clinical conditions in these patients and minimize free radical production and cellular oxidative stress.

Assessment of antioxidants status and superoxide dismutase activity in HIV-infected children

Objective: This study aims to assess the nutritional status of selenium, copper and zinc; and also the erythrocyte superoxide dismutase activity of HIV-infected children compared to a control group. Methods: A cross-sectional study was carried out with prepubertal HIV-infected children (n = 51) and their healthy siblings (n = 32). All biochemical measurements including plasma selenium, serum copper levels, serum and erythrocyte zinc levels and erythrocyte super-oxide dismutase activity were evaluated according to dietary, clinical and biochemical parameters. Results: Compared to the control group, the HIV-infected children had lower z-score values for height-for-age (p = 0.0006), higher prevalence of stunting (11.8%) (p = 0.047), lower selenium levels (p = 0.0006) and higher copper levels (p = 0.019). No difference was found concerning superoxide dismutase activity (p > 0.05). The HIV-infected group presented a higher proportion (45.1%) of children with zinc intakes below the estimated average requirement (p = 0.014); however, no association with zinc biochemical parameters was found. Conclusion: HIV-infected children have an inadequate selenium and copper nutritional status, which could influence the progression to AIDS. An adequate micronutrient status could improve the clinical conditions in these patients and minimize free radical production and cellular oxidative stress. .

Vitaminas antioxidantes e prevenção da arteriosclerose na infância / Antioxidant vitamins and prevention of atherosclerosis in childhood

OBJETIVO: Revisar os potenciais efeitos antioxidantes das vitaminas A, C e E na prevenção do desenvolvimento da arteriosclerose na infância, com ênfase na prevenção da dislipidemia. FONTES DE DADOS: Pesquisa bibliográfica em revistas científicas, livros técnicos e publicações de órgãos oficiais dos últimos 20 anos. Utilizaram-se as bases de dados Lilacs, SciELo e Medline em português, inglês e espanhol, com as palavras-chave: "antioxidantes", "arteriosclerose", "dislipidemias", "peroxidação de lipídeos", "infância", "vitamina A", "vitamina C" e "vitamina E". SÍNTESE DE DADOS: A prevalência de dislipidemia na infância e na adolescência mostra frequência crescente, provavelmente relacionada às mudanças dos hábitos alimentares e à redução na prática de atividades físicas. O elevado nível plasmático da lipoproteína de baixa densidade (LDL-c) é fator de risco para o desenvolvimento da arteriosclerose. O consumo de frutas, verduras e legumes, ricos em antioxidantes, é um dos fatores de maior importância na prevenção da peroxidação lipídica. A baixa ingestão dessas fontes naturais de antioxidantes sugere a necessidade de intervenção nutricional para atingir as metas diárias de consumo de vitaminas A, C e E, não sendo preconizada a sua suplementação medicamentosa. CONCLUSÕES: O pediatra e o nutricionista devem orientar as famílias sobre o consumo de alimentos saudáveis, principalmente frutas, verduras e legumes, por seu potencial efeito antioxidante, especialmente nos primeiros anos de vida.

OBJECTIVE: To review the potential antioxidant effects of vitamins A, C and E in the prevention of atherosclerosis development during childhood, emphasizing the prevention of dyslipidemia. DATA SOURCES: Bibliographic search in scientific journals, technical books and official publications of the last 20 years. Lilacs, SciElo and Medline databases were searched for articles in Portuguese, Spanish and English using a combination of the following terms: "antioxidants", "atherosclerosis", "dyslipidemia", "lipid peroxidation", "childhood", "vitamin A", "vitamin C" and "vitamin E". DATA SYNTHESIS: There is an increasing prevalence of dyslipidemia in children and adolescents, probably related to changes in dietary habits and to the reduced practice of physical activities. The high plasma concentration of low-density lipoprotein (LDL-c) is a risk factor for atherosclerosis development. The consumption of nutrients rich in antioxidants, such as fruits and vegetables, is very important to prevent lipid peroxidation. The low intake of these natural antioxidants sources suggests the need for nutritional intervention to achieve the daily ingestion targets of vitamins A, C and E. Vitamin supplementation is not recommended. CONCLUSIONS: Children should be encouraged to increase ingestion of fruits and vegetables due to their potential antioxidant effect, especially in the first years of life.