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IntroductionPompe disease (PD) is a glycogen disorder caused by the deficient activity of acid alpha-glucosidase (GAA). We sought to review the latest available evidence on the safety and efficacy of recombinant human GAA enzyme replacement therapy (ERT) for infantile-onset PD (IOPD).MethodsWe systematically searched the MEDLINE (via PubMed) and Embase databases for prospective clinical studies evaluating ERT for IOPD on pre-specified outcomes. Meta-analysis was also performed.ResultsOf 1,722 articles identified, 16 were included, evaluating 316 patients. Studies were heterogeneous and with very low certainty of evidence for most outcomes. A moderate/high risk of bias was present for most included articles. The following outcomes showed improvements associated with alglucosidase alfa, over natural history of PD/placebo, for a mean follow-up of 48.3 months: left ventricular (LV) mass {mean change 131.3 g/m2 [95% confidence interval (CI) 81.02, 181.59]}, time to start ventilation (TSV) [HR 0.21 (95% CI: 0.12, 0.36)], and survival [HR 0.10 (95% CI: 0.05, 0.19)]. There were no differences between the pre- and post-ERT period for myocardial function and psychomotor development. Adverse events (AEs) after ERT were mild in most cases.ConclusionOur data suggest that alglucosidase alfa potentially improves LV mass, TSV, and survival in IOPD patients, with no important safety issues.Systematic Review RegistrationPROSPERO identifier (CRD42019123700).

Background: In phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. Our aim was to assess different weaning approaches used by health professionals across Europe. Methods: A cross sectional questionnaire (survey monkey®) composed of 31 multiple and single choice questions was sent to European colleagues caring for inherited metabolic disorders (IMD). Centres were grouped into geographical regions for analysis. Results: Weaning started at 17–26 weeks in 85% (n = 81/95) of centres, >26 weeks in 12% (n = 11/95) and 26 weeks. First solids were mainly low Phe vegetables (59%, n = 56/95) and fruit (34%, n = 32/95).A Phe exchange system to allocate dietary Phe was used by 52% (n = 49/95) of centres predominantly from Northern and Southern Europe and 48% (n = 46/95) calculated most Phe containing food sources (all centres in Eastern Europe and the majority from Germany and Austria). Some centres used a combination of both methods.A second stage Phe-free L-amino acid supplement containing a higher protein equivalent was introduced by 41% (n = 39/95) of centres at infant age 26–36 weeks (mainly from Germany, Austria, Northern and Eastern Europe) and 37% (n = 35/95) at infant age > 1y mainly from Southern Europe. 53% (n = 50/95) of centres recommended a second stage Phe-free L-amino acid supplement in a spoonable or semi-solid form. Conclusions: Weaning strategies vary throughout European PKU centres. There is evidence to suggest that different infant weaning strategies may influence longer term adherence to the PKU diet or acceptance of Phe-free L-amino acid supplements; rendering prospective long-term studies important. It is essential to identify an effective weaning strategy that reduces caregiver burden but is associated with acceptable dietary adherence and optimal infant feeding development. Keywords: Weaning, Infant, Phenylketonuria, Phenylalanine, Phe-free infant formula

Background: In infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe. Methods: We sent a cross sectional, survey monkey® questionnaire to European health professionals working in IMD. It contained 31 open and multiple-choice questions. The results were analysed according to different geographical regions. Results: Ninety-five centres from 21 countries responded. Over 60% of centres commenced diet in infants by age 10 days, with 58% of centres implementing newborn screening by day 3 post birth. At diagnosis, infant hospital admission occurred in 61% of metabolic centres, mainly in Eastern, Western and Southern Europe. Breastfeeding fell sharply following diagnosis with only 30% of women still breast feeding at 6 months.53% of centres gave pre-measured Phe-free infant formula before each breast feed and 23% alternated breast feeds with Phe-free infant formula. With standard infant formula feeds, measured amounts were followed by Phe-free infant formula to satiety in 37% of centres (n = 35/95), whereas 44% (n = 42/95) advised mixing both formulas together. Weaning commenced between 17 and 26 weeks in 85% centres, ≥26 weeks in 12% and

Organizational life encompasses complex processes and daily activities aimed at increasing efficiency and effectiveness. Workplace meetings are one of such activities that, despite their usefulness in helping organizations to achieve their goals, can be an overlooked practice, with high financial burdens. This exploratory study intends to build an organizational diagnosis formula aimed at assessing the cost-benefit effectiveness of workplace meetings. Based on a sample of seven meetings in a management unit of a higher education public institution, our data show that each meeting results, on average, in six decisions, with the estimated cost in personnel time for each meeting of 496.68€ and a resulting productivity ratio of 198.67€ per decision. The application of our diagnosis formula might contribute to maximize the effectiveness of meetings and organizational performance.

Societal Impact Statement Biological samples and their associated information are an essential resource used by scientists, governments, policymakers, practitioners and communities to ensure that biodiversity can be appropriately protected and sustainably used. Yet, considering the enormous task of documenting the vast numbers of as‐yet‐unknown plant and fungal species, greater international coordination for biological collecting and recording is necessary, built on equitable collecting practices and standards. Here, we propose five commitments to accelerate and enhance scientific knowledge of plant and fungal diversity, while increasing collaboration, benefit sharing and efficiency. Summary Almost all life depends on plants and fungi, making knowledge of their diversity and distribution—primarily derived from biological collections—fundamental to national and international conservation, restoration and sustainable use commitments. However, it is estimated that some 15% of all plant species and over 90% of all fungal species have not yet been scientifically described, hampering our ability to assess and demonstrate the impact of efforts to halt biodiversity loss. In addition, organisations and researchers around the world lack a concerted strategy for increasing complementarity and avoiding overlap in botanical and mycological research, particularly in relation to the collection of specimens. We here present the 2030 Declaration on Scientific Plant and Fungal Collecting, summarising a commitment towards such a necessary strategy. Its components were identified from discussions during and after a series of four workshops and plenary discussions at the 2023 State of the World's Plants and Fungi symposium convened by the Royal Botanic Gardens, Kew, and were then consolidated into the present form by the authors. The Declaration was subsequently opened up for endorsement by signatories. Collectively, we agree on a set of five commitments for cataloguing the world's flora and funga, designed to maximise efficiency, facilitate knowledge exchange and promote equitable collaborations: (1) use evidence‐based collection strategies; (2) strengthen local capacity; (3) collaborate across taxa and disciplines; (4) collect for the future; and (5) share the benefits. This Declaration is a first step towards increased global and regional coordination of scientific collecting efforts.

Background/objectives: Flaxseed has functional properties in the reduction of the risk of chronic non-communicable diseases such as cardiovascular disease, diabetes and cancer. Regardless of its high energy density, the consumption of flaxseed tends to promote body weight maintenance. The purpose of this study was to evaluate energy and macronutrient balance after flaxseed consumption. Subjects/methods: Twenty four healthy volunteers were allocated into 3 experimental groups, when they consumed flaxseed (FS), defatted flaxseed flour (FF), or flaxseed oil (FO). During the control period they were provided a diet without flaxseed products for 7-9 days. Following that diets containing 70 g of one of the flaxseed products were consumed for another 7-9 day- period. Test foods were consumed exclusively in the laboratory and fecal excretion was collected during the study. There was a higher energy excretion (P < 0.05) in the FF and FS groups, compared to their control and FO group. Results: The excretions of total lipid and the PUFA α-linolenic acid were higher in FS group (P < 0.05). Conclusions: The intake of 70 g/day of FS and FF raised lipid and energy excretion, which may mitigated the effect of flaxseed consumption on body weight.Introducción/objetivos: La linaza tiene propiedades funcionales en la reducción del riesgo de enfermedades crónicas no transmisibles tales como las enfermedades cardiovasculares, diabetes y cáncer. A pesar de su alta densidad de energía, el consumo de linaza tiende a favorecer el mantenimiento del peso corporal. El propósito de este estudio fue evaluar la energía y el equilibrio de macronutrientes después del consumo de linaza. Materiales y métodos: Veinticuatro voluntarios sanos fueron distribuidos en 3 grupos experimentales, cuando consumieron linaza (LI), harina de linaza desgrasada (LD), o aceite de linaza (AL). Durante el período de control se les proporcionó una dieta de 7-9 días sin productos de linaza. Enseguida, durante otro período de 7-9 días, fueron consumidas dietas que contenían 70g de uno de los productos de linaza. Los alimentos de prueba fueron consumidos exclusivamente en el laboratorio y la excreción fecal se recogió durante el estudio. Hubo una excreción de energía más alta (P < 0,05) en los grupos LD y LI, en comparación con el grupo de control y el AL. Resultados: Las excreciones de los lípidos totales y el ácido graso α-linolénico, AGPI, fueron mayores en el grupo LI (P < 0,05). Conclusiones: La ingesta de 70 g/día de LI y LD aumentó la excreción de lípidos y energía, lo que puede haber mitigado el efecto del consumo de linaza en el peso corporal.

Hay diversos métodos disponibles para medir el consumo de energía en el ser humano. Debido al alto costo y pequeño número de laboratorios capaces de utilizar la calorimetría directa y la técnica del agua doblemente marcada, se ha realizado un número relativamente pequeño de mediciones del gasto energético utilizándose estos métodos. Así, cada día más, los investigadores se atienen en alto grado a los métodos de calorimetría indirecta (CI) y bioimpedancia eléctrica (BIA) y los clínicos a las ecuaciones de predicción para calcular los requerimientos de energía total en sus estudios y pacientes respectivamente. Objetivo: Evaluar el Gasto Energético Basal (GEB) y el Gasto Energético Total (GET) de individuos saludables comparando los resultados de CI, BIA y ecuaciones predictivas con los de requerimiento estimado de energía (EER), Fleisch, FAO/WHO/UNU 1985 y 2001. Material y métodos: Se evaluó el gasto energético en 81 individuos de ambos sexos por CI y BIA. De estos, fueron calculados el GEB por las ecuaciones FAO/WHO/UNU 1985 y 2001 y el GET por EER, Fleisch y FAO/WHO/UNU 1985 y 2001. Los datos se analizaron con el programa Sigma Stat for Windows versión 2.03 y se utilizó eltest de Dunn's para la comparación de las medianas obtenidas por los métodos y ecuaciones (p < 0,05). Resultados: Se verificó que el 60,5% de los participantes eran hombres. Los individuos en su mayoría eran eutróficos. La BIA mostró diferencias significativas entre los sexos. Las medianas de GEB, obtenidas por BIA y CI, presentaron diferencias estadísticamente significativas solamente para mujeres (p < 0,05) con una subestimación de los resultados del GEB obtenidos por la BIA en relación a los obtenidos por CI. Diferencias significativas fueron encontradas entre las fórmulas predictivas y la CI para ambos sexos, demostrando también una subestimación de esas en relación a la CI. Se observó una sobreestimación de GET con las fórmulas predictivas de la FAO/WHO/UNU/85 y 2001 en relación a la EER y Fleisch, para ambos sexos. Conclusión: La estimación del GEB puede ser realizada tanto por BIA como por CI, pero la BIA debe ser usada con criterios, ya que subestima de forma significativa para mujeres. En relación a las ecuaciones predictivas se puede observar que la sobreestimación puede desarrollar un balance energético positivo en poblaciones con riesgos para el sobrepeso y obesidad. Así son necesarias comparaciones para identificar las limitaciones de cada método.There are many available methods to measure the human energy consumption. Due to the high cost and small number of laboratories able to perform it, the direct calorimetry and doubly labeled water technique are rarely used to measure the energy expenditure. As consequence, there is an increase use of the indirect calorimetry and bioelectrical impedance analysis methods by researchers and predictive equations by physicians to calculate the total energy requirements in research and in clinic. Objective: To evaluate the basal energy expenditure (GEB) and total energy expenditure (GET) of healthy subjects comparing results of indirect calorimetry (CI), bioelectrical impedance analysis (BIA) and predictive equations such as estimated energy requirements (EER), Fleish, FAO/WHO/UNU 1985 and 2001. Material and methods: It was evaluated the energy expenditure in 81 subjects of both gender by CI and BIA. Of these, the GEB by predictive equations FAO/WHO/UNU 1985 and 2001 and the GET by EER, Fleisch and FAO/WHO/UNU 1985 and 2001 were calculated. The data was analyzed with the software Sigma Stat for Windows version 2.03 and Dunn's test was used for comparison of mean obtained with methods and equations (p < 0.05). Results: It was verified that 60.5% of the participants were men. The majority of subjects were eutrofic. BIA showed significant differences between men and women. The differences between the values of GEB, obtained by BIA and CI, were statistically significant only for women (p < 0.05) with an underestimation of the GEB results obtained by BIA in relation to those obtained by CI. Significant difference was found between the predictive equations and CI for both sexes, also demonstrating an underestimation of the equations in relation to CI. It was observed an overestimation of GET with the predictive equations of FAO/WHO/UNU/85 and 2001 in relation to EER and Fleisch for both sexes. Conclusion: The estimation of GEB can be carried out by both BIA and CI, although BIA must be used with criteria since it underestimates in a significant way for women. In relation to the predictive equations, overestimation can develop positive energy balance in populations with risks for overweight and obesity. Thus, it is necessary comparisons to identify the limitations of each method.

Isoprene (C5H8) is a hydrocarbon gas emitted by many tree species and has been shown to protect photosynthesis under abiotic stress. Under optimal conditions for photosynthesis, ~70%-90% of carbon used for isoprene biosynthesis is produced fromrecently assimilated atmospheric CO2. While the contribution of alternative carbon sources that increase with leaf temperature and other stresses have been demonstrated, uncertainties remain regarding the biochemical source(s) of isoprene carbon. In this study, we investigated leaf isoprene emissions (Is) from neotropical species Inga edulis Mart. as a function of light and temperature under ambient (450 μmol m-2 s-1) and CO2-free (0 μmol m-2 s-1) atmosphere. Is under CO2-free atmosphere showed light-dependent emission patterns similar to those observed under ambient CO2, but with lower light saturation point. Leaves treated with the photosynthesis inhibitor DCMU (3-(3,4-dichlorophenyl)-1,1-dimethylurea) failed to produce detectable Is in normal light under a CO2-free atmosphere. While strong temperature-dependent Is were observed under CO2-free atmosphere in the light, dark conditions failed to produce detectable Is even at the highest temperatures studied (40 °C). Treatment of leaves with 13C-labeled sodium bicarbonate under CO2-free atmosphere resulted in Is with over 50% containing at least one 13C atom. Is under CO2-free atmosphere and standard conditions of light and leaf temperature represented 19% ± 7% of emissions under ambient CO2. The results show that the reassimilation of leaf internal CO2 contributes to Is in the neotropical species I. edulis. Through the consumption of excess photosynthetic energy, our results support a role of isoprene biosynthesis, together with photorespiration, as a key tolerance mechanism against high temperature and high light in the tropics.

RESUMO Neste trabalho, os autores sinonimizam algumas variedades da família Piperaceae.

Neste trabalho os autores referem-se à heterofilia de Paullinia carpopodea Camb., e tecem considerações sobre as prováveis causas que a determinam. Mencionam a estrutura nodal trilacunar e as características da vascularizaçSo foliar, citando a ocorrência de floema interno nos feixes mais desenvolvidos do pecíolo e da nervura principal. Focalizam, também, os laticíferos ramificados, que se anastomasam segundo um padrão reticulado. Registram a presença de tricomas tectores e glandulares, do tipo maleiforme, nas duas epidermes e chamam a atenção para a ocorrência de estômatos anomocíticos na epiderme adaxial, sobre a nervura mediana e nas secundárias. Tecem considerações quanto aos tipos e distribuição dos estômatos na epiderme abaxial. Evidenciam domácias marsupiformes nas axilas da nervura principal, nas quais se alojam insetos da ordem Thysanoptera, pequenos ácaros não identificados e agrupamentos de ovos. Referem-se também à presença de estruturas com características de hidatódiosepitema, na região do mucron.

Background: Exercise has been identified as an allied health strategy that can support the management of depression in older adults, yet the relative effectiveness for different exercise modalities is unknown. To meet this gap in knowledge, we present a systematic review and network meta-analysis of randomised controlled trials (RCTs) to examine the head-to-head effectiveness of aerobic, resistance, and mind-body exercise to mitigate depressive symptoms in adults aged ≥ 65 years. Methods: A PRISMA-NMA compliant review was undertaken on RCTs from inception to September 12 th, 2019. PubMed, Web of Science, CINAHL, Health Source: Nursing/Academic Edition, PsycARTICLES, PsycINFO, and SPORTDiscus were systematically searched for eligible RCTs enrolling adults with a mean age ≥ 65 years, comparing one or more exercise intervention arms, and which used valid measures of depressive symptomology. Comparative effectiveness was evaluated using network meta-analysis to combine direct and indirect evidence, controlling for inherent variation in trial control groups. Results: The systematic review included 82 RCTs, with 69 meeting eligibility for the network meta-analysis ( n = 5,379 participants). Pooled analysis found each exercise type to be effective compared with controls (Hedges’ g = -0.27 to -0.51). Relative head-to-head comparisons were statistically comparable between exercise types: resistance versus aerobic (Hedges’ g = -0.06, PrI = -0.91, 0.79), mind-body versus aerobic (Hedges’ g = -0.12, PrI = -0.95, 0.72), mind-body versus resistance (Hedges’ g = -0.06, PrI = -0.90, 0.79). High levels of compliance were demonstrated for each exercise treatment. Conclusions: Aerobic, resistance, and mind-body exercise demonstrate equivalence to mitigate symptoms of depression in older adults aged ≥ 65 years, with comparably encouraging levels of compliance to exercise treatment. These findings coalesce with previous findings in clinically depressed older adults to encourage personal preference when prescribing exercise for depressive symptoms in older adults. Registration: PROSPERO CRD42018115866 (23/11/2018).

Background: Exercise has been identified as an allied health strategy that can support the management of depression in older adults, yet the relative effectiveness for different exercise modalities is unknown. To meet this gap in knowledge, we present a systematic review and network meta-analysis of randomised controlled trials (RCTs) to examine the head-to-head effectiveness of aerobic, resistance, and mind-body exercise to mitigate depressive symptoms in adults aged ≥ 65 years. Methods: A PRISMA-NMA compliant review was undertaken on RCTs from inception to September 12 th, 2019. PubMed, Web of Science, CINAHL, Health Source: Nursing/Academic Edition, PsycARTICLES, PsycINFO, and SPORTDiscus were systematically searched for eligible RCTs enrolling adults with a mean age ≥ 65 years, comparing one or more exercise intervention arms, and which used valid measures of depressive symptomology. Comparative effectiveness was evaluated using network meta-analysis to combine direct and indirect evidence, controlling for inherent variation in trial control groups. Results: The systematic review included 81 RCTs, with 69 meeting eligibility for the network meta-analysis ( n = 5,379 participants). Pooled analysis found each exercise type to be effective compared with controls (Hedges’ g = -0.27 to -0.51). Relative head-to-head comparisons were statistically comparable between exercise types: resistance versus aerobic (Hedges’ g = -0.06, PrI = -0.91, 0.79), mind-body versus aerobic (Hedges’ g = -0.12, PrI = -0.95, 0.72), mind-body versus resistance (Hedges’ g = -0.06, PrI = -0.90, 0.79). High levels of compliance were demonstrated for each exercise treatment. Conclusions: Aerobic, resistance, and mind-body exercise demonstrate equivalence to mitigate symptoms of depression in older adults aged ≥ 65 years, with comparably encouraging levels of compliance to exercise treatment. These findings coalesce with previous findings in clinically depressed older adults to encourage personal preference when prescribing exercise for depressive symptoms in older adults, irrespective of severity. Registration: PROSPERO CRD42018115866 (23/11/2018).