RESUMO
The causes of weight loss in Huntington's disease (HD) are not entirely clear. The aim was to identify risk factors that are associated with a loss of metabolically active tissues, i.e. fat-free mass. A consecutive cohort of non-diabetic HD participants (manifest HD, n = 43; CAG: mean 43.6.0 ± 3.6; preHD, n = 10; CAG: mean 41.4 ± 1.4) and 36 healthy controls was recruited. Twenty-five HD participants were early-stage HD (UHDRS Total Functional Capacity [TFC] stages I and II), 12 mid-stage HD (TFC stage III), and 6 participants were in late-stage HD (TFC stages IV and V). Food intake, basic metabolic rate and glucose homeostasis were assessed. In addition, fat-free mass was determined using bioelectric impedance analysis, and leptin, insulin and ghrelin as key metabolic regulators. Sex ratio and age were similar in HD participants (71 % women; age 50.6 ± 10.9) and controls (66 % women; age 46.4 ± 14.5). Body mass index (BMI) was lower in HD participants than controls (median 24.1 vs. 25.9, p = 0.04). However, fat-free mass and basic metabolic rate were not statistically different between groups and showed no association with disease burden. In controls and HD participants, leptin was the most important predictor of fat-free mass. While BMI was lower in HD participants, fat-free mass was similar to controls with leptin as its most important predictor. Leptin levels and fat-free mass measurements using bioelectric impedance analysis may be good screening tools to identify HD patients at risk for weight loss.
Assuntos
Índice de Massa Corporal , Gorduras/metabolismo , Doença de Huntington/diagnóstico , Doença de Huntington/metabolismo , Adulto , Composição Corporal , Contagem de Células , Suplementos Nutricionais , Impedância Elétrica , Feminino , Humanos , Proteína Huntingtina , Doença de Huntington/genética , Linfócitos/patologia , Masculino , Pessoa de Meia-Idade , Proteínas do Tecido Nervoso/genética , Avaliação Nutricional , Valor Preditivo dos Testes , Repetições de Trinucleotídeos/genéticaRESUMO
AIM OF STUDY: The study served to assure the quality of our catering, to locate problems, and to define further optimization measures at the Bern University Hospital. The main objective was to investigate whether the macronutrient and energy content of the hospital food complies with the nutritional value calculated from recipes as well as with the recommendations issued by the German Nutrition Society (DGE). MATERIAL AND METHODS: Prospective, randomized, single-center quality study. Complete standard meals were analyzed over seven consecutive days for each seasonal menu plan in one year. The quantitative and qualitative chemical content of a randomly chosen menu was determined by an external laboratory. RESULTS: Sixty meals were analyzed. The amount of food served and the ratio of macronutrients contained in the food satisfactorily reflected all recipes. Not surprisingly, the energy and carbohydrate content of our meals was lower than in the German recommendations, because the report of the DGE is based on the sum of meals, snacks and beverages consumed over the whole day and not only on the main meals, as we analyzed. CONCLUSIONS: Periodic quality control is essential in order to meet recommendations and patients' expectations in hospital catering. Members of the catering service should undergo regularly repeated skills training, and continuous efforts should be made to ensure portion size for all delivered meals. Food provision in the hospital setting needs to be tailored to meet the demands of the different patient groups, to optimize nutritional support, and to minimize food waste.
Assuntos
Serviço Hospitalar de Nutrição/normas , Necessidades Nutricionais , Carboidratos da Dieta/análise , Gorduras na Dieta , Proteínas Alimentares/análise , Ingestão de Energia , Análise de Alimentos , Humanos , Estudos Prospectivos , Controle de Qualidade , Cloreto de Sódio/análiseRESUMO
Diseases associated with cobalamin deficiency often present a variety of neurological disorders apart from the well known megaloblastic anaemia as haematological manifestation. The peripheral and the central nervous system can be affected in different levels by the metabolic changes due to an impaired Vitamin B12 metabolism. Based upon an observed case we discuss the manifestation of cerebral convulsion possibly due to a secondarily acquired cobalamin deficiency. We conclude that in de novo cerebral convulsion in the elderly a cobalamin deficiency could play an important role.