A comprehensive review of healthy effects of vegetarian diets.

AIMS: A comprehensive review comparing the effect of vegetarian (V) and non-vegetarian (NV) diets on the major cardiometabolic diseases' outcomes was performed. DATA SYNTHESIS: We performed literature research (up to December 31, 2022) of the evidence separately for vascular disease (VD), obesity (OB), dyslipidemia (Dysl), hypertension (HPT), type 2 diabetes (T2D), metabolic syndrome (MetS), analyzing only cohort studies and randomized controlled studies (RCTs) and comparing the effect of V and NV diets. Cohort studies showed advantages of V diets compared to NV diets on incidence and/or mortality risk for ischemic heart disease, overweight and OB risk. Most cohort studies showed V had lower risk of HPT and lower blood pressure (BP) than NV and V diets had positive effects on T2D risk or plasma parameters. The few cohort studies on the risk of MetS reported mixed results. In RCTs, V diets, mainly low-fat-vegan ones, led to greater weight loss and improved glycemic control than NV diets and in the only one RCT a partial regression of coronary atherosclerosis. In most RCTs, V diets significantly reduced LDL-C levels (but also decreased HDL-C levels) and BP. CONCLUSIONS: In this comprehensive review of the association between V diets and cardiometabolic outcomes, we found that following this type of diet may help to prevent most of these diseases. However, the non-uniformity of the studies, due to ethnic, cultural, and methodological differences, does not allow for generalizing the present results and drawing definitive conclusions. Further, well-designed studies are warranted to confirm the consistency of our conclusions.

Evaluation of nutritional quality of biscuits and sweet snacks sold on the Italian market: the Food Labelling of Italian Products (FLIP) study.

OBJECTIVE: The present study aimed at surveying the nutritional quality of prepacked biscuits and sweet snacks sold on the Italian market, and at identifying whether the product type and other information reported on the pack could discriminate the overall quality of products analysed. DESIGN: Data on energy, nutrient and salt content of the products from two different categories of prepacked sweet cereal products (i.e. biscuits and sweet snacks) were collected from thirteen retailers present on the Italian market. Based on the product type, nutrition and health claim (NHC) and gluten-free (GF) declaration, a comparison of nutrient profile within each category was performed. SETTING: This work is part of the Food Labelling of Italian Products (FLIP) study that aims at systematically investigating the overall quality of the prepacked foods sold on the Italian market. RESULTS: A total of 1290 products were analysed (63 % biscuits and 37 % sweet snacks). After comparing different product types within each category, a high intra-type product variability was evidenced, which was more pronounced for biscuits. Overall, NHC-carrying products seemed to have a better nutrition profile than those without claims, except for salt content. Conversely, a comparison between GF and gluten-containing products did not show consistent results within the two categories analysed. CONCLUSIONS: Due to the high intra-type variability within each category, the different characteristics and regulated information reported on the pack do not seem to be a clear marker of the overall nutritional quality of biscuits and snacks.

Are the dietary habits of treated individuals with celiac disease adherent to a Mediterranean diet?

BACKGROUND AND AIMS: The only treatment for celiac disease (CD) is strict, lifelong adherence to a gluten-free (GF) diet. To date, there are contrasting data concerning the nutritional adequacy of GF products and diet. There have been no studies that have assessed the adherence of individuals with CD to a Mediterranean diet (MD), a protective dietary regimen against major non-communicable diseases (NCDs). Therefore, we examined the adherence to an MD of a group of Italian individuals with CD and compared it with that of a healthy control group. METHODS AND RESULTS: In a cross-sectional study, a sample of individuals with CD and a group of healthy subjects were included. The dietary habits of all participants were recorded using a validated food frequency questionnaire, and the adherence to an MD was determined using the Italian Mediterranean Index. Typical Mediterranean food consumption was not significantly different between individuals with CD and the healthy participants, except for fruits (P = 0.017). However, individuals with CD consumed significantly higher amounts of potatoes (P = 0.003) and red and processed meat (P = 0.005) than healthy participants. The resulting mean Italian Mediterranean Index was significantly higher in healthy participants than in individuals with CD (P < 0.001). CONCLUSION: The results raise questions concerning the food choices of individuals with CD, suggesting the need of encouraging them to make better food choices more in line with an MD, which would improve their nutritional status and better protect them from NCDs at long term. PROTOCOL REGISTRATION: ClinicalTrials.gov (ID NCT01975155) on November 4 2013.

Position paper on vegetarian diets from the working group of the Italian Society of Human Nutrition.

BACKGROUND: Interest in vegetarian diets is growing in Italy and elsewhere, as government agencies and health/nutrition organizations are emphasizing that regular consumption of plant foods may provide health benefits and help prevent certain diseases. METHODS AND RESULTS: We conducted a Pubmed search, up to September, 2015, for studies on key nutrients (proteins, vitamin B12, iron, zinc, calcium, vitamin D, and n-3 fatty acids) in vegetarian diets. From 295 eligible publications the following emerged: Vegetarians should be encouraged to supplement their diets with a reliable source of vitamin B12 (vitamin-fortified foods or supplements). Since the plant protein digestibility is lower than that of animal proteins it may be appropriate for vegetarians to consume more proteins than recommended for the general population. Vegetarians should also be encouraged to habitually consume good sources of calcium, iron and zinc - particularly vegetables that are low in oxalate and phytate (e.g. Brassicaceae), nuts and seeds, and calcium-rich mineral water. Calcium, iron, and zinc bioavailability can be improved by soaking, germination, and sour-dough leavening that lower the phytate content of pulses and cereals. Vegetarians can ensure good n-3 fatty acid status by habitually consuming good sources of a-linolenic acid (walnuts, flaxseeds, chia seeds, and their oils) and limiting linoleic acid intake (corn and sunflower oils). CONCLUSIONS: Well-planned vegetarian diets that include a wide variety of plant foods, and a reliable source of vitamin B12, provide adequate nutrient intake. Government agencies and health/nutrition organizations should provide more educational resources to help Italians consume nutritionally adequate vegetarian diets.

Dietary glycemic index, glycemic load, and cancer risk: results from the EPIC-Italy study.

Factors linked to glucose metabolism are involved in the etiology of several cancers. High glycemic index (GI) or high glycemic load (GL) diets, which chronically raise postprandial blood glucose, may increase cancer risk by affecting insulin-like growth factor. We prospectively investigated cancer risk and dietary GI/GL in the EPIC-Italy cohort. After a median 14.9 years, 5112 incident cancers and 2460 deaths were identified among 45,148 recruited adults. High GI was associated with increased risk of colon and bladder cancer. High GL was associated with: increased risk of colon cancer; increased risk of diabetes-related cancers; and decreased risk of rectal cancer. High intake of carbohydrate from high GI foods was significantly associated with increased risk of colon and diabetes-related cancers, but decreased risk of stomach cancer; whereas high intake of carbohydrates from low GI foods was associated with reduced colon cancer risk. In a Mediterranean population with high and varied carbohydrate intake, carbohydrates that strongly raise postprandial blood glucose may increase colon and bladder cancer risk, while the quantity of carbohydrate consumed may be involved in diabetes-related cancers. Further studies are needed to confirm the opposing effects of high dietary GL on risks of colon and rectal cancers.

Dietary glycemic index, glycemic load and metabolic profile in children with phenylketonuria.

BACKGROUND AND AIMS: No data exist in the current literature on the glycemic index (GI) and glycemic load (GL) of the diet of phenylketonuric (PKU) children. The aims of this study were to examine the dietary GI and GL in PKU children on a low-phenylalanine (Phe)-diet and to evaluate whether an association may exist between the carbohydrate quality and the metabolic profile. METHODS: Twenty-one PKU children (age 5-11 years) and 21 healthy children, gender and age matched, were enrolled. Dietary (including GI and GL) and blood biochemical assessments were performed. RESULTS: No difference was observed for daily energy intake between PKU and healthy children. Compared to healthy controls, PKU children consumed less protein (p = 0.001) and fat (p = 0.028), and more carbohydrate (% of total energy, p = 0.004) and fiber (p = 0.009). PKU children had higher daily GI than healthy children (mean difference (95% confidence interval), 13.7 (9.3-18.3)) and higher GL (31.7 (10.1-53.2)). PKU children exhibited lower blood total and low density lipoprotein cholesterol (LDL) levels (p < 0.01) and higher triglyceride level (p = 0.014) than healthy children, while glucose and insulin concentrations did not differ. In PKU children the dietary GL was associated with triglyceride glucose index (Spearman's correlation coefficient = 0.515, p = 0.034). CONCLUSION: In PKU children a relationship of the dietary treatment with GI and GL, blood triglycerides and triglyceride glucose index may exist. Improvement towards an optimal diet for PKU children could include additional attention to the management of dietary carbohydrate quality.

Knowledge about sources of dietary fibres and health effects using a validated scale: a cross-country study.

OBJECTIVES: Dietary fibre (DF) is one of the components of diet that strongly contributes to health improvements, particularly on the gastrointestinal system. Hence, this work intended to evaluate the relations between some sociodemographic variables such as age, gender, level of education, living environment or country on the levels of knowledge about dietary fibre (KADF), its sources and its effects on human health, using a validated scale. STUDY DESIGN: The present study was a cross-sectional study. METHODS: A methodological study was conducted with 6010 participants, residing in 10 countries from different continents (Europe, America, Africa). The instrument was a questionnaire of self-response, aimed at collecting information on knowledge about food fibres. The instrument was used to validate a scale (KADF) which model was used in the present work to identify the best predictors of knowledge. The statistical tools used were as follows: basic descriptive statistics, decision trees, inferential analysis (t-test for independent samples with Levene test and one-way ANOVA with multiple comparisons post hoc tests). RESULTS: The results showed that the best predictor for the three types of knowledge evaluated (about DF, about its sources and about its effects on human health) was always the country, meaning that the social, cultural and/or political conditions greatly determine the level of knowledge. On the other hand, the tests also showed that statistically significant differences were encountered regarding the three types of knowledge for all sociodemographic variables evaluated: age, gender, level of education, living environment and country. CONCLUSIONS: The results showed that to improve the level of knowledge the actions planned should not be delineated in general as to reach all sectors of the populations, and that in addressing different people, different methodologies must be designed so as to provide an effective health education.

Knowledge about dietary fibres (KADF): development and validation of an evaluation instrument through structural equation modelling (SEM).

OBJECTIVES: Because there is scientific evidence that an appropriate intake of dietary fibre should be part of a healthy diet, given its importance in promoting health, the present study aimed to develop and validate an instrument to evaluate the knowledge of the general population about dietary fibres. STUDY DESIGN: The present study was a cross sectional study. METHODS: The methodological study of psychometric validation was conducted with 6010 participants, residing in 10 countries from three continents. The instrument is a questionnaire of self-response, aimed at collecting information on knowledge about food fibres. Exploratory factor analysis (EFA) was chosen as the analysis of the main components using varimax orthogonal rotation and eigenvalues greater than 1. In confirmatory factor analysis by structural equation modelling (SEM) was considered the covariance matrix and adopted the maximum likelihood estimation algorithm for parameter estimation. RESULTS: Exploratory factor analysis retained two factors. The first was called dietary fibre and promotion of health (DFPH) and included seven questions that explained 33.94% of total variance (α = 0.852). The second was named sources of dietary fibre (SDF) and included four questions that explained 22.46% of total variance (α = 0.786). The model was tested by SEM giving a final solution with four questions in each factor. This model showed a very good fit in practically all the indexes considered, except for the ratio χ(2)/df. The values of average variance extracted (0.458 and 0.483) demonstrate the existence of convergent validity; the results also prove the existence of discriminant validity of the factors (r(2) = 0.028) and finally good internal consistency was confirmed by the values of composite reliability (0.854 and 0.787). CONCLUSIONS: This study allowed validating the KADF scale, increasing the degree of confidence in the information obtained through this instrument in this and in future studies.

Glycemic index and glycemic load of commercial Italian foods.

BACKGROUND AND AIM: The glycemic index (GI) and glycemic load (GL) are useful parameters in the nutritional classification of carbohydrate foods. Diets characterized by a low GI and/or a low GL have been repeatedly and independently associated with decreased risk of diabetes and other chronic diseases. The aim of this study is to report the GI and GL value of carbohydrate-rich foods available on the Italian market and mostly consumed in Italy. METHODS AND RESULTS: GI values were determined according to FAO/WHO (1997) and ISO (2010). Overall, the 141 commercial foods that were analyzed represent food categories that are the source of >80% carbohydrate intake in Italy. The food items chosen were based mainly on the market share of the brand within each food category and grouped into 13 food categories: 1) beverages: fermented milk drink, juice, smoothie, soft drink; 2) biscuits; 3) breads; 4) bread substitutes; 5) breakfast cereals; 6) cakes and snacks; 7) candy and confectionery; 8) cereals; 9) desserts and ice-creams; 10) marmalade and jam; 11) pasta; 12) pizza; 13) sugar and sweetener. CONCLUSION: This database of commercial Italian foods partly overcomes the lack of information on GI and GL of local foods, contributing to a better understanding of the association between GI/GL and health and providing a more informed choice to Italian consumers and health practitioners.

Food selection based on high total antioxidant capacity improves endothelial function in a low cardiovascular risk population.

BACKGROUND AND AIMS: Oxidative stress has been advocated as a major cause for cardiovascular disease (CVD), and low plasma antioxidant concentrations are associated with endothelial dysfunction, the first step towards atherosclerosis. However, although the antioxidant content in fruits and vegetables may explain at least in part their protective effect against CVD, supplementation with antioxidant vitamins fails to improve endothelial function and reduce CVD risk. The aim of this study was to investigate the impact of a diet rich in antioxidants on endothelial function measured by flow-mediated dilatation (FMD) in volunteers at low cardiovascular risk. METHODS AND RESULTS: In a crossover trial, 24 subjects (13 women, mean age 61 ± 3 years), received, in a randomised order, a 14-day high (HT) and a 14-day low (LT) antioxidant diets, with a 2-week wash-out (WO) in between. Both diets were comparable in daily portions of fruits and vegetables, and in alcohol, fibre and macronutrient intake, but differed in their total antioxidant capacity. Before and after each diet, anthropometrics, blood pressure, fasting plasma glucose, lipid profile, hepatic enzymes, circulating antioxidant concentrations, high sensitivity C-reactive protein (hs-CRP) and FMD were assessed. FMD increased significantly during the HT diet compared to the LT (p < 0.000). FMD values were 2.3% higher after HT compared with LT (p < 0.001) after adjustment for age, gender and diet order. α-tocopherol increased significantly (p < 0.05) and hs-CRP and of γ-glutamyltranspeptidase decreased significantly (p < 0.05 and p < 0.01, respectively) during the HT diet, compared with the LT diet. CONCLUSIONS: A short-term HT diet improves endothelial function in volunteers at low cardiovascular risk, which may further reduce their risk of CVD.

Total dietary antioxidant capacity and lung function in an Italian population: a favorable role in premenopausal/never smoker women.

BACKGROUND/OBJECTIVES: Antioxidant-rich foods may favorably influence lung function. We examined possible associations between the total dietary antioxidant capacity (TAC) and pulmonary function in a healthy Italian population. SUBJECTS/METHODS: Until May 2009, 22,300 persons were randomly recruited from the general population in the Moli-sani project. A sample only including healthy women (5824) and men (5848) was analyzed. TAC was measured in foods by three different assays and the ferric reducing-antioxidant power (FRAP) assay was selected as the better indicator of dietary TAC. The European Investigation into Cancer and Nutrition Food Frequency Questionnaire was used for dietary assessment. The association between quintiles of dietary FRAP and pulmonary indexes was assessed using analysis of variance separately for men and women. RESULTS: After adjustment for confounders, women in the highest quintile of FRAP intake had +39 ml forced expiratory volume in the first second (FEV(1)) and +54 ml forced vital capacity, compared with those in the lowest quintile (P for trend ≤0.006). Stratified analysis showed that this relationship only occurred in women who were premenopausal/never smokers. In this subgroup, the observed effect of higher FRAP intake on FEV(1) was equivalent to an improvement in pulmonary age of 3.3 years. In men, all significant associations between pulmonary function and TAC were lost after adjustment for confounding. CONCLUSIONS: Dietary TAC may have a favorable role in respiratory health, particularly in premenopausal/never smoker women.

The effect of breakfasts varying in glycemic index and glycemic load on dietary induced thermogenesis and respiratory quotient.

BACKGROUND AND AIM: Glycemic index (GI) and Glycemic Load (GL) are parameters of carbohydrate bioavailability able to influence risk of chronic diseases. GL can be lowered either by reducing carbohydrate intake or by reducing the GI of the carbohydrate moiety of a mixed meal. These two approaches might have a different impact on Dietary-Induced Thermogenesis (DIT) and preferential substrate oxidation in the postprandial period, which are variables known to be involved in the regulation of body weight and body composition. This dietary, crossover intervention trial was designed to evaluate the effect on DIT and Respiratory Quotient (RQ) of three isocaloric breakfasts different in GI and/or GL (high GI and high GL [HGI-HGL] vs. low GI and low GL [LGI-LGL]; vs. high GI and low GL [HGI-LGL]) followed by a standard meal. METHODS AND RESULTS: RQ and DIT were measured in 16 lean young males by indirect calorimetry for 8h. DIT resulted significantly higher after the LGI-LGL compared to the HGI-HGL breakfast (p<0.05). Postprandial changes in RQ differed among all breakfasts (p<0.001). RQ increased from baseline after the two breakfasts with highest carbohydrate content and significantly more after the HGI-HGL than after the LGI-LGL (p<0.02), whereas it decreased after the HGI-LGL breakfast, which contained a higher amount of fat. CONCLUSIONS: Reducing the GL of a meal by reducing GI seems an effective strategy to increase energy expenditure while maintaining a good rate of lipid oxidation. This might be related to different profiles of postprandial hormones affecting substrate oxidation.

[Enzyme replacement therapy of lysosomal storage diseases]. / Thérapies enzymatiques substitutives des maladies lysosomales.

Extraction and purification of an acid ß-glucosidase from human placenta (alglucerase) for the treatment of Gaucher disease, replaced a few years later by a recombinant enzyme (imiglucérase, Cerezyme(®)), has paved the way to the development of enzyme replacement therapies (ERT) for the treatment of lysosomal storage diseases (LSD) among which Fabry disease for which the long-term efficacy of the two currently available preparations (agalsidase alfa, Replagal(®) and Fabrazyme(®)) is still being investigated. Mucopolysaccharidosis (MPS) type I (Hurler and Scheie diseases), II (Hunter syndrome) and VI (Maroteaux-Lamy disease) also benefit from ERT using laronidase (Aldurazyme(®)), idursulfase (Elaprase(®)) and galsulfase (Naglazyme(®)), respectively. ERT reduces the hepatosplenomegaly and improves the physical and respiratory capacities of MPS patients with a globally acceptable safety profile although the possibility of infusion-associated should always be kept in mind. Alglucosidase alpha (Myozyme(®)) improves the cardiomyopathy and life expectancy of infants suffering from Pompe disease and is under evaluation for the treatment of the juvenile and adult forms of the disease. CNS involvement remains a major challenge for many LSD and innovative research and approaches are needed to address the fact that recombinant enzymes do not cross the blood-brain barrier and therefore are not expected to lead to any improvement in CNS damages, except if alternative routes such as intrathecal administration would be developed. Molecular chaperones (e.g. migalastat for Fabry disease) and inhibitors of glucosylceramide synthesis (e.g. eliglustat tartrate for Gaucher disease) are currently under investigation in various clinical trials.

Intake of the plant lignans matairesinol, secoisolariciresinol, pinoresinol, and lariciresinol in relation to vascular inflammation and endothelial dysfunction in middle age-elderly men and post-menopausal women living in Northern Italy.

BACKGROUND AND AIMS: It has been suggested that lignan intake may decrease the risk for cardiovascular disease (CVD) by modifying traditional risk factors as well as aortic stiffness. However, the role of dietary lignans on the vascular system is largely unknown. The objective was to investigate whether dietary intake of plant lignans in a free-living population was associated with markers of vascular inflammation and function. METHODS AND RESULTS: We performed a cross-sectional study in 242 (151 males) men and post-menopausal women. Anthropometric characteristics and lignan intake were evaluated. Soluble intercellular adhesion molecule-1 (sICAM-1), insulin, high-sensitive C-reactive protein, glucose, total cholesterol, HDL-cholesterol and triacylglycerols were measured in fasting blood samples. Brachial flow-mediated dilation (FMD) measurements were available for 101 subjects (56 males). Median (interquartile range) daily intake of matairesinol (MAT), secoisolariciresinol (SECO), pinoresinol (PINO), lariciresinol (LARI), and total lignans was 20.9 microg (17.4), 335.3 microg (289.1), 96.7 microg (91.1), 175.7 microg (135.8), and 665.5 microg (413.7), respectively, as assessed by 3-day weighed food record. Plasma concentrations of sICAM-1 (whole sample) significantly decreased (mean (95%CI) = 358 microg/L (320-401), 276 microg/L (252-303), 298 microg/L (271-326), and 269 microg/L (239-303), P per trend 0.013) and FMD values (FMD sub-group) significantly increased (4.1% (2.2-6.0), 5.7% (4.3-7.2), 6.4% (4.9-7.8), and 8.1% (6.3-10.0), P per trend 0.016) across quartiles of energy-adjusted MAT intake, even after adjustment for relevant clinical and dietary variables. Intake of SECO was also inversely related to plasma sICAM-1 (P per trend 0.018), but not to FMD values. No relationship between intake of PINO, LARI or total lignans and either sICAM-1 or FMD values was observed. CONCLUSIONS: Higher MAT intakes in the context of a typical Northern Italian diet are associated to lower vascular inflammation and endothelial dysfunction, which could have some implications in CVD prevention.

Efficacy and tolerance of gastrostomy feeding in Duchenne muscular dystrophy.

Undernutrition occurs often in individuals with Duchenne muscular dystrophy (DMD). Between 1997 and 2007, a gastrostomy was placed in 25 patients with DMD (median: 23 years old; range, 11-38 years) for weight loss (n=22) and/or swallowing disorders (n=13). We evaluated nutritional status using the weight-for-age (W/A) ratio, comparing the values to the reference curve for DMD patients. During the first 9 months, nutritional status improved: the W/A ratio increased and reached a plateau. The W/A ratio was 69% (range, 45-128%) at the start and increased to 87% (range, 49-164%) at the maximal follow-up of 22 months (P<0.001). However, the W/A ratio did not reach the median value for age. Complications occurred in 21 patients (84%), but caused no mortality. Our data suggest that gastrostomy is well tolerated by, and effective for improving the nutritional status of, individuals with DMD.

Phenotype genotype analysis in 15 patients presenting a congenital myasthenic syndrome due to mutations in DOK7.

Congenital myasthenic syndromes (CMSs) are a heterogeneous group of diseases caused by genetic defects affecting neuromuscular transmission. Mutations of DOK7 have recently been described in recessive forms of CMS. Dok-7 is a cytoplasmic post-synaptic protein co-activator of the muscle-specific receptor-tyrosine kinase (MuSK) involved in neuromuscular synaptogenesis and maintenance. We report clinical, morphological and molecular data on 15 patients with mutations in DOK7. Eleven different mutations (5 novel) were identified and all patients but one were found to carry at least the common c.1124_1127dupTGCC mutation. Patients with DOK7 mutations have a particular limb-girdle pattern, without tubular aggregates but a frequent lipidosis on the muscle biopsy. Changes in pre- and post-synaptic compartments of the neuromuscular junction were also observed in muscle biopsies: terminal axons showed defective branching which resulted in a unique terminal axon contacting en passant postsynaptic cups. Clinical features, muscle biopsy findings or response to therapy were confusing in several patients. Characterization of this distinct phenotype is essential to provide clues for targeted genetic screening and to predict the therapeutic response to anticholinesterase treatments or ephedrine as has been suggested.

Intervention study with a high or low antioxidant capacity diet: effects on circulating beta-carotene.

BACKGROUND AND OBJECTIVES: A cross-sectional observation suggests that total antioxidant capacity (TAC) of the diet positively affects plasma concentrations of beta-carotene independent of beta-carotene intake. This study was carried out to investigate the effect of two dietary strategies, designed to be comparable in fruits, vegetables, fibre, alcohol and beta-carotene intake but substantially different in their TAC, on changes in antioxidant intake and antioxidant status, and in particular in circulating beta-carotene concentrations. SUBJECTS: A randomized cross-over intervention trial involving 33 healthy participants and consisting of two 14-day dietary periods (high TAC diet, HT; low TAC diet, LT) with a 14-day washout in between was conducted. RESULTS: Energy, macronutrient, dietary fibre, alcohol and beta-carotene intake was not significantly different between LT and HT, whereas intake of other carotenoids and dietary TAC was significantly higher in the HT than in the LT (P<0.001). Circulating carotenoids (with the exception of alpha-carotene, which followed an inverse trend) and alpha-tocopherol decreased significantly during the LT and increased during the HT period. Among these, beta-carotene almost doubled its concentration in plasma after the HT diet. CONCLUSIONS: The increase in circulating beta-carotene along with the increase in dietary TAC suggests that plasma beta-carotene could be a marker of TAC intake rather than of beta-carotene intake itself. This may explain, in part, why beta-carotene supplementation alone has shown no benefit in chronic disease prevention and adds to a putative beneficial role of high dietary TAC diets, which merits further investigation.

Dilative arteriopathy and basilar artery dolichoectasia complicating late-onset Pompe disease.

BACKGROUND: Strokes related to intracranial aneurysm or arteriopathy have been reported in a few patients with late-onset Pompe disease. These reports suggested that cerebral vessel involvement could be an underrecognized complication of this disease. METHODS: We report cerebral artery involvement in three French patients with late-onset Pompe disease. RESULTS: The first patient died at age 35 years from complications of a giant fusiform aneurysm of the basilar artery, and her 34-year-old sister showed evidence of dolichoectatic basilar artery on magnetic resonance angiography. A dilative arteriopathy complicated with carotid artery dissection was diagnosed in the third patient, aged 50 years. Two patients are currently being treated with enzyme replacement therapy (alglucosidase alfa), and regular angiographic follow-up showed the absence of progression of vascular abnormalities in one of them. CONCLUSION: These observations, combined with previously reported cases, confirm that Pompe disease should be recognized as a predisposing condition to dilative arteriopathy and cerebral aneurysm formation, although the real incidence of these vascular complications remains unknown.