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Fabry disease (FD) is an X-linked lysosomal disorder caused by α-galactosidase A enzyme deficiency. Gastrointestinal (GI) manifestations are reported in FD with a prevalence of about 50%, usually treated by Enzymatic Replacement Therapy (ERT) or oral treatment. Since FODMAPs (Fermentable Oligosaccharides, Disaccharides, Monosaccharides, and Polyols) can be involved in GI manifestations and dysbiosis in FD patients, a low-FODMAP diet could represent an alternative adjunctive treatment in FD subjects, as well as being useful for reducing symptoms in Irritable Bowel Syndrome (IBS). We retrospectively assessed data from 36 adult FD patients followed at the Inherited Metabolic Rare Diseases Adult Centre of the University Hospital of Padova (mean age 47.6 ± 16.2 years). Patients were screened for GI symptoms by IBS severity score and Gastrointestinal Symptom Rating Scale (GSRS) questionnaires. In symptomatic patients, the low-FODMAP diet was proposed in order to improve GI manifestations; it consists of a phase of elimination of fermentable saccharides, succeeded by a gradual reintegration of the same. Severe or moderate GI symptoms were found in 61.1% of patients, with no correlation to the therapy in use, and significantly more severe in the classical form of FD. The protocol was completed by seven patients affected by severe GI manifestations, significantly higher than the others. The low-FODMAP diet significantly improved indigestion, diarrhoea, and constipation. This dietetic protocol seemed to have a positive impact on intestinal symptoms, by identifying and reducing the intake of the foods most related to the onset of disorders and improving the clinical manifestations. A low-FODMAP diet may be an effective alternative approach to improve intestinal manifestations and quality of life, and nutrition can play an important role in the multidisciplinary care of patients with FD.

Background: Fatigue is defined as an overwhelming sense of tiredness not responsive to rest or sleep, and it affects every day occupations, social functioning, and work. Patients with inflammatory bowel disease (IBD) report fatigue more frequently than healthy population, but the precise mechanisms underlying its presence are unknown. This study aimed to evaluate the prevalence of fatigue in IBD and its relation with potential causative factors. Methods: A survey consisting of different questionnaires that aimed to evaluate fatigue, depression, anxiety, sleep disorders, and the presence of sarcopenia and malnutrition, was sent by email to 244 IBD outpatients of the Gastroenterology Unit of Academic Hospital of Padua . Demographics and clinical data, including the levels of fecal calprotectin (FC) and C-reactive protein (CRP), and current pharmacological treatments were obtained from patients’ medical records. Results: Ninety-nine (40.5%) subjects answered the survey. Ninety-two (92.9%) patients reported fatigue, with sixty-six having mild to moderate fatigue and twenty-six severe fatigue. After univariate analysis, subjects reporting severe fatigue were more likely to have abnormal values of CRP (OR 3.6) and to demonstrate scores suggesting the presence of sarcopenia, malnutrition, anxiety, depression, compromised sleep quality, and lower quality of life scores. Multivariate analysis showed that abnormal values of CRP (OR 5.1), severe anxiety (OR 3.7) and sarcopenia (OR 4.4) were the factors independently associated with severe fatigue. Conclusion: Fatigue has a high prevalence in subject affected by IBD. Subjects with altered CRP, sarcopenia and severe anxiety appear more at risk of severe fatigue.

An adequate protein intake prevents the loss of fat-free mass during weight loss. Laparoscopic sleeve gastrectomy (SG) jeopardizes protein intake due to post-operative dietary restriction and intolerance to protein-rich foods. The purpose of this study is to evaluate protein intake in the first three months after SG. We evaluated, 1 month and 3 months after surgery, 47 consecutive patients treated with SG. Protein intake, both from foods and from protein supplementation, was assessed through a weekly dietary record. Patients consumed 30.0 ± 10.2 g of protein/day on average from foods in the first month, with a significant increase to 34.9 ± 4.8 g of protein/day in the third month (p = 0.003). The use of protein supplementation significantly increased total protein intake to 42.3 ± 15.9 g protein/day (p &lt, 0.001) in the first month and to 39.6 ± 14.2 g of protein/day (p = 0.002) in the third one. Compliance with supplement consumption was 63.8% in the first month and only 21.3% in the third month. In conclusion, both one and three months after SG, protein intake from foods was not sufficient. The use of modular supplements seems to have a significant impact on protein intake, but nevertheless it remains lower than recommended.

BackgroundLittle evidence is available about the impact of diet on disease activity of axial spondyloarthritis (axSpA). This study evaluated the impact of a 6-month nutritional advice based on the Mediterranean diet on the disease activity of axSpA.MethodsWe prospectively collected the information of a group of axSpA patients who were offered nutritional advice for a 6-month period, who were compared to axSpA patients followed at the same center who were not on a specific diet. A nutritionist gave suggestions for dietary modification at baseline and thereafter every 2 months until month 6. Adherence to the Mediterranean diet was evaluated with the PREDIMED questionnaire ranging from 0 (no adherence) to 10 (optimal adherence); disease activity was evaluated with ASDAS-CRP. A multivariable regression analysis was conducted to identify independent predictors of PREDIMED and of ASDAS-CRP improvement (improvement ≥ 20% of each score).ResultsA total of 161 patients were included: 81 receiving nutritional advice and 80 controls; 47 in the nutritional group and 63 controls had complete information until month 6. Overall, 40 (36.4%) were females, the mean age was 51.7 ± 1.3 years, and 58 (52.7%) were affected with psoriasis. No relevant change of anthropometric or laboratory measures was observed in either group. Adherence to the Mediterranean diet was moderate (PREDIMED score 6.7 ± 1.8 at baseline; 7.6 ± 2.1 at month 6) and improved more in the nutritional group compared to controls (p= 0.020). Predictors of a PREDIMED improvement ≥ 20% were receiving nutritional advice (OR 4.53, 1.36–15.1,p= 0.014), age (per 10-year increase OR 1.05, 1.02–1.68,p= 0.007), and BMI (OR 0.77, 0.63–0.9,p= 0.006). An ASDAS-CRP improvement ≥ 20% was more frequent in the nutritional group compared to controls (p= 0.020). A PREDIMED improvement ≥ 20% was associated with a ASDAS-CRP improvement ≥ 20% (OR 6.75,1.8–25.3,p= 0.005). Psoriasis and disease duration were negatively but not significantly associated to the ASDAS-CRP improvement.ConclusionsImproving adherence to the Mediterranean diet may have a beneficial impact on the activity of axSpA. Patients with a lower BMI and older patients are more prone to modify their diet towards the Mediterranean diet following nutritional advice. Patients with psoriasis may have a limited benefit from dietary improvement.Study registrationProtocol No. 52723, Padova Hospital Medical Ethical Committee (October 11, 2010).

Assessment of heart rate variability (HRV) and cardiac ectopic beats is a clinically relevant topic. The present exploratory observational study aimed to inspect the relationships of lifestyle, dietary patterns, and anthropometrics with HRV, premature ventricular complexes (PVCs), and supraventricular premature complexes (SVPCs). A cross-sectional study enrolling subjects undergoing Holter monitoring was performed. Sociodemographic and clinical characteristics, body composition (full-body bio-impedentiometry), dietary patterns (validated food frequency questionnaire and 24 h dietary recall), and quality of life were assessed. Generalized additive models were estimated to evaluate the relationships between outcomes of interest and variables collected. The study enrolled 121 consecutive patients undergoing 24 h Holter monitoring. Upon univariable analysis, HRV was found to have an inverse association with mass of body fat (MBF) (p-value 0.015), while doing physical activity was associated with a significantly higher HRV (p-value 0.036). Upon multivariable analysis, fruit consumption in the 24 h dietary recall was found to be directly associated with HRV (p-value 0.044). The present findings might be useful for improving the management of patients attending cardiac rhythm labs, and to tailor ad hoc prevention strategies (modification of lifestyle and eating habits) based on Holter parameters.

Low-protein diets (LPDs) are the mainstream treatment for inborn errors of intermediary protein metabolism (IEIPM), but dietary management differs worldwide. Most studies have investigated pediatric populations and their goals such as growth and metabolic balance, showing a tendency toward increasing overweight and obesity. Only a few studies have examined nutritional status and dietary intake of adult IEIPM patients on LPDs. We assessed nutritional parameters (dietary intake using a 7-day food diary record, body composition by bioimpedance analysis, and biochemical serum values) in a group of 18 adult patients with urea cycle disorders (UCDs) and branched chain organic acidemia (BCOA). Mean total protein intake was 0.61 ± 0.2 g/kg/day (73.5% of WHO Safe Levels) and mean natural protein (PN) intake was 0.54 ± 0.2 g/kg/day; 33.3% of patients consumed amino acid (AA) supplements. A totally of 39% of individuals presented a body mass index (BMI) > 25 kg/m2 and patients on AA supplements had a mean BMI indicative of overweight. All patients reported low physical activity levels. Total energy intake was 24.2 ± 5 kcal/kg/day, representing 72.1% of mean total energy expenditure estimated by predictive formulas. The protein energy ratio (P:E) was, on average, 2.22 g/100 kcal/day. Plasmatic levels of albumin, amino acids, and lipid profiles exhibited normal ranges. Phase angle (PA) was, on average, 6.0° ± 0.9°. Fat mass percentage (FM%) was 22% ± 9% in men and 36% ± 4% in women. FM% was inversely and significantly related to total and natural protein intake. Data from IEIPM adults on LPDs confirmed the pediatric trend of increasing overweight and obesity despite a low energy intake. A low protein intake may contribute to an increased fat mass. Nutritional parameters and a healthy lifestyle should be routinely assessed in order to optimize nutritional status and possibly reduce risk of cardiovascular degenerative diseases in adult UCD and BCOA patients on LPDs.

The aim of the study was to investigate whether women affected by unexplained infertility may have undiagnosed dietary imbalances which negatively affect fertility. Secondarily, we investigated whether varying degrees of nutritional abnormalities may benefit from different periconceptional dietary supplementations, evaluating the most effective intervention in improving pregnancy rate after in vitro fertilization (IVF). We conducted a survey on 2 cohorts of patients (group A: unexplained infertility and group B: healthy first trimester spontaneous pregnancies) with the scope of investigating and comparing their dietary status discriminating women without dietary abnormalities (cohort 1) from those with abnormalities exclusively in micronutrient intake (cohort 2) or combined abnormalities in both micronutrient and macronutrient intake and associated obesity (cohort 3). All women included in group A were offered the opportunity to receive a prescription for one of the 3 designated daily dietary supplementation schemes (subgroups A1, A2, and A3) which were to be implemented in the 3 months immediately prior to beginning IVF treatment. When compared with fertile women, patients having unexplained infertility showed significant abnormalities in dietary habits. These differences ranged from a minimal imbalance in micronutrient intake (potentially avoidable with dietary supplementation) to severe combined macronutrient and micronutrient imbalance frequently associated with obesity (partially amendable by inositol supplementation and frequently requiring long-term dietary reeducation before establishment of fertility). Nutritional investigation and treatment may explain and resolve a portion of cases of unexplained infertility, improving the outcome of IVF treatment and, with minimal imbalances, likely restore spontaneous fertility.

Lipid profile could be modified by Mediterranean diet (MD) and by red yeast rice (RYR). We assessed the lipid-lowering effects of MD alone or in combination with RYR on dyslipidemic statin-intolerant subjects, with or without type 2 diabetes, for 24 weeks. We evaluated the low-density lipoprotein (LDL) cholesterol level, total cholesterol (TC), high-density lipoprotein (HDL) cholesterol, triglyceride, liver enzyme, and creatinine phosphokinase (CPK) levels. We studied 171 patients: 46 type 2 diabetic patients treated with MD alone (Group 1), 44 type 2 diabetic patients treated with MD associated with RYR (Group 2), 38 dyslipidemic patients treated with MD alone (Group 3), and 43 dyslipidemic patients treated with MD plus RYR (Group 4). The mean percentage changes in LDL cholesterol from the baseline were-7.34±3.14% (P<0.05) for Group 1;-21.02±1.63% (P<0.001) for Group 2;-12.47±1.75% (P<0.001) for Group 3; and-22±2.19% (P<0.001) for Group 4 with significant intergroup difference (Group 1 versus Group 2,P<0.001; Group 3 versus Group 4,P>0.05). No significant increase in AST, ALT, and CPK levels was observed in all groups. Our results indicate that MD alone is effective in reducing LDL cholesterol levels in statin-intolerant patients with a presumably low cardiovascular risk, but associating MD with the administration of RYR improves patients’ LDL cholesterol levels more, and in patients with type 2 diabetes.

Background: Chronic intensive exercise is associated with a greater induction of oxidative stress and with an excess of endogenous advanced glycation end-products (AGEs). Curcumin can reduce the accumulation of AGEs in vitro and in animal models. We examined whether supplementation with curcumin and Boswellia serrata (BSE) gum resin for 3 months could affect plasma levels of markers of oxidative stress, inflammation, and glycation in healthy master cyclists. Methods. Forty-seven healthy male athletes were randomly assigned to Group 1, consisting of 22 subjects given a Mediterranean diet (MD) alone (MD group), and Group 2 consisted of 25 subjects given a MD plus curcumin and BSE (curcumin/BSE group). Interleukin-6 (IL-6), tumor necrosis factor-α (TNFα), high-sensitivity c-reactive protein (hs-CRP), total AGE, soluble receptor for AGE (sRAGE), malondialdehyde (MDA), plasma phospholipid fatty acid (PPFA) composition, and non-esterified fatty acids (NEFA) were tested at baseline and after 12 weeks. Results: sRAGE, NEFA, and MDA decreased significantly in both groups, while only the curcumin/BSE group showed a significant decline in total AGE. Only the changes in total AGE and MDA differed significantly between the curcumin/BSE and MD groups. Conclusions. Our data suggest a positive effect of supplementation with curcumin and BSE on glycoxidation and lipid peroxidation in chronically exercising master athletes.

Background. Medical nutritional therapy is the most important method for normalizing glucose levels in pregnancy. In this setting, there is a new problem to consider relating to migrants, their personal food preferences, and ethnic, cultural, and religious aspects of their diet. We compared maternal and fetal outcomes between two multiethnic groups of pregnant women, one adopting a food plan that included dishes typical of the foreign women's original countries (the “ethnic meal plan” group), while the other group adopted a standard meal plan.Findings. To develop the meal plan, each dish chosen by the women was broken down into its principal ingredients. The quantity of each food was given in tablespoons, teaspoons, slices, and cups, and there were photographs of the complete dish. The group treated with the ethnic meal plan achieved a better metabolic control at the end of the pregnancy and a lower weight gain (though the difference was not statistically significant). As for fetal outcome, the group on the ethnic meal plan had babies with a lower birth weight and there were no cases of macrosomia or LGA babies.Conclusions. This preliminary study indicates the positive effect of an ethnic approach to diet on the outcome of pregnancy.