Obesity is the most prevalent nutritional disorder in cats (Felis catus; Hoenig et al., 2011). High carbohydrate diets, prescribed for weight loss, may contribute to adiposity (Thiess et al., 2004). The effects of a high fat (HF; 30% fat, 10% carbohydrate), high carbohydrate (HC; 10% fat, 46% carbohydrate) and a moderate diet (15% fat, 30% carbohydrate) supplemented with a calorie restriction mimetic (mannoheptulose (MH); 8 mg/kg BW), fed to energy requirements, on feline metabolism and behaviour were investigated (n=20; 4 ± 2.5 kg). An 11 week acclimation procedure was designed to adapt cats to 24-hr restriction within a chamber used for indirect calorimetry. Stress indicative behaviour (Kessler and Turner, 1997) declined with repreated exposure to increasing lengths of restriction within chambers and on week 11 stress levels were low and consistent (P<0.05). Neither the HF nor HC diet impacted body weight (p>0.05); however, HF feeding caused an increase in body fat (0.75 kg (baseline) vs. 1 kg (86d)) after long-term feeding. Energy expenditure (EE) was not impacted by dietary fat/carbohydrate. Respiratory quotients (RQ) increased and decreased with exposure to the HC (fasted= 0.80 ± 0.008; fed= 0.87 ± 0.008), HF (fasted= 0.76 ± 0.008; fed= 0.78 ± 0.008) diet, respectively. Glucose to insulin (G:I) ratio increased with HF feeding; indicating improved insulin sensitivity. Physical activity, measured using accelerometers, declined with HF (-1.6 counts/hr) and HC (-2.8 counts/hr) feeding from baseline. T-maze performance decreased and increased with HF (-0.85 score/10) and HC (0.85 score/10) feeding from baseline (p<0.05). MH did not impact body weight or composition (p>0.05). Area under the curve for EE increased during the 15-22 hour post feeding with MH treatment (2370.3 (-MH) vs. 3292.0 (+MH) ± 0.0002). RQ and G:I were not impacted by MH (p>0.05). MH increased play motivation, measured using obstruction tests (p<0.05). Diets high in carbohydrate are not ideal for weight loss since they negatively impact insulin sensitivity and voluntary EE. Diets promoting elevated EE, activity and normal glucose/insulin profiles are ideal for weight control and MH offers a unique opportunity for use in weight loss regimes. / This work was funded by Procter and Gamble Co.

There are significant differences in the metabolism of nutritional substrates in polytrauma patients. The actual metabolic needs of the patient may not be achieved despite the guidelines and recommendations for energy supply in the parenteral and enteral form of nutrition are followed. Subsequently this may lead to the occurrence of complications. The aim of the study was to compare the energy intake of each nutrient substrate supplied with nutrition by finding the nutritional substrate utilisation value which is obtained from indirect calorimetry measurements in patients with multiple injuries at the surgical ICU 1 of the Hradec Králové Teaching Hospital. By comparing this data you can obtain the optimum value for the nutritional needs of the patients. In the study we used seven spontaneously breathing polytrauma patients, three women and four men, of an average age of 41±21 years. Utilisations of nutritive substrates of each patient were obtained by indirect calorimetry. Accurate information on the amount and composition of nutrition supplied over 24 hours was also obtained. The patients received an average of 3.80±1.40 g/kg/day of carbohydrates, 1.03±0.56 g/kg/day of lipids and 1.40 ± 0.44 g/kg /day of proteins. The total energy intake was on average 30.94±11.03 kcal/kg/day. In six of seven...

Purpose. To investigate the validity of the Sense Wear Pro Armband (SWA) to measure energy expenditure (EE) in healthy participants using wheelchair ergometry as an exercise modality. Method. Minute by minute EE was measured simultaneously using the SWA and indirect calorimetry(IC) during three different wheeling speeds including self-selected speed (0.81 m/s), moderate speed (1.11 m/s), and fast speed (1.73 m/s). Results. Twenty healthy volunteers (age = 34.0 (5.8) years)participated. The intraclass correlation coefficients (ICCs) were 0.50 (p=0.010), 0.59 (p=0.003), and 0.68 (p=0.000) for the self-selected speed, moderate speed, and fast speed wheeling, respectively. The SWA overestimated EE 57.8%, 57.4 %, and 63.7% for self-selected speed, moderate speed, and fast speed, respectively. Conclusions. The SWA failed to provide an accurate estimate of EE as measured by indirect calorimetry for wheelchair ergometry exercise in healthy subjects. The SWA overestimated EE for all exercise intensities. / REHABILITATION SCIENCE-PHYSICAL THERAPY

No description available.

Growth hormone signaling influences longevity but the mechanism through which decreased GH action extends lifespan in mice is unknown. It is likely that the key to understanding this phenomenon, and the process of aging itself, is to understand the alterations in metabolism caused by decreased GH action. We investigated changes in energy metabolism in long-lived mice, in hope that these findings can suggest means of improving human health and longevity. These studies consisted of three projects. The influence of altered GH signaling on metabolism was tested by monitoring oxygen consumption, respiratory quotient, and heat production. Intriguingly, long-lived mice have increased oxygen consumption, and decreased respiratory quotient; while short lived mice had opposite effects. These data indicate that decreased GH signaling associates with increased metabolism per unit of body weight and may beneficially affect mitochondrial flexibility by increasing the capacity for fat oxidation; while GH excess generally produces opposite metabolic effects. We then hypothesized that the metabolic characteristics observed in young long-lived mice would persist into old age. Further, we investigated whether caloric restriction or every-other-day diet, two life extending feeding regimens, had any interaction with the metabolic phenotype observed in long-lived mice. The results support our hypothesis that the alterations in metabolism observed in young long-lived mice persist into old age. Neither dietary regimen significantly altered oxygen consumption in GHRKO mice, however, every-other-day diet reduced 24-hour oxygen consumption per gram body weight. These experiments showed that GHRKO mice had increased oxygen consumption regardless of age and life extending dietary interventions we placed them on. We hypothesized that increased oxygen consumption in long-lived mice is the result of increased thermogenesis. To test this hypothesis, we measured oxygen consumption in long-lived mice and controls at the standard lab temperature 23°C, and at 30°C, the murine thermoneutral temperature. When the oxygen consumption of long-lived mice was measured at 30°C, the differences between long-lived and normal mice measured at 23°C were abrogated. These data indicate that increased energy utilization for thermogenesis may contribute to extended longevity of these mutants. Collectively, our results provide important insights into the metabolic characteristics of long-lived mice.

Running performance has increased immensely during the last few years, coinciding with multiple shattered world records in relatively short amount of time. Improvements in footwear material and design are likely reasons for this increase in running performance. Previous studies on the effect of footwear on running economy (RE), a determinant of running performance, have not included participant-blinding. Furthermore, they have yet to compare multiple carbon-fiber plated running shoes available for purchase, what differences there are across price ranges and shoe categories, and if there is such a thing as a placebo-effect. Aim: (1) Descriptively compare a set of heterogeneous running shoes, with regards to running economy, Foot Strike Type (FST), vertical oscillation, ground contact time, stride length and cadence; including (2) a ‘sham’ and ‘normal condition’ of the same running shoe model; and (3) explore the participant’s perception of the study-specific blinding protocol. Method: A Single Subject Experimental Study (N=1), comparing nine different shoe conditions using a crossover design. The assessment of RE was conducted using indirect calorimetry with mixing-chamber in a climate-controlled facility. Spatiotemporal parameters were assessed using a Garmin HRM-Run™, and foot strike type was visually assessed using a frame-by-frame approach based on 2D-video at 240 fps. Results: The average running economy across all shoe tests varied between 16.02 to 17.02 W/kg, with the ‘worst’ shoe costing 6.24% W/kg more than the ‘best’ shoe. The descriptive difference between the ‘sham’ and ‘normal condition’ were negligible and within the range of measurement error. Spatiotemporal parameters were overall descriptively similar between the shoes, with a few outliers who differed with regards to measure of central tendency or dispersion. FST differed between the shoes including the ‘sham’ and ‘normal condition’, but were overall consistent with the participant’s habitual FST. The study-specific blinding procedure was perceived to work well, but may also be improved in some remarks. Conclusion: Descriptive difference in some, but not all, physiological and biomechanical parameters were observed between the shoe conditions in this study, including the ‘sham’ and ‘normal condition’. Blinding procedures in experimental footwear research may be feasible and adopted with future studies.

Vários estudos analisando a taxa de metabolismo de repouso (TMR) contribuíram com equações cuja proposta era estabelecer padrões que pudessem ser genericamente utilizadas para se estimar a TMR. A equação de Harris-Benedict (HB), permanece como o método mais comumente utilizado para estimar a TMR. Porém, em indivíduos obesos o uso de equações preditivas da TMR pode levar a resultados conflitantes. Indivíduos obesos submetidos a dietas hipocalóricas podem apresentar uma diminuição da TMR e do gasto energético total. Isto pode ser a causa da redução na velocidade da perda de peso durante o tratamento. Outros estudos mostram que a TMR, quando corrigida para a massa livre de gordura (MLG), apresenta pouca variabilidade e propõem uma correlação entre MLG e TMR. Porém ainda existem dificuldades em se afirmar ou não se a redução de massa corporal também reduz a TMR. O presente estudo teve como objetivos determinar a TMR de indivíduos com obesidade grau III (IMC > 40 kg/m2) do sexo feminino obtida por meio de calorimetria indireta (CI) e comparar com a TMR estimada por meio da equação de HB utilizando-se peso atual e peso ideal. Relacionar a TMR medida por CI com a composição corporal e validar a relação entre a TMR e a MLG nestes indivíduos antes e após a perda de peso. As pacientes foram internadas na Unidade Metabólica da Divisão de Nutrologia do Departamento de Clínica Médica do HCFMRP-USP, durante um período de 8 semanas. No início e no final do estudo foram realizadas: avaliação nutricional incluindo antropometria, bioimpedância e calorimetria indireta. As pacientes foram submetidas a uma dieta hipocalórica durante a internação. A TMR medida por calorimetria indireta (CI) no início e final do estudo foi de 2540 ± 417 e 1924 ± 275 kcal/dia, respectivamente (p<0,05). Quando calculado pela equação de HB utilizando-se peso atual, os valores encontrados foram 2074 ± 214 e 1941 ± 190 kcal/dia (p<0,05). Utilizando-se o peso ideal a TMR calculada foi de 1343 ± 59 kcal/dia. A TMR medida por CI foi, em média, 18 % maior que a calculada por HB pelo peso atual e 47 % maior que a calculada por HB utilizando-se o peso ideal no início do estudo. No final do estudo estes valores passaram para 1% e 30% respectivamente. Comparando-se a TMR medida por CI e calculada por HB usando peso atual tem-se, no início do estudo uma diferença significante (p<0,05) que não se repete no final do estudo (p>0,05). A TMR, quando corrigida para massa livre de gordura no início e no final do estudo foi de 46 ± 6 e 35 ± 5 kcal/d/MLG (p<0,05) respectivamente e quando corrigida para a gordura corporal (GC) foi de 33 ± 6 e 30 ± 5 kcal/d/GC (p<0,05) respectivamente. Os dados encontrados no presente estudo não permitem afirmar que o uso da equação de HB possa estimar a TMR de maneira confiável em indivíduos obesos grau III do sexo feminino. Porém os dados sugerem que logo após submeter esses indivíduos à dieta hipocalórica, com conseqüente perda de peso a equação de HB se torna confiável para estimar a TMR. Pacientes obesos ingerindo dieta livre deveriam ter a TMR obtida por meio da equação de HB corrigida por um fator de 20% a mais. / Studies analyzing resting energy expenditure (REE) have contributed with equations that were intended to establish a pattern that could be used generally to estimate the REE. Harris Benedict’s (HB) equation remains as the most used in estimating the REE. But in obese subjects, the use of predictive equations can lead to conflicting results. Obese individuals undergoing a hypo caloric diet may have a reduction in the REE and in the total energy expenditure. These are the most probably cause of the slowing on weight loss during the treatment. Some studies show that when the REE is relative to the fat free mass (FFM) there is less variability and their proposal is a correlation between FFM and REE. But it is difficult to confirm if a reduction in total body mass also can diminish the REE. The objective of this study was to define the REE of female subjects with grade III obesity (body mass index (BMI) > 40 kg/m2) using indirect calorimetry (IC) and to compare this REE with the one estimated with HB equation using real body weight and ideal body weight, and try to correlate the REE obtained by IC with the body composition, validating the relation between REE and FFM in these individual before and after weight loss. The patients were under hospital regimen in the Metabolic Unit of the Nutrology Division of the Internal Medicine Department of the HCFMRP-USP, for an 8 weeks period. At the beginning and at the end of the study, nutritional assessment was realized, including anthropometry, bioimpedance and indirect calorimetry. The patients were undergoing a hypo caloric diet during the 8 week period. The REE obtained by indirect calorimetry (IC) at the beginning and at the end of the study was 2540 ± 417 e 1924 ± 275 kcal/day, respectively (p<0,05). When estimated with the HB equation using real weight the values were 2074 ± 214 e 1941 ± 190 kcal/day (p<0,05). Using the ideal weight, the calculated REE was 1343 ± 59 kcal/day. At the beginning of the study, REE obtained by IC was 18 % greater than the REE calculated with HB equation using the real weight and 47 % greater than the one calculated using the ideal body weight. At the end of the study these values changed to 1% e 30% respectively. There is a significant difference (p<0,05) when comparing the REE obtained by IC with the one calculated using the HB equation with real weight at the beginning of the study, but this does not happen at the end of the study (p>0,05). The REE corrected by the FFM at the beginning and at the end of the study was 46 ± 6 e 35 ± 5 kcal/d/FFM (p<0,05) respectively and the REE corrected by the fat mass (FM) was 3 ± 6 e 30 ± 5 kcal/d/FM (p<0,05) respectively. The data found in this study does not allow affirming that the use of HB equation to predict REE in female grade III obese subjects is reliable. But the data suggest that immediately after using a hypo caloric diet, the REE calculated using HB equation and real weight is reliable. When calculating the REE of female grade III obese patients undergoing a free diet using HB equation, the obtained value should be increased in 20 %.

Objectives: Negative energy balance can impair regeneration of the respiratory epithelium and limit the functionality of respiratory muscles, which can prolong mechanical ventilation. The present study sought to quantify and identify deviation in energy requirements of patients with septic shock during and upon liberation from mechanical ventilation. Methods: Patients admitted into intensive care with initial diagnosis of septic shock and mechanical ventilation-dependent were recruited. Their metabolic requirements before and after liberation from mechanical ventilation were measured by indirect calorimetry. Paired t-test was used to examine the variance between the two modes of breathing and Spearman rho correlation coefficient to examine relationship of selected indicators. Results: Thirty-five patients, 20 males and 15 females mean age 69 ±10 years, body height of 1.58 ±0.08 meters, and ideal body weight 59.01 ±7.63 kg were recruited. Median APACHEII score was 22, length of stay in the intensive care was 45 ±65 days and duration on mechanical ventilation was 24 ±25 days. Measured energy expenditure during ventilation was 2090 ±489 kcal∙d-1 upon liberation from ventilation was 1910 ±579 kcal∙d-1, and actual caloric intake was 1148 ±495 kcal∙d-1. Paired-t test showed that measured energy expenditure (p=0.02), actual calories provision and energy expenditure with (p=0.00) and without (p=0.00) ventilator support were all significantly different. Mean carbohydrate oxidation was 0.17 ±0.09 g·min-1 when patients were on mechanical ventilation compared to 0.14 ±0.08 g·min-1 upon liberalization from it, however, the results were not statistically significant. Furthermore, mean lipid oxidation was 0.08 ±0.05 g·min-1 during mechanical ventilation and 0.09±0.07 g·min-1 upon liberalization from it and the mean difference was not statistically significant. Spearman correlation coefficient showed a positive relationship between actual calorie provision and duration of stay in intensive care (r=0.41 and p=0.01) and duration on mechanical ventilation (r=0.55 and p=0.00). Oxygen consumption (r=0.49 and p=0.00) and carbon dioxide production (r=0.4 and p=0.02) were moderately strong and positive during and upon liberation from mechanical ventilation. Correlation between lipid oxidation and oxygen consumption during ventilation (r=0.74, p=0.00) and after ventilation (r=0.82, p=0.00) as well as lipid oxidation and carbon dioxide production during ventilation (r=0.37, p=0.03) and liberation from ventilator (r=0.91, p=0.00) were significantly correlated with each other in grams per minute only. Conclusions: This is a pioneering study to examine energy expenditure and substrate utilization and oxidation within a single cohort of patients. The lower measured energy expenditure upon liberation from mechanical ventilation among critically ill patients could result from positive pressure support from ventilation, the repeated cycle of “rest” and “work” during weaning from ventilators and the asynchronization between self-initiated breathing effort and the ventilatory support. The positive relationship in duration on mechanical ventilation and length of stay with calorie consumption could be longer stay led to more time for progression to reach nutrition targets. . Any discrepancy in energy expenditure and substrate utilization with and without ventilatory support should be monitored. Future studies are important to examine whether matching energy expenditure with energy intake could promote positive clinical outcomes.

Introdução: A prevalência da obesidade infanto juvenil tem aumentado, sendo importante que mais profissionais estejam habilitados para o manejo. Objetivo: Comparar o efeito, após 12 meses, de três intervenções: programa de atividade física (AF), manejo dietoterápico (D) e atendimento ambulatório de referência em um hospital terciário (AMO) sobre o índice de massa corporal (IMC), composição corporal, taxa metabólica (TMB) e perfil metabólico de crianças e adolescentes com obesidade. Procedimentos Metodológicos: Ensaio clínico randomizado em 82 sujeitos de 8 a 15 anos, com obesidade. No grupo D, recebia dieta ajustada a partir da TMB por calorimetria indireta; no grupo AF, educador físico orientava prática de AF no domicílio e, no grupo AMO, orientações para introdução e manutenção de hábitos saudáveis. O acompanhamento foi mensal e os dados antropométricos, composição corporal, taxa metabólica basal e exames laboratoriais foram determinados no início, aos 6 e 12 meses de seguimento. O projeto foi aprovado pelo Comitê de Ética e Pesquisa do HCPA número 10-0011 e registrado www.clinicaltrials.gov NCT012973774. Resultados: Foram avaliados 82 sujeitos. A diferença (percentual) entre os grupos AF, D e AMO, respectivamente, foi de: escore Z do IMC -7,1% (-10,8 a -3,3), -5,0% (-8,4 a -1,5) e –15,2%(- 19,5 a -10,9); p=0,001; Peso (Kg) massa gorda 9,2% (4,3 a 14,2), 10,4% (4,8 a 15,9) e 4,3% (-1,7 a 10,4), p=0,248; Peso(Kg) massa livre de gordura 10,5%(7,5 a 13,4), 7,0% (4,1 a 10,0) e 6,7% (2,8 a 10,7) p=0,203; TMB 50,3%(2,2 a 232), 31,6%(-23,7 a 147), 38,6%(-17,3 a 232), p=0,669. As alterações laboratoriais mais frequentes no início do estudo nos grupos AMO, AF e D foram, respectivamente, valor HDL baixo (82,1%, 77,8%, 63%), insulina jejum 15 μUI/mL (71,4%, 81,5% e 66,7%) e homeostasis model assessment insulin resistence índex alterado (67,9%, 81,5%, 63%). O diagnóstico de síndrome metabólica (SM) foi feito em 20% das crianças obesas. No grupo AMO houve redução maior significativa do escore Z do IMC, da circunferência da cintura (CC), aumento do valor do HDL, redução da insulina de jejum e do HOMA-IR, redução da pressão sistólica e no número de componentes da SM. No grupo AF houve uma redução maior no colesterol total, no valor do LDL e dos triglicerídeos (TG). O grupo D também mostrou melhora no escore Z do IMC, redução do valor do colesterol total (CT), do LDL e dos TG. Apesar da redução dos indivíduos com diagnóstico de síndrome metabólica no grupo AMO (de 7 para 2) e AF (de 4 para 1) não houve diferença significativa quando comparados os 3 grupos. Conclusões: As três intervenções foram efetivas no tratamento da obesidade de crianças e adolescentes. Este estudo comprova a necessidade de estratégias combinadas e a longo prazo no manejo da obesidade na criança e adolescente para reduzir as alterações cardio metabólicas presentes nesta população e evitar a progressão dos fatores de risco para doenças cardiovasculares, metabólicas e morte prematura. Portanto, estas 3 estratégias podem ser utilizadas no manejo da obesidade infanto juvenil. / Background: The prevalence of paediatric obesity has increased in recent years, and health care workers must be trained to treat these conditions. Objective: To compare the effect of three year-long interventions: physical activity (PA) programme, dietary intervention (D) and treatment in an outpatient obesity clinic (OOC), on the body mass index (BMI), body composition, basal metabolic rate (BMR) and biochemical measurements of children and adolescents with obesity. Methodological Procedures: Randomized clinical trial of 82 participants aged between 8 and 15, diagnosed with obesity. Participants in the PA group received individualised home-based physical activity programmes from a physical education teacher. In the D group, participants received dietary counselling based on BMRs as measured by indirect calorimetry. In the OOC group, participants were given instructions on how to adopt and maintain healthy lifestyle habits. Participants had monthly appointments with physical educators, nutritionists or health care workers, depending on participant group. Anthropometric data, body composition, BMR and biochemical measurements were assessed at baseline, and after 6 and 12 months of treatment. Project approved by Comitê de Ética e Pesquisa do HCPA número 10-0011 and registred www.clinicaltrials.gov NCT012973774. Results: Differences between the PA, D and OOC groups over the course of the study were as follows: BMI Z score -7.1% (-10.8 to -3.3), -5.0% (-8.4 to -1.5) and –15.2%(-19.5 to -10.9); p=0.001; Fat mass (Kg) 9,2% (4,3 a 14,2), 10,4% (4,8 a 15,9) and 4,3% (-1,7 a 10,4), p=0,248; Fat-free mass weight(Kg) 10.5%(7.5 to 13.4), 7.0% (4.1 to 10.0) and 6.7% (2.8 to 10.7) p=0.203; BMR 50.3%(2.2 to 232), 31.6%(-23.7 to 147), 38.6%(-17.3 to 232), p=0.669. The most frequent laboratory anormalities at baseline in groups OOC, PA and D were, respectively, value low HDL (82.1%, 77.8%, 63%), fasting insulin 15 μUI / mL (71.4% , 81.5% and 66.7%) and high homeostasis model assessment insulin resistence index (67.9%, 81.5%, 63%). The diagnosis of metabolic syndrome was made in 20% of obese children. OOC group was significantly greater reduction in BMI Z score, waist circumference, total cholesterol, increasing the value of HDL, reduced fasting insulin and HOMA-IR, systolic blood pressure reduction and the number of metabolic syndrome components. In the PA group, there was a greater reduction in total cholesterol in the amount of LDL and TG. Group D also showed improvement in BMI Z score, reducing the value of total cholesterol, LDL and TG. Despite the reduction of individuals diagnosed with metabolic syndrome in the group OOC (7 to 2) and PA (4 to 1) no significant difference when comparing the three groups. Conclusions: All three interventions led to a reduction in BMI Z score, and increased fat free mass, BMR and biochemical measurements over the course of a year. This study demonstrates the need for combined strategies and long-term management of obesity in children and adolescents to reduce cardio metabolic changes present in this population and prevent the progression of risk factors for cardiovascular and metabolic diseases and premature death. Therefore, all three interventions are suitable treatments for paediatric obesity.

INTRODUÇÃO: A doença de Gaucher (DG) é um erro inato do metabolismo, do grupo das doenças lisossômicas, causado pela atividade deficiente da enzima glicocerebrosidase. Os tipos mais comuns da DG são o tipo I, que é o mais freqüente e não apresenta comprometimento neurológico; o II, agudo e neuropático; e o III, subagudo e neuropático. Todos os tipos caracterizam-se pela heterogeneidade clínica, com manifestações sintomáticas e de intensidade distintas, tais como hepatoesplenomegalia, alterações ósseas e hematológicas. Alguns estudos descrevem alterações metabólicas como gasto energético basal (GEB) aumentado – hipermetabolismo - em pacientes sem tratamento. A terapia de escolha para a DG é a reposição enzimática (TRE), a qual consegue reverter muitas das manifestações da doença. OBJETIVOS: 1) Avaliar o GEB por meio de calorimetria indireta em pacientes com DG do Centro de Referência do Rio Grande do Sul; 2) Avaliar o estado nutricional dos pacientes incluídos no estudo; 3) Relacionar o GEB com as condições clínicas dos pacientes. METODOLOGIA: Estudo transversal, prospectivo, controlado. Os pacientes atendidos no CRDG foram convidados a participar do estudo (n= 29), sendo que 17 concordaram (média de idade= 30,0 ± 17,2 anos, sexo masculino= 8; DG tipo III= 3 pacientes). Os pacientes com DG tipo I (n= 14; sexo masculino= 6) foram pareados por sexo, idade e índice de massa corporal (IMC) com controles hígidos para avaliação do GEB. Para determinação dos valores de VO2 e VCO2 foi utilizado um ergoespirômetro (MedGraphics Cardiorespiratory Diagnostic Systems, modelo CPX-D). Os pacientes e os controles receberam orientação prévia quanto ao jejum e o repouso e no dia da calorimetria foram pesados e medidos, sendo então calculado o IMC. Os pacientes não apresentavam outras morbidades, nem estavam em uso de medicamentos que poderiam interferir no GEB. Nas análises estatísticas, foi utilizado o GEB em kcal/kg/dia. RESULTADOS: A avaliação do estado nutricional revelou que, no grupo dos pacientes com DG tipo I, cinco estavam com sobrepeso e os demais eutróficos; no grupo com DG tipo III, dois pacientes encontravam-se desnutridos e um eutrófico. Foram realizadas 19 avaliações do GEB em 17 pacientes: dois pacientes a realizaram no período pré e após 6 meses de TRE. A média de idade e de IMC dos pacientes com DG tipo I e dos controles foi de 32,8 ± 17,6 e 32,1 ± 16,6 anos e 23,3 ± 3,1 e 22,4 ± 3,1kg/m2, respectivamente. A idade dos pacientes com DG tipo III foi, respectivamente, 12, 17 e 20 anos. Quatorze pacientes estavam recebendo TRE (média de tempo de TRE= 6,6 ± 5,3 anos; média de dose de enzima= 27,1 ± 11,7 UI/kg/inf. de imiglucerase). A média de GEB dos pacientes com DG tipo I em TRE (n= 12) foi 27,1% maior do que a dos controles (p= 0,007). O GEB de pacientes em TRE (n=12) comparado aos sem TRE (n= 4) não apresentou diferença (p= 0,92). Comparando o GEB dos pacientes em TRE e o de seus controles com o GEB estimado pela equação de Harris-Benedict, observou-se que os pacientes apresentaram GEB 6,3% maior do que o estimado (p= 0,1), enquanto que seus controles tiveram GEB 17,0% menor do que o estimado (p= 0,001). O GEB medido dos pacientes com DG tipo III foi, respectivamente, 14%, 72% e 16% maior do que o estimado pela equação de Harris e Benedict. Não foi encontrada associação significativa entre GEB e as seguintes variáveis: idade, peso, estatura, escore de gravidade, quantidade de enzima recebida, idade de início de TRE, tempo de tratamento e presença ou ausência de megalias. A correlação do GEB com o IMC foi negativa e significativa, conforme esperado. DISCUSSÃO/CONCLUSÕES: O estado nutricional classificado pelo IMC mostrou que a maior parte dos pacientes com DG tipo I estava eutrófica; no entanto, um terço apresentou pré-obesidade. Dois dos três pacientes com DG tipo III encontravam-se desnutridos. Todos os pacientes, mesmo em TRE, apresentaram um GEB significativamente maior do que os controles. A TRE não consegue normalizar o hipermetabolismo desses pacientes. / INTRODUCTION: Gaucher disease (GD) is an inborn error of metabolism of the group of lysosomal diseases, caused by the deficient activity of the glucocerebrosidase enzyme. The most common types of GD are: type I, which is the most frequent and does not present neurological compromise; type II, which is acute and neuropathic; and type III, which is subacute and neuropathic. All types are characterized by clinical heterogeneity and symptomatic manifestations of various intensity, such as hepatoesplenomegaly and bone and hematological alterations. Some studies have described metabolic alterations, such as increased basal metabolic rate (BMR), that is, hypermetabolism, in untreated patients. The therapy of choice for GD is enzyme replacement therapy (ERT), which can stop many manifestations of the disease. OBJECTIVES: 1) To evaluate BMR by means of indirect calorimetry in patients with GD seen at the Reference Center for Gaucher Disease of Rio Grande do Sul (RCGD); 2) To evaluate the nutritional status of patients included in the study; 3) To relate BMR with clinical conditions presented by patients. METHODS: The present was a prospective, controlled, cross-over study. Patients seen at the RCGD were invited to participate in the study (n=29); of these, 17 agreed to participate (mean age=30.0 ± 17.2 years, male= 8; GD type III=3 patients). Patients with GD type I (n=14; male= 6) were paired by gender, age, and body mass index (BMI) to healthy controls to evaluate BMR. To determine the values of VO2 and VCO2 an ergospirometer was used (MedGraphics Cardiorespiratory Diagnostic Systems, model CPX-D). Patients and controls received previous orientation as to fasting and resting and, on the day of the calorimetry, were weighed and measured in order for the BMI to be calculated. Patients did not present any other morbidity, neither were they making use of any medication that could interfere with BMR. In the statistical analyses, BMR in kcal/kg/day was used. RESULTS: The evaluation of the nutritional status showed that, in the group of patients with GD type I, five patients were overweight; the other were eutrophic; in the group of patients with GD type III, two patients were malnourished; one was eutrophic. Nineteen evaluations of BMR were conducted in 17 patients; two patients conducted the evaluation in the period pre-ERT and after 6 months of ERT. Mean age and mean BMI of patients with GD type I and controls were 32.8 ± 17.6 and 32.1 ± 16.6 years and 23.3 ± 3.1 and 22.4 ± 3.1kg/m2, respectively. The age of patients with GD type III was, respectively, 12, 17 and 20 years. Fourteen patients were receiving ERT (mean time of ERT=6.6 ± 5.3 years; mean enzyme dose=27.1 ± 11.7 UI/kg/inf of imiglucerase). The mean BMR of patients with GD type I on TRE (n=12) was 27.1% higher when compared to controls (p=0.007). When compared to patients not on ERT (n=4), the BMR of patients on ERT (n=12) did not show any difference (p=0.92). Comparing the BMR of patients on ERT and that of their controls with the BMR estimated by the Harris-Benedict equation, we observed that patients showed a 6.3% higher BMR than the estimated (p=0.1), while the BMR of their controls was 17.0% lower than the estimated (p=0.001). The BMR of patients with GD type III was, respectively, 14%, 72% and 16% higher than the estimated by the Harris-Benedict equation. No significant association was found between BMR and the following variables: age; weight; height; severity score; amount of enzyme received; age at beginning of ERT; time of treatment; and presence or absence of megalies. The correlation between BMR and BMI was negative and significant, as expected. DISCUSSION/CONCLUSIONS: The nutritional status classified by BMI showed that most patients with GD type I were eutrophic; however, one third of the patients showed pre-obesity. Two of the three patients with GD type III were malnourished. All patients, even on ERT, showed a significantly higher BMR when compared to controls. In conclusion, ERT was not able to normalize the hypermetabolism of these patients.