Nutritional status evaluation in patients affected by bethlem myopathy and ullrich congenital muscular dystrophy.

Collagen VI mutations lead to disabling myopathies like Bethlem myopathy (BM) and Ullrich congenital muscular dystrophy (UCMD). We have investigated the nutritional and metabolic status of one UCMD and seven BM patients (five female, three male, mean age 31 ± 9 years) in order to find a potential metabolic target for nutritional intervention. For this study, we used standard anthropometric tools, such as BMI evaluation and body circumference measurements. All results were compared to dual-energy X-ray absorptiometry (DXA), considered the "gold standard" method. Energy intake of each patient was evaluated through longitudinal methods (7-day food diary) while resting energy expenditure (REE) was predicted using specific equations and measured by indirect calorimetry. Clinical evaluation included general and nutritional blood and urine laboratory analyses and quantitative muscle strength measurement by hand-held dynamometry. BM and UCMD patients showed an altered body composition, characterized by low free fat mass (FFM) and high fat mass (FM), allowing us to classify them as sarcopenic, and all but one as sarcopenic-obese. Another main result was the negative correlation between REE/FFM ratio (basal energy expenditure per kilograms of fat-free mass) and the severity of the disease, as defined by the muscle megascore (correlation coefficient -0.955, P-value <0.001). We postulate that the increase of the REE/FFM ratio in relation to the severity of the disease may be due to an altered and pathophysiological loss of energetic efficiency at the expense of skeletal muscle. We show that a specific metabolic disequilibrium is related to the severity of the disease, which may represent a target for a nutritional intervention in these patients.

Body composition, eating disorder psychopathology, and psychological distress in anorexia nervosa: a longitudinal study.

BACKGROUND: Although the effect of immediate weight restoration on body composition and body fat distribution has previously been studied in anorexia nervosa (AN), its influence in women with AN on eating disorder psychopathology and psychological distress has not previously been investigated to our knowledge. OBJECTIVES: We assessed body composition and fat mass distribution before and after body weight restoration and investigated any relation between changes in body fat patterns of patients with AN treated in a specialist inpatient unit and their eating disorder and psychological distress features. DESIGN: Body composition was measured by using dual-energy X-ray absorptiometry in 50 female, adult patients with AN before and after complete weight restoration [body mass index (BMI; in kg/m²) ≥18.5] and 100 healthy control subjects matched by age and posttreatment BMI of study group participants. Eating disorder psychopathology and psychological distress were assessed in the AN group before and after weight restoration by using the Eating Disorder Examination interview and the Global Severity Index of the Brief Symptom Inventory (BSI-GSI), respectively. RESULTS: After the achievement of complete weight restoration, patients with AN had higher trunk (P < 0.001), android (P < 0.001), and gynoid (P < 0.001) fat masses and lower arm (P < 0.001) and leg (P = 0.001) fat masses with respect to control subjects. No relation was shown between body-composition variables and eating disorder psychopathology in the AN group, and the only significant predictor of change in BSI-GSI was the baseline BSI-GSI score. CONCLUSION: The normalization of body weight in patients with AN is associated with a preferential distribution of body fat in central regions, which does not, however, seem to influence either eating disorder psychopathology or psychological distress scores.

Measured physical activity in anorexia nervosa: features and treatment outcome.

OBJECTIVE: To assess the role of measured physical activity (PA) in anorexia nervosa treatment outcome, and to compare the PA of patients with anorexia nervosa with age-matched controls. METHOD: PA was assessed by means of Sense Wear Armband before and after a cognitive-behavioral inpatient treatment in 53 consecutive females with anorexia nervosa, and in 53 healthy age-matched controls. RESULTS: At baseline, patients with anorexia nervosa exhibited a higher duration of moderate-vigorous PA (MVPA≥3 Metabolic Equivalent Tasks (METs)) than controls (t = 2.91; p = .004). Dropouts had higher duration (sec) and expenditure (kcal·day(-1)) of MVPA than completers. At the end of treatment, completers had a higher number of daily steps, MVPA duration, and expenditure than controls. However, PA was not correlated to eating disorder psychopathology either before or after treatment. DISCUSSION: PA is higher in patients with anorexia nervosa than age-matched controls both before and after treatment, and is associated with treatment dropout.

Short-term multidisciplinary non-pharmacological intervention is effective in reducing liver fat content assessed non-invasively in patients with nonalcoholic fatty liver disease (NAFLD).

BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) ranges from simple steatosis to steatohepatitis, and cirrhosis in patients with alcohol intake less than 20 g/day, and is usually associated with insulin resistance (IR). AIM: Given that no drugs are specifically approved for NAFLD, we tested the efficacy of a non-pharmacological multidisciplinary intervention based on a personalized diet, physical activity and behavior therapy. METHODS: In this open non-randomized study, personalized diet, physical exercise and behaviour therapy for 3 months were prescribed in 12 consecutive patients with NAFLD. Lifestyle, including total caloric intake, physical activity and resting energy expenditure was monitored by a SenseWear Armband. Insulin Resistance (IR) was measured by HOMA and oral glucose insulin sensitivity tests (OGIS); fat liver content was estimated by two different semi-quantitative scores and by the Doppler Power Index (DPI). RESULTS: Data show that the multidisciplinary intervention produced a significant reduction of total caloric intake, a 8% reduction in body weight, a modest increase in daily physical activity, a significant (P<0.001) reduction of aminotransferases and a decrease of total hepatic fat content. CONCLUSIONS: A 3-month multidisciplinary intervention inducing at least 8% of weight loss, improves liver tests and decreases liver fat content.

Body Fat Mass Assessment: A Comparison between an Ultrasound-Based Device and a Discovery A Model of DXA.

Objective. To examine measurement of body composition by ultrasound compared with a reference technique:dual energy X-ray absorptiometry (DXA). We evaluated the accuracy of a portable ultrasound-based device in estimating total body fat mass with those assessed by DXA in adult. Methods. Body fat mass has been estimated using a portable ultrasound-based device in comparison with a contemporary reference DXA apparatus: the Hologic Discovery A. Anthropometric data has been assessed in order to maximize the output of the software associated with the ultrasound-based device. A cross-validation between ultrasound technique (US) and DXA was developed in this study. Total body fat mass estimated by ultrasound was compared with this DXA model in a sample of 83 women and 41 men. Results. Ultrasound technique (US) of body fat (BF) was better correlated with DXA in both women (r (2) = 0.97, P < 0.01) and men (r (2) = 0.92, P < 0.01) with standard errors of estimates (SEE) being 2.1 kg and 2.2 kg, respectively. Conclusion. The use of a portable device based on a US produced a very accurate BF estimate in relation to DXA reference technique. As DXA absorptiometry techniques are not interchangeable, the use of our ultrasound-based device needs to be recalibrated on a more contemporary DXA.

Comparison between dual-energy X-ray absorptiometry and skinfolds thickness in assessing body fat in anorexia nervosa before and after weight restoration.

BACKGROUND & AIMS: The aim of the study was to evaluate the correspondence between body fat mass composition (percentage) measured with dual-energy X-ray absorptiometry (DXA) and estimated by means of skinfold thicknesses (ST) measurement in patients with anorexia nervosa (AN), before and after weight gain. METHODS: Percentage body fat (%BF) was measured with DXA and estimated by ST measurements using Siri, Brozek, and Heyward equations in 27 adult patients with AN before and after weight gain (pre- and post-treatment) achieved with inpatient treatment and in 42 healthy age-matched controls. RESULTS: Due to Lohman criteria and Bland Altman plot there is no correspondence between the %BF measured with DXA and the %BF estimated by predictive equations based on ST measurements in patients with AN before and after weight gain, with the exception of Brozek equation which showed a mild agreement in pre-treatment AN. However, a correspondence was observed between the two procedures in healthy controls. CONCLUSIONS: Our data supporting the use of ST measurements do not appear to be an alternative to DXA in estimating body fat percentage, before and after weight gain in patients with AN.

Resting energy expenditure assessment in anorexia nervosa: comparison of indirect calorimetry, a multisensor monitor and the Müller equation.

The aim of this study was to compare the estimations provided by three different means of measuring the resting energy expenditure (REE) in anorexia nervosa (AN) patients. REE was measured, after 24 h of refeeding, using a portable multisensor body monitor [SenseWear Pro2 Armband (SWA)], FitMate™ method and the Müller equation for individuals with body mass index < 18.5, the latter being based on dual-energy X-ray absorptiometry assessment of body composition. The mean differences between REE values estimated by SWA and those provided by the Müller equation and the FitMate™ method were significantly different from zero in both cases. In contrast, the mean differences between FitMate™ method and Müller equation were weakly significantly different from zero, and a significant correlation was noted between these two methods. In conclusion, the SWA does not appear to be an alternative to FitMate™ and Müller equation methods for assessing REE in AN patients.

Influence of lifestyle habits, nutritional status and insulin resistance in NAFLD.

Non alcoholic fatty liver disease (NAFLD) is associated with obesity, diabetes and insulin resistance (IR). The aim of our study was to assess the relationship between IR, anthropometry, lifestyle habits, resting energy expenditure (REE) and degree of fatty liver at ultrasound in 48 overweight patients with NAFLD as compared to 24 controls without fatty liver, matched for age. Nutritional status, alcohol intake and physical activity were assessed by skinfold thickness measurements, a 7-day diary, and SenseWear armband (SWA). REE was assessed by both SWA (REE-SWA) and a Vmax metabolic cart (REE-Vmax). Fatty liver was measured by US and the Doppler Power Index was calculated. IR was assessed using the HOMA index. There was significant correlation between waist circumference, HOMA, Doppler power index and fatty liver grade at US. Multivariate analysis showed that alteration of waist circumference, Doppler power index, and HOMA were the major significant predictors of fatty liver. Our data demonstrated a significant association between NAFLD and central adiposity and IR.

Fluid intake and nutritional risk in non-critically ill patients at hospital referral.

The association between hyporexia/anorexia, reduced food intake and disease-related malnutrition at hospital admission is well established. However, information on fluid intake according to nutritional risk has never been provided. Thus, we assessed the attitude and adequacy of fluid intake among case-mix hospitalised patients according to nutritional risk. A sample of 559 non-critically ill patients randomly taken from medical and surgical wards was evaluated. Nutritional risk was diagnosed by the Nutritional Risk Screening 2002. Usual fluid consumption the week before admission was assessed and categorised as < 5 and > or = 5 cups/d (1 cup = 240 ml), with the acceptable intake being > or = 5 cups/d. Prevalence of nutritional risk was 57.2%, and 46.2% of the patients reported a fluid intake < 5 cups/d. Multiple-adjusted logistic regression revealed that age > or = 65 years (OR: 1.88 (95% CI: 1.03, 3.43); P < 0.04), energy intake (for every 25% increase in food intake compared with estimated requirements, OR: 0.37 (95% CI: 0.25, 0.55); P < 0.001) and the number of drugs taken (every three-drug increase, OR: 0.63 (95 % CI: 0.44, 0.90); P < 0.02) were independently associated with inadequate fluid intake (< 5 cups/d). A significant independent association was also found with nutritional risk (OR: 0.64 (95% CI: 0.43, 0.95); P < 0.03). Nutritional risk appears to be positively associated with greater fluid intake in non-acute hospitalised patients, but both the reasons and the consequences of this relationship, as well as the impact on clinical practice, need to be explored. However, water replacement by oral nutritional support should take advantage of the patients' attitude to assuming a greater fluid intake, limiting at the same time fluid overload during the refeeding phase.

Low adherence of a clinically healthy Italian population to nutritional recommendations for primary prevention of chronic diseases.

BACKGROUND AND AIM: Several data demonstrated that dietary habits significantly affect the health state of the population. During recent years all the major scientific associations have provided nutritional recommendations for primary prevention of chronic diseases but few data are available about prevalence of adherence to these recommendations in an otherwise healthy population. The aims of this study were to evaluate dietary habits, and to assess the adherence of the general population to the recommendations for correct nutritional behaviour. METHODS AND RESULTS: Dietary habits, anthropometric and biochemical parameters were evaluated in a population of 932 (367 M; 565 F) clinically healthy subjects living in Florence, enrolled in an epidemiologic study conducted between 2002 and 2004. By comparing the dietary pattern with the nutritional guidelines, the study population reported a hyperproteic and hyperlipidic nutritional pattern, with a considerably low contribution from polyunsaturated fats (PUFA). A low fibre intake is shown in both genders. In addition, food consumption pattern showed an increased consumption of some foods such as meat, both fresh and processed, and a low intake of some "healthy" foods like fruit and vegetables. CONCLUSIONS: We found several nutritional flaws in the dietary habits of a clinically healthy Italian population. In particular, we reported a high intake of animal protein and total fats with a very low contribution from PUFA.