Evaluation of the Nutritional Status of Gaucher Disease Type I Patients under Enzyme Replacement Treatment.

(1) Background: Gaucher disease (GD) is a rare lysosomal storage disease. The few studies analyzing Resting Energy Expenditure (REE) in GD involved mainly untreated patients and supported a hypermetabolic condition possibly due to the associated inflammatory state. Definitive conclusions could not be drawn also because of the heterogeneity and the small size of the samples investigated. In order to expand current knowledge concerning, in particular the condition of patients under Enzyme Replacement Therapy (ERT), we evaluated the nutritional status of a relatively large sample of GD patients followed at Federico II University Hospital in Naples, Italy. (2) Methods: The study, having a cross-sectional design and involving 26 patients on ERT, included routine biochemical analyses, bioelectrical impedance analysis, indirect calorimetry, and administration of food frequency and physical activity questionnaires. The results in GD patients were compared with those from an appropriate control group. (3) Results: GD patients had normal biochemical parameters in 80% of cases, except for HDL-cholesterol, consumed a hyper-lipidic diet, and had a 60% prevalence of overweight/obesity. Body composition did not differ between patients and controls; however, measured REE was significantly lower than predicted and was reduced in comparison with the healthy controls. (4) Conclusions: This study provided novel elements to the present knowledge about REE and the nutritional status of GD patients under ERT. Its results warrant confirmation in even larger GD population samples and a more in-depth investigation of the long-term effects of treatment superimposed on the basic pathophysiological disease condition.

Prevalence of Sarcopenia in Women with Breast Cancer.

Sarcopenia is a common finding in patients with cancer and potentially influences the patient's outcome. The aim of this study was to evaluate the prevalence of sarcopenia, according to the European Working Group on Sarcopenia in Older People, in a sample of women with breast cancer (BC) and a BMI lower than 30 kg/m2. This cross-sectional study was conducted in patients with BC, stage 0-III, and receiving therapy for BC; the women were recruited at the Department of Clinical Medicine and Surgery, Federico II University, Naples, Italy. A control group with similar age and BMI was selected from the internal database. Anthropometry, bioimpedance analysis (BIA) and hand grip strength (HGS) were measured to detect sarcopenia. A total of 122 patients (mean age 49.3 ± 11.0 years, BMI 24.6 ± 3.0 kg/m2) and 80 healthy controls were analyzed. Sarcopenia was found in 13.9% patients with BC, while none of the subjects in the control group was sarcopenic. By comparing BC patients with and without sarcopenia and the control group, the fat-free mass of sarcopenic BC patients were significantly lower than those of both non-sarcopenic BC patients and the control (p < 0.05). The phase angle was also significantly lower in sarcopenic patients (−0.5 degrees, p = 0.048) than in the control group. Considering the prevalence of sarcopenia in patients with BC, our findings suggest the usefulness of body composition and HGS evaluation for early screening of sarcopenia to reduce the risk of associated complications.

Nutritional Screening and Anthropometry in Patients Admitted From the Emergency Department.

BACKGROUND: Due to the high prevalence of malnutrition among hospitalized patients, screening and assessment of nutritional status should be routinely performed upon hospital admission. The main objective of this observational study was to evaluate the prevalence of and the risk for malnutrition, as identified by using three nutritional screening tests, and to observe whether some anthropometric and functional parameters used for nutritional evaluation were related to these test scores. METHODS: This single-center observational study included 207 patients admitted from the emergency department for hospitalization in either the internal medicine or surgery units of our institution from September 2017 to December 2018. The prevalence of malnutrition among this patient sample was evaluated by using the Nutritional Risk Screening (NRS-2002), the Subjective Global Assessment (SGA) and the Global Leadership Initiative on Malnutrition (GLIM) criteria. Body mass index (BMI), bioimpedance analysis (BIA), handgrip strength (HGS) and calf circumference (CC) assessments were also performed. RESULTS: According to the NRS-2002, 93% of the patients were at no risk or at low nutritional risk (NRS score < 3), and 7% were at a high nutritional risk (NRS score ≥ 3). On the other hand, according to the SGA, 46.3% of the patients were well-nourished (SGA-a), 49.8% were moderately malnourished (SGA-b), and 3.9% were severely malnourished (SGA-c). Finally, according to the GLIM criteria, 18% patients were malnourished. Body weight, body mass index (BMI), phase angle (PhA), CC and HGS were significantly lower in the patients with NRS scores ≥ 3, SGA-c and in patients with stage 1 and stage 2 malnutrition, according to the GLIM criteria. CONCLUSION: The NRS-2002, the SGA and the GLIM criteria appear to be valuable tools for the screening and assessment of nutritional status. In particular, the lowest NRS-2002, SGA and GLIM scores were associated with the lowest PhA and CC. Nevertheless, a weekly re-evaluation of patients with better screening and assessment scores is recommended to facilitate early detection of changes in nutritional status.

A proposal for reference values of hand grip strength in women with different body mass indexes.

OBJECTIVE: Hand grip strength (HGS) is frequently used in clinical practice, resulting in a potential marker of nutritional status. This study aimed to develop reference values of HGS in Italian women with different categories of body mass index (BMI). Additionally, the main predictors of HGS were identified. METHODS: A cross-sectional study was conducted in Italian women between ages 16 and 55 y with different categories of BMI at the Department of Clinical Medicine and Surgery, Federico II University Hospital, Naples Italy. The whole sample was divided into tertiles according to BMI: 15 to 17.29 kg/m2 (T1), 17.3 to 19.9 kg/m2 (T2), and 20 to 25 kg/m2 (T3). Anthropometry, bioimpedance analysis, and muscle strength by an HGS test were evaluated. The cut-off values for HGS were developed for all participants and stratified by age group. Finally, a multivariate linear regression analysis was performed to assess the main predictors of HGS. RESULTS: A total of 529 women with a mean age of 23.2 ± 7.0 y and an average BMI of 18.9 ± 2.5 kg/m2 were analyzed. HGS was higher for the dominant hand than for the non-dominant hand in all BMI tertiles. On both sides, according to age groups, HGS increased with increasing age in T1 and T3, whereas it increased in the women between ages 20 and 30 y in T2 only. Multivariate linear regression analysis showed that predictors of HGS varied according to tertiles. Specifically, we found that body weight (R2 = 0.252) was the main predictor in T1, whereas phase angle (PhA) was the main determinant in both T2 (R2 = 0.240) and T3 (R2 = 0.216). CONCLUSION: This study defined the normal reference values of HGS in Italian women with different BMI ranges, stratifying the sample group by age. Additionally, the main predictors of HGS were assessed for each BMI tertile. In primary malnutrition (T1), the main predictor of HGS was body weight, whereas in the other two tertiles (T2, T3), the PhA was the main predictor of HGS.

Nutritional indicators and metabolic alterations in outpatients with anorexia nervosa: a retrospective study.

PURPOSE: In patients living with Anorexia Nervosa (AN), dehydration and haemoconcentration, may prevent a correct interpretation of laboratory nutritional parameters. Our study aims to evaluate if some indicators of disease severity, as body mass index (BMI), Phase Angle (PhA) and months of amenorrhea may be predictors of metabolic alterations (serum albumin, liver enzymes). METHODS: In 154 outpatients with AN, case history was collected, and anthropometric and laboratory parameters measured. Patients were divided according to the following tertiles (T) of BMI, duration of amenorrhea and PhA: (1) BMI (T1 < 15.6; T2 15.6-16.8; T3 > 16.8 kg/m2); (2) Amenorrhea duration (T1 < 7; T2 7-14; T3 > 14 months); (3) PhA value (T1 < 4.64; T2 4.64-5.35; T3: > 5.35°). ROC curves were used to determine which of these three indicators (BMI, PhA and amenorrhea duration) might better identify patients belonging to Group A or B (less than 3 or more metabolic abnormalities). RESULTS: The most frequent registered metabolic alterations were for alkaline phosphatase (ALP), alanine aminotransferase, cholesterol and hemoglobin. Aspartate aminotransferase, ALP and gamma glutamyl transferase abnormalities were frequent in the first tertiles of all the three indicators. Albumin was low in the T1 of BMI and PhA. No differences in nutritional alterations emerged according to amenorrhea duration. PhA had the best performance (AUCs: 0.721) in identifying patients with 3 or more abnormalities, with the optimal cut-off value of 4.5°. CONCLUSIONS: Our data confirmed PhA as the more reliable predictor of metabolic alterations, followed by BMI and amenorrhea duration, especially in the first tertile. EVIDENCE-BASED MEDICINE: Level 2.

New predictive equations for estimating resting energy expenditure in subjects with normal weight and overweight.

OBJECTIVE: The aim of this study was to develop and validate new predictive equations for estimating resting energy expenditure (REE) in subjects with normal weight and overweight, considering anthropometric parameters as well as raw variables from bioimpedance analysis (BIA). METHODS: Adult participants with normal weight and overweight were recruited and randomly split into calibration and validation groups. Indirect calorimetry (IC) and BIA were performed in all subjects. New predictive equations were developed using the following models: model 1 with age, weight, stature, and body mass index (BMI) as predictors; and model 2: model 1 + raw BIA variables (bioimpedance index and phase angle). The accuracy of the new equations at both the group (bias) and individual (within ±10%) levels was tested in the validation group. Three published predictive equations were also compared, with the REE values measured by IC. RESULTS: A total of 2483 adults were included for developing and validating the new equations. All selected formulas, including the new ones, showed a bias of <5% in estimating REE at the group level. Accuracy at the individual level was slightly higher for the new equations, especially for the equation based on raw BIA variables (men = 70.3%; women = 72.3%). CONCLUSIONS: Compared to the equations in the literature, the new equations showed good accuracy at both the group and individual levels, with a slight improvement in individual accuracy for the formula including raw BIA variables. However, future research is required to verify the role of the raw BIA variables in predicting REE in subjects with normal weight and overweight.

Bilio-pancreatic diversion: report of a case involving bone metabolism complications.

Prolonged vitamin D deficiency may result in lower calcium absorption and osteoporosis, leading to pathologic fractures. We report the case of a young woman with severe, complicated osteoporosis, which developed several years after biliopancreatic diversion that was performed to treat morbid obesity. Chronic low vitamin D levels provided a continuous stimulus for parathormone secretion, which resulted in parathyroid hyperplasia/adenoma and autonomous production of the hormone.

Long-Term Outcomes from a 10-Year Follow-Up of Women Living with a Restrictive Eating Disorder: A Brief Report.

BACKGROUND: This study aimed to evaluate several socio-demographic and long-term clinical outcomes in a cohort of women living with a restrictive eating disorder. METHODS: Patients were asked to fill in a general data collection form aiming to investigate their current conditions and to attend the outpatient unit for a 10-year follow-up clinical and laboratory evaluation. RESULTS: Forty-four patients completed the follow-up general data collection form and 20 agreed to attend the outpatient unit for the 10 year-follow-up evaluation. In total, 52% of patients were single, 55% had achieved a university degree, and 55% had steady employment. After 10 years, there was a clear improvement in biochemical markers, but cholesterol levels were still slightly high. The prevalence of osteopenia in the whole sample was 70% when measured on the lumbar column and 20% on the total body, while osteoporosis was found in 10% of patients and only on the lumbar column. CONCLUSION: According to the collected data, women with a history of restrictive eating disorders appear to re-adapt well to social life by obtaining the level of their unaffected peers in terms of education and employment.

Body composition, segmental bioimpedance phase angle and muscular strength in professional volleyball players compared to a control group.

BACKGROUND: The assessment of body composition is central in evaluating athletes' nutritional status and the effects of training. The aim of this study was to evaluate body composition and the relation between bioimpedance phase angle (PhA) and muscular strength in elite female volleyball players. METHODS: Twelve volleyball players (age 23.8±3.6 years; weight 63.0±5.1 kg; height 170±4 cm; BMI 21.9±1.3 kg/m2) and 22 non-athletic females, who served as a control group (age 23.6±2.0 years; weight 60.7±4.8 kg; height 167±5 cm; BMI 21.9±1.3 kg/m2), participating in the study. Skinfold thickness measures were used and segmental bioelectrical impedance analysis (BIA) was performed. Additionally, hand grip strength was used to evaluate muscular strength. RESULTS: Volleyball players had lower FM and higher FFM than controls (FM(kg)=15.7±2.7 vs. 18.0±3.0, P=0.036; FM(%)=24.8±3.0 vs. 29.5±3.8, P=0.001; FFM(kg)=47.4±3.5 vs. 42.8±3.6, P=0.001). Both whole-body and segmental PhA were higher in volleyball players (P<0.05) than in controls, whereas no differences were observed for hand grip strength. A positive relation was found between hand grip strength and whole-body and upper limbs PhA in all subjects, resulting stronger in volleyball players (r=0.696, P=0.012 and r=0.821, P=0.001, respectively). CONCLUSIONS: The data confirmed that body composition differed between volleyball players and non-athletic subjects. In addition, a strong correlation between PhA and hand grip strength in both volleyball players and in controls was found. However, further evaluations are needed to investigate the use of hand grip strength in sport performance.

Resting Energy Expenditure, Body Composition and Phase Angle in Anorectic, Ballet Dancers and Constitutionally Lean Males.

BACKGROUND: The prevalence of anorexia nervosa among males is increasing but few data are available in the literature. This cross sectional study aims to evaluate resting energy expenditure (REE) and phase angle as a marker of qualitative changes of fat free mass (FFM) in three leanness groups as compared with control subjects. METHODS: 17 anorectic (AN) males, 15 constitutionally lean (CL) individuals, 12 ballet dancers (DC), and 18 control (CTR) subjects were evaluated. REE was measured by indirect calorimetry (V max29- Sensormedics), and body composition was evaluated by bioimpedance analysis (BIA) at 50 kHz (DS Medica). Phase angle (a bioimpedance variable related to nutritional status) was used to evaluate differences in FFM characteristics between these three types of leanness. RESULTS: REE, adjusted for FFM and fat mass (FM), were significantly higher in CL and lower in AN individuals (1783 ± 47 vs. 1291 ± 58 kcal, p < 0.05) compared to the other groups. Body composition was similar in AN and CL whereas dancers had the highest FFM (58.9 ± 4.8 kg, p < 0.05); anorectic males showed the lowest phase angle (5.8 ± 1.2 degrees vs. other groups, p < 0.05) and dancers the highest phase angle (7.9 ± 0.7 degree vs. other group, p < 0.05). CONCLUSIONS: Our findings confirm that phase angle could be a useful marker of qualitative changes, above all in the field of sport activities. On the other hand, there is the need to further evaluate the relationship between resting energy expenditure, body composition and endocrine status in different conditions of physical activity and dietary intake.

Fecal Short Chain Fatty Acids and Dietary Intake in Italian Women With Restrictive Anorexia Nervosa: A Pilot Study.

Nutritional disorders such as Anorexia Nervosa (AN) can shape the composition of gut microbiota and its metabolites such as short chain fatty acid (SCFA). This study aims to compare fecal SCFA along with dietary intake of women with restrictive AN (r-AN = 10) and those of sex-matched lean controls (C = 8). The main fecal short chain fatty acids (SCFA) were assessed by gas chromatography equipped with a flame ionization detector. All participants completed 7-day food record and underwent indirect calorimetry for measuring resting energy expenditure (REE). Butyrate and propionate fecal concentrations were significantly reduced in r-AN patients compared to controls. The intake of carbohydrate and fat was significantly lower in r-AN patients than controls as well as energy intake and REE; whereas the amount of protein and fiber did not differ between groups. These preliminary results showed that r-AN patients had a reduced excretion of fecal SCFA, likely as a mechanism to compensate for the lower energy and carbohydrate intake observed between groups. Therefore, further studies need to be performed in patients with AN to explore the link between nutritional disorders, gut microbiota and its metabolites.

Prediction of body composition in anorexia nervosa: Results from a retrospective study.

BACKGROUND & AIMS: The assessment of body composition is crucial in evaluating nutritional status in female subjects with anorexia nervosa (AN) and improving their clinical management. The aim of this retrospective study was to assess the accuracy of selected BIA (bioimpedance analysis) equations for fat-free mass (FFM) in female AN subjects and to formulate a specific equation for these subjects. METHODS: Eighty-two restrictive female AN subjects (age 20.5 ± 3.7 yrs, BMI 15.7 ± 1.7 kg/m2) were studied. Body composition was determined with dual-energy X-ray absorptiometry (DXA) and estimated by BIA using five different equations. Linear correlation analysis was carried out to evaluate the association of FFM with selected variables. Multiple regression analysis was used to formulate specific equations to predict FFM in AN. RESULTS: All predictive equations underestimated FFM at the population level with a bias from -5.6 to -11.7%, while the percentage of accurate predictions varied from 12.2% to 35.4%. More interestingly, multiple regression analysis clearly indicates that, in addition to weight, ZI100 or RI also emerged as independent predictors of DXA-derived FFM, increasing the prediction power of the equation well above that observed with anthropometric characteristics only. CONCLUSIONS: This study shows that the selected predictive BIA equations considered exhibit an insufficient accuracy at the population and the individual level. Predictive formulas based on body weight plus BIA parameters such as RI and ZI100 offer a rather accurate prediction of FFM (with high R squared).

Effects of Armolipid Plus on small dense LDL particles in a sample of patients affected by familial combined hyperlipidemia.

AIM: The aim of this study was to test small dense LDL changes with Armolipid Plus treatment in patients with familial combined hyperlipidemia (FCHL). METHODS: After 4 weeks, 30 patients with FCHL were included in an 8-week, randomized, double-blind study and were taking, in addition to the standard diet, either placebo or Armolipid Plus. RESULTS: The placebo group showed no statistically significant differences in the studied parameters; instead, in the Armolipid Plus group, statistically significant reduction differences were detected in BMI (p = 0.010), LDL score (p = 0.035) and an increase in mean LDL particle diameter (p = 0.040). CONCLUSION: The combination of a standard diet with Armolipid Plus is able to reduce LDL score and increase LDL particle diameter in a group of FCHL after 8 weeks of treatment.

Lifestyle educational program strongly increases compliance to nonpharmacologic intervention in hypertensive patients: a 2-year follow-up study.

The authors investigated the efficacy of a lifestyle educational program, organized in small group meetings, in improving the outcome of a nonpharmacologic intervention. One hundred and eighty-eight hypertensive patients with stable blood pressure (BP) levels and drug therapy in the previous 6 months were randomly divided into educational care (EC) and usual care (UC) groups. They were followed at 3-month intervals up to 2 years. In addition to the visits in an outpatient clinic, patients in the EC program participated in small group meetings in order to improve their knowledge of the disease and reinforce their motivation for treatment. At baseline, EC and UC groups were similar for age, sex, body mass index (BMI), blood pressure (BP) levels, and pharmacologic treatment. Patients in the EC group had significantly reduced total energy, total and saturated fats, and sodium intake. Physical activity was significantly increased in the EC group as well. At the end of the 1-year follow-up, BMI (P<.001), visceral fat (P<.001), and BP (P<.001) were significantly lower in the EC group compared with the UC group. Pharmacologic treatment during the study was similar for all classes of drugs apart from diuretics whose dose was higher in the UC group at the end of the study.