Gastrointestinal Manifestations and Low-FODMAP Protocol in a Cohort of Fabry Disease Adult Patients.

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.

Regulatory and Educational Initiatives to Prevent Food Choking Injuries in Children: An Overview of the Current Approaches.

Choking injuries are one of the major causes of death among children ages 0-3, and most of these injuries are related to food. This work provides an overview of the current recommendations for food choking prevention and educational targets as a basis for developing a unified common set of knowledge for primary prevention policies development. Guidelines published by professional membership organizations and national governments in the English language were considered. All of these guidelines provide lists of hazardous food items and recommendations for food preparation to minimize choking hazard. Together with recommendations for food preparation, also recommendations aimed at stakeholders (food manufacturers, health care providers, and public authorities) are provided, underlining that this severe public health problem should be further addressed by adopting integrated public health interventions. Our overview stressed the importance of developing educational and primary prevention policies to sensitize adult supervisors and to regulate dangerous food products in the market.

Assessment of Protein Intake in the First Three Months after Sleeve Gastrectomy in Patients with Severe Obesity.

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 < 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.

Curcumin and Boswellia serrata Modulate the Glyco-Oxidative Status and Lipo-Oxidation in Master Athletes.

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.