Search Results (54)

This study reviews the development of the U.S. maple syrup industry, assesses its strengths, weaknesses, opportunities, and threats (SWOT), and derives recommendations for the industry to attain a more sustainable development. While the industry faces the challenges of increasing yield and production volatility, a downward trend in producer prices since 2008, increasing competition from imports, and impacts of trade policies, etc., it needs innovative strategies to turn its weaknesses and threats into strengths and opportunities. Major recommendations, based on a comprehensive review of the industry’s development and trends and a SWOT analysis, include establishing a national or regional producer governance organization, similar to the Quebec Maple Syrup Producers (QMSP) or the American Honey Producers Association, to advocate for maple syrup producers on issues like trade policies, quality standards and certification, environmental regulations, and to enhance maple syrup producers’ market power, increasing the investment and adoption of climate-resilient technologies, developing more value-added maple syrup products according to consumer preferences and demand, and strengthening the marketing and promotion efforts of industrial organizations, government agents and private enterprises through collaboration for the goal of increasing the demand for U.S. maple syrup in the domestic and foreign markets. Full article

Among the species used for syrup production, sugar maple (Acer saccharum Marsh.) is preferred by producers, while red maple (Acer rubrum L.) is considered less productive in terms of sap yield and sugar content. This study aims to measure the volume and physicochemical characteristics of the sap produced from two red maples and two sugar maples during the 2023 sugar season in a commercial sugarbush in Laterrière (QC, Canada). Sap exudation was measured continuously with the gravity method using automatic rain gauges. Sap production was discontinuous and heterogeneous, reaching 2.6 L during the most productive day. No significant difference was found in the daily production between species, but we observed a difference in the cumulative sap production (7 L in red maple vs. 13.5 L in sugar maple) due to a longer period of sap exudation in the latter. Despite daily variations in pH, Brix values, sucrose concentration, osmolality, and conductivity, no differences in physicochemical characteristics were detected between species. Full article

This paper examines a new artisanal food product made from bigleaf maple trees (Acer macrophyllum). These trees are indigenous to, and thrive in, the moist and mild climate of the Pacific Northwestern regions of the United States and Canada. Small producers have a new artisanal product from this historically valuable tree. The sap is collected from family-owned woodland trees to produce maple syrup. To better understand the gastronomic potential of bigleaf maple syrup, we conducted sensory evaluations with culinary professionals. In this study undertaken in Oregon, the syrup from three small independent producers was subjected to sensory hedonic and attribute assessments by 62 chefs. The results of the sensory assessments and the details of the testing methods and analysis are presented. This paper examines the agreement among the chefs and the implications for the culinary acceptance of this new artisanal product. We discuss in detail (1) the complexity of flavor; (2) the most likely use of bigleaf maple syrup in various foods or as a sweetener; (3) and the four most important reasons for purchasing bigleaf maple syrup consisting of both environmental and taste attributes. Full article

Background/Objectives: This study aims to evaluate the efficacy and outcomes of renal replacement therapy (RRT) in pediatric patients with metabolic diseases, specifically focusing on the impact of hemodialysis (HD) and peritoneal dialysis (PD) on clinical parameters, toxin reduction, and long-term survival. Methods: This retrospective study included 10 pediatric patients (eight females and two males) treated at a pediatric nephrology department between 2020 and 2023. Patients diagnosed with metabolic disorders, including maple syrup urine disease (MSUD), methylmalonic acidemia (MMA), and glycogen storage disease (GSD), underwent RRT. Clinical data, demographic information, and biochemical parameters were collected and analyzed. Results: Among the patients, 50% were diagnosed with MSUD, 30% with MMA, and 20% with GSD. RRT, including HD and PD, was administered to manage acute metabolic crises. HD was particularly effective in rapidly reducing toxic metabolite levels. Patients treated with HD showed significant reductions in leucine and ammonium levels, with median reductions of 94.5% and 86%, respectively. Overall, 60% of the patients demonstrated long-term survival, highlighting the critical role of RRT in managing metabolic crises. In conclusion, RRT, including HD and PD, is crucial in managing pediatric metabolic disorders by effectively reducing toxic metabolite levels and improving clinical outcomes. Conclusions: The results of this study are consistent with previous research, highlighting the critical role of RRT in the acute management of metabolic crises and supporting its adoption as a standard treatment method. Full article

Inconclusive and false-positive newborn screening (NBS) results can cause parental stress and increase healthcare expenditures. These results can be reduced by improving NBS algorithms. This was recently done for Congenital Hypothyroidism (CH), Congenital Adrenal Hyperplasia (CAH) and Maple Syrup Urine Disease (MSUD) in the Dutch NBS program. The current study estimates the financial consequences of these improved algorithms related to the reduction in inconclusive results and false-positives. For each improved algorithm, the care pathway of an inconclusive/false-positive result was analyzed. The costs associated with the improvements, based on the change in inconclusive results/false-positives, were assessed to estimate the cost reduction per year. The improvements resulted in a reduction of inconclusive results and/or false-positives, without increasing false-negatives. For CH, false positives decreased by 26 per year with a related cost reduction of EUR 31,156. For CAH, 95 second heel punctures and seven false-positives per year were avoided, leading to a related cost reduction of EUR 7340. For MSUD, five false-positives per year were avoided with a related cost reduction of EUR 11,336. The improved screening algorithms led to a cost reduction of EUR 49,832 annually. Together with the known negative psychosocial effects associated with an inconclusive or false-positive NBS result, these results highlight the importance of improving NBS algorithms. Full article

Despite the widespread empirical adoption of calcium hypochlorite (Ca(ClO)₂) for sanitizing 3/16-inch tubing after the maple sap collection season, there remains a dearth of scientific data on its best practice and effectiveness. To address this gap, we cultivated microbial mass in tubing through continuous maple sap flow at 7 °C for 5 weeks in the lab. The tubing was sanitized with 200, 400, or 600 ppm Ca(ClO)₂ and retained Ca(ClO)₂ for either 10 min, 1 h, 7 days, or 6 weeks. Half of the tubing segments underwent microbial analysis, while the other half were stored for 6 weeks post-flushing of the Ca(ClO)₂ to determine microbial survival/growth. The level and presence of the microbial load were determined, and the inner tubing surfaces were visualized using scanning electron microscopy (SEM). The initial microbial load in the tubing was approximately 4–5 log CFU/cm². A 10-min and 1 h contact time with 200 ppm Ca(ClO)₂, and a 10-min exposure to 400 ppm Ca(ClO)₂, achieved reductions of 2.4–2.8 log for Pseudomonas spp., 1.6–2.5 log for mold and yeast, and 2.3–3.3 log for psychrotrophic microorganisms. Microorganisms were recovered from the enrichment process after retaining 200 ppm Ca(ClO)₂ for 6 weeks, indicating insufficient inactivation. Consequently, the data suggests the use of at least 400 ppm Ca(ClO)₂ for 1 day. The SEM images supported the microbial count results, offering valuable insights for educating maple syrup producers on optimal tubing sanitation practices. Full article

The treatment for Maple Syrup Urine Disease (MSUD) consists of a hypoproteic diet with integration therapy to limit leucine intake, ensuring adequate energy, macronutrients, and micronutrients to prevent catabolism and promote anabolism. We conducted a retrospective cross-sectional study at the Metabolic Rare Disease Unit, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy. Patients with MSUD who were over 3 years old, not treated with liver transplantation, and who provided written consent, were included. The study aimed to describe the dietary treatment of patients with MSUD, evaluate growth data, and analyze the effect of a low-protein and semi-synthetic diet on body composition. Data on height, weight, BMI, waist circumference, food intake, physical activity, and DEXA scans were collected. Thirteen subjects (11 classic MSUD, 2 intermediate MSUD) were included, of which 5 < 18 years old. Results indicated that patients with MSUD follow a balanced diet and have body compositions like healthy subjects in terms of fat and lean mass. A high incidence of osteopenia was observed from a young age, with a positive correlation between protein intake and lean mass and a negative correlation between BCAA-free mixture consumption and bone mineral density z-score. The study highlights the positive effects and potential consequences of the semi-synthetic diet on the body composition of patients with MSUD. A similar study involving all Italian metabolic centers treating MSUD is recommended. Full article

Historically, tree sap has been used globally for medicinal purposes, in fermented beverages, and for syrup production. Maple tree sap is notably concentrated into syrup and is valued as a natural sweetener rich in phenolic compounds and minerals compared to refined sugar. Recently, syrups from other trees like black walnut (Juglans nigra) and sycamore (Platanus occidentalis) have gained popularity, yet their properties are not well understood scientifically. To address this gap, we collected sycamore, black walnut, and maple syrup samples and analyzed their physicochemical and functional properties. Our findings showed significant differences among the syrups in pH, browning intensity, and water activity (p < 0.05). Sycamore syrup had the highest total phenolic content, followed by black walnut and maple syrups. Both black walnut and sycamore syrups exhibited similar antioxidant activity, significantly higher than maple syrup (p < 0.05). High-resolution mass spectrometry identified 54 phenolic acids and 22 flavonoids in these syrups, including Acetylsalicylic acid, 3,5-Dihydroxybenzoic acid, and syringic acid, known for their antioxidant and anti-inflammatory properties. Additionally, sycamore syrups and most black walnut syrups displayed varying degrees of antimicrobial activity against Gram-positive and/or Gram-negative microorganisms. This study offers insights into the properties and potential health benefits of these specialty tree syrups. Full article

Amino acid (AA)-related inherited metabolic disorders (IMDs) and urea cycle disorders (UCDs) require strict dietary management including foods low in protein such as fruits, vegetables and starchy roots. Despite this recommendation, there are limited data on the AA content of many of these foods. The aim of this study is to describe an analysis of the protein and AA content of a range of fruits, vegetables and starchy roots, specifically focusing on amino acids (AAs) relevant to AA-related IMDs such as phenylalanine (Phe), methionine (Met), leucine (Leu), lysine (Lys) and tyrosine (Tyr). AA analysis was performed using high-performance liquid chromatography (HPLC) on 165 food samples. Protein analysis was also carried out using the Dumas method. Foods were classified as either ‘Fruits’, ‘Dried fruits’, ‘Cruciferous vegetables’, ‘Legumes’, ‘Other vegetables’ or ‘Starchy roots’. ‘Dried fruits’ and ‘Legumes’ had the highest median values of protein, while ‘Fruits’ and ‘Cruciferous vegetables’ contained the lowest median results. ‘Legumes’ contained the highest and ‘Fruits’ had the lowest median values for all five AAs. Variations were seen in AA content for individual foods. The results presented in this study provide useful data on the protein and AA content of fruits, vegetables and starchy roots which can be used in clinical practice. This further expansion of the current literature will help to improve diet quality and metabolic control among individuals with AA-related IMDs and UCDs. Full article

Microorganisms have significant potential to control fungal contamination in various foods. However, the identification of strains that exhibit robust antifungal activity poses challenges due to highly context-dependent responses. Therefore, to fully exploit the potential of isolates as antifungal agents, it is crucial to systematically evaluate them in a variety of biotic and abiotic contexts. Here, we present an adaptable and scalable method using a robotic platform to study the properties of 1022 isolates obtained from maple sap. We tested the antifungal activity of isolates alone or in pairs on M17 + lactose (LM17), plate count agar (PCA), and sucrose–allantoin (SALN) culture media against Kluyveromyces lactis, Candida boidinii, and Saccharomyces cerevisiae. Microorganisms exhibited less often antifungal activity on SALN and PCA than LM17, suggesting that the latter is a better screening medium. We also analyzed the results of ecological interactions between pairs. Isolates that showed consistent competitive behaviors were more likely to show antifungal activity than expected by chance. However, co-culture rarely improved antifungal activity. In fact, an interaction-mediated suppression of activity was more prevalent in our dataset. These findings highlight the importance of incorporating both biotic and abiotic factors into systematic screening designs for the bioprospection of microorganisms with environmentally robust antifungal activity. Full article

Currently, tandem mass spectrometry-based newborn screening (NBS), which examines targeted biomarkers, is the first approach used for the early detection of maple syrup urine disease (MSUD) in newborns, followed by confirmatory genetic mutation tests. However, these diagnostic approaches have limitations, demanding the development of additional tools for the diagnosis/screening of MUSD. Recently, untargeted metabolomics has been used to explore metabolic profiling and discover the potential biomarkers/pathways of inherited metabolic diseases. Thus, we aimed to discover a distinctive metabolic profile and biomarkers/pathways for MSUD newborns using untargeted metabolomics. Herein, untargeted metabolomics was used to analyze dried blood spot (DBS) samples from 22 MSUD and 22 healthy control newborns. Our data identified 210 altered endogenous metabolites in MSUD newborns and new potential MSUD biomarkers, particularly L-alloisoleucine, methionine, and lysoPI. In addition, the most impacted pathways in MSUD newborns were the ascorbate and aldarate pathways and pentose and glucuronate interconversions, suggesting that oxidative and detoxification events may occur in early life. Our approach leads to the identification of new potential biomarkers/pathways that could be used for the early diagnosis/screening of MSUD newborns but require further validation studies. Our untargeted metabolomics findings have undoubtedly added new insights to our understanding of the pathogenicity of MSUD, which helps us select the appropriate early treatments for better health outcomes. Full article

The use of antibiotics to promote growth and prevent diarrhea in livestock production has raised concerns about the emergence of antibiotic-resistant bacteria. Probiotics, live microorganisms that confer health benefits, have been proposed as alternatives to antibiotics. In this study, we produced and characterized a downgraded maple syrup-based feed supplement containing Bacillus velezensis FZB42 as a potential synbiotic for animal nutrition. An optimized fermentation medium was developed through a central composite design to produce B. velezensis FZB42 at both the laboratory and pilot scale, reaching a concentration of 6.15 ± 0.46 × 10⁹ CFU/mL. Subsequently, B. velezensis FZB42 was incorporated into a protective whey permeate matrix and spray-dried, resulting in a 31.4% yield with a moisture content of 4.38%. The survival of B. velezensis FZB42 in a simulated gastrointestinal tract was evaluated using the TIM-1 system, revealing a survival rate of 16.05% after passage through the gastric, duodenal, jejunal, and ileal compartments. These findings highlight the possibility of B. velezensis FZB42 being an economically viable and possibly functional synbiotic supplement and effective alternative to antibiotic growth promoters in livestock production. Full article

Strict adherence to a diet is an essential pillar of long-term treatment for many inborn errors of metabolism (IEMs). Tools that educate patients about dietary management can positively condition adherence and prevent morbidity. We designed a free online dietary calculation program (Odimet^®, version 2.1.) for IEMs patients in 2008, updated in 2022, that provides detailed information on the content of amino acids, protein, lipids, carbohydrates, vitamins and minerals in >3000 food products, including specific medical foods for IEM. We analyzed the statistics on visits to Odimet^® to evaluate its usefulness for long-term dietary management during a 5-year period focusing on three periods: pre-pandemic (15 March 2018–14 March 2020); pandemic 1 (15 March 2020–14 March 2021); and pandemic 2 period (15 March 2021–15 March 2023), in 120 patients with the following distribution: 84 patients with phenylketonuria (PKU); 12 with maple syrup urine disease (MSUD); 11 with urea cycle disorders (UCDs); and 13 with classical galactosemia. The evolutionary levels of their specific metabolic markers were evaluated, showing that globally, both pediatric and adult patients maintain a good metabolic control, even during a pandemic (median levels of phenylalanine in pediatric PKU patients 213.4 µmol/L and 482.3 µmol/L in adults; of leucine in MSUD patients: 144.2 µmol/L; of glutamine in UCDs: 726.8 µmol/L; and of galactose 1-phosphate levels in galactosemia: 0.08 µmol/L). The proportion of patients using Odimet^® ranges from 78–100%. An increase in the number of diets being calculated was observed during COVID-19 pandemic. Currently, 14,825 products have been introduced (3094 from the general database, and 11,731 added by users to their own profiles). In 2023 63 emergency dietary adjustments in the studied intoxication-type pathologies were calculated in Odimet^®. Our results suggest that its regular use contributes to maintaining metabolic stability in IEMs patients, allowing them to adapt their menus to their lifestyle, and represents a powerful complementary tele-health tool which can be used to perform remote real-time dietary follow-up. Full article

Background: Cardiac involvement is reported in a significant proportion of patients with classical organic acidurias (OAs), contributing to disability and premature death. Different cardiac phenotypes have been described, among which dilated cardiomyopathy (DCM) is predominant. Despite recent progress in diagnosis and treatment, the natural history of patients with OAs remains unresolved, specifically with regard to the impact of cardiac complications. We therefore performed a retrospective study to address this issue at our Referral Center for Pediatric Inherited Errors of Metabolism. Methods: Sixty patients with OAs (propionic (PA), methylmalonic (MMA) and isovaleric acidemias and maple syrup urine disease) diagnosed from 2000 to 2022 were systematically assessed at baseline and at follow-up. Results: Cardiac anomalies were found in 23/60 OA patients, all with PA or MMA, represented by DCM (17/23 patients) and/or acquired long QT syndrome (3/23 patients). The presence of DCM was associated with the worst prognosis. The rate of occurrence of major adverse cardiac events (MACEs) at 5 years was 55% in PA with cardiomyopathy; 35% in MMA with cardiomyopathy; and 23% in MMA without cardiomyopathy. Liver transplantation was performed in seven patients (12%), all with PA or MMA, due to worsening cardiac impairment, and led to the stabilization of metabolic status and cardiac function. Conclusions: Cardiac involvement was documented in about one third of children diagnosed with classical OAs, confined to PA and MMA, and was often associated with poor outcome in over 50%. Etiological diagnosis of OAs is essential in guiding management and risk stratification. Full article

Inborn error of metabolism disorders (IEMs) are a family of diseases resulting from single-gene mutations that lead to the accumulation of metabolites that are usually toxic or interfere with normal cell function. The etiological link between metabolic alteration and the symptoms of IEMs is still elusive. Several metabolites, which accumulate in IEMs, were shown to self-assemble to form ordered structures. These structures display the same biophysical, biochemical, and biological characteristics as proteinaceous amyloid fibrils. Here, we have demonstrated, for the first time, the ability of each of the branched-chain amino acids (BCAAs) that accumulate in maple syrup urine disease (MSUD) to self-assemble into amyloid-like fibrils depicted by characteristic morphology, binding to indicative amyloid-specific dyes and dose-dependent cytotoxicity by a late apoptosis mechanism. We could also detect the presence of the assemblies in living cells. In addition, by employing several in vitro techniques, we demonstrated the ability of known polyphenols to inhibit the formation of the BCAA fibrils. Our study implies that BCAAs possess a pathological role in MSUD, extends the paradigm-shifting concept regarding the toxicity of metabolite amyloid-like structures, and suggests new pathological targets that may lead to highly needed novel therapeutic opportunities for this orphan disease. Full article