cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.14275991.v1
title: Acute hepatic porphyrias for the neurologist: current concepts and perspectives

description: ABSTRACT Background: Acute hepatic porphyrias represent an expanding group of complex inherited metabolic disorders due to inborn errors of metabolism involving heme biosynthesis. Objective: We aimed to review the main clinical and therapeutic aspects associated with acute hepatic porphyrias. Methods: The authors provided a wide non-systematic review of current concepts and recently acquired knowledge about acute hepatic porphyrias. Results: Acute neurovisceral attacks are the most common and life-threatening presentation of this group and are often considered the main clinical manifestation by clinicians during differential diagnosis and the start of proper diagnostic work-up for acute porphyrias. However, atypical presentations with central nervous system involvement, neuropsychiatric disturbances, and some subtypes with photosensitivity usually make the definite diagnosis difficult and late. Early therapeutic interventions are essential during emergency treatment and intercritical periods to avoid recurrent severe presentations. The availability of new disease-modifying therapeutic proposals based on small interfering RNA (siRNA)-based therapies, complementary to the classic intravenous glucose infusion and hemin-based treatments, emphasizes the importance of early diagnosis and genetic counseling of patients. Conclusions: This review article highlights the main biochemical, pathophysiological, clinical, and therapeutic aspects of acute hepatic porphyrias in clinical practice.
DOI: 10.6084/m9.figshare.14276024
title: Acute chorea: case series from the emergency room of a Brazilian tertiary-level center

description: ABSTRACT Background: Chorea is a movement disorder characterized by random, brief and migratory involuntary muscle contractions. It is defined as acute when present within hours to days. Three main causes for this scenario have emerged as most likely: vascular, toxic-metabolic and inflammatory. Objectives: To identify the prevalence of the main etiologies and major clinical findings of acute chorea in the emergency room of a tertiary-level referral center; and to suggest an approach for guiding the diagnostic workup and clinical management. Methods: We retrospectively reviewed the clinical aspects and neuroimaging data of 10 patients presenting with acute chorea at the neurological emergency room of our hospital from 2015 to 2019. Results: Stroke was the most common etiology (50% of the cases). All of them were ischemic. It was noteworthy that only one case demonstrated the classical ischemic topographic lesion at the contralateral subthalamic nuclei. Regarding nonvascular etiologies, nonketotic hyperglycemia was the major cause, followed by drug-related chorea. One patient showed inflammatory etiology, which was probably Sydenham chorea reactivation. Conclusion: Acute chorea is an uncommon and challenging problem at the emergency room, often associated with potentially treatable causes. We suggest that use of the acronym DANCE (Diagnosis of chorea, Acute stroke protocol, Normal glucose levels, Check neuroimaging, Exposure to drugs) could form a potential initial approach in the evaluation, in order to emphasize causes that require prompt proper management (e.g. thrombolysis).
DOI: 10.6084/m9.figshare.14276027
title: The impact of COVID-19 on patients with epilepsy

description: ABSTRACT Background: The COVID-19 pandemic and social distancing can have adverse impacts on adult people with epilepsy (PWE). Objective: To investigate the seizure frequency, the perceived well-being, and the presence of anxiety symptoms in PWE during the COVID-19 pandemic period. Methods: Data from a questionnaire on the repercussions of COVID-19 were analyzed in relation to the clinical variables of 114 PWE, with a significance level of p&lt;0.05. Results: There were 26 cases of COVID-19 in PWE and/or family members (22.8%). During the pandemic period, 11 PWE (9.6%) reported an increase in seizures, but unrelated to COVID-19. Also, the number of crises in PWE with previous depressive disorders increased, with differences between epilepsies. Symptoms of depression, impaired well-being, and concern for their lifestyle were significant in PWE with a previous diagnosis of depression. Impaired well-being, increased anxiety, nervousness, and tiredness, and the concern with being infected were mentioned by a high number of PWE in the pandemic. Conclusion: Seizure frequency increased during the pandemic period, a finding associated with clinical variables of epilepsy. PWE with depression had worse perceived well-being. Changes in well-being and increased anxiety and nervousness were frequent in the pandemic.
DOI: 10.6084/m9.figshare.14276028
title: Long-term safety of azathioprine for treatment of neuromyelitis optica spectrum disorders

description: ABSTRACT Background: Azathioprine is a common first-line therapy for neuromyelitis optica spectrum disorder (NMOSD). Objective: The aim of this study was to determine whether long-term treatment (&gt;10 years) with azathioprine is safe in NMOSD. Methods: We conducted a retrospective medical record review of all patients at the School of Medicine of the University of São Paulo (São Paulo, Brazil) who fulfilled the 2015 international consensus diagnostic criteria for NMOSD and were treated with azathioprine for at least 10 years. Results: Out of 375 patients assessed for eligibility, 19 were included in this analysis. These patients’ median age was 44 years (range=28-61); they were mostly female (17/19) and AQP4-IgG seropositive (18/19). The median disease duration was 15 years (range=10-39) and most patients presented a relapsing clinical course (84.2%). The median duration of treatment was 11.9 years (range=10.0-23.8). The median annualized relapse rates (ARR) pre- and post-treatment with azathioprine were 1 (range=0.1-2) and 0.1 (range=0-0.35); p=0.09. Three patients (15.7%) had records of adverse events during the follow-up, which consisted of chronic B12 vitamin deficiency, pulmonary tuberculosis and breast cancer. Conclusion: Azathioprine may be considered a safe agent for long-term treatment (&gt;10 years) of NMOSD, but continuous vigilance for infections and malignancies is required.
DOI: 10.6084/m9.figshare.14276030
title: Silicon nutrition counteracts salt-induced damage associated with changes in biochemical responses in apple

description: ABSTRACT Salt stress is an environmental challenge that adversely influences plant responses. Silicon (Si) nutrition plays critical roles in plant tolerance to salt stress. Apple (Malus domestica Borkh.), a salt sensitive fruit species, was used in the present experiment to investigate the influences of Si on salt stress as well as on alterations of biochemical responses. Apple cv Fuji grafted on M9 clonal rootstock was exposed to salt stress for 4 months with 35 mmol·L-1 NaCl. CaSiO3 doses (0.5, 1 and 2 mmol·L-1) were applied to the roots of the salt-stressed apple plants except control. Si application resulted in mitigation of salt stress in apple plants. The highest chlorophyll a, b and a + b were obtained from the 1 mmol·L-1 Si treatment (5.37, 2.41 and 7.78 μg·g-1 fw, respectively). Moreover, Si treatment had higher chlorophyll content compared to the control as well as salt exposed plants. Silicon applications led to a reduction in malondialdehyde (MDA) content even lower than control. The 0.5 mmol·L-1 Si treatment had the highest values of ascorbate peroxidase (APX) and phenolic content. The results show that Si nutrition plays important roles in apple salt tolerance via biochemical mechanisms and that it can be used in areas subject to salt stress for apple growing.
DOI: 10.6084/m9.figshare.14276027.v1
title: The impact of COVID-19 on patients with epilepsy

description: ABSTRACT Background: The COVID-19 pandemic and social distancing can have adverse impacts on adult people with epilepsy (PWE). Objective: To investigate the seizure frequency, the perceived well-being, and the presence of anxiety symptoms in PWE during the COVID-19 pandemic period. Methods: Data from a questionnaire on the repercussions of COVID-19 were analyzed in relation to the clinical variables of 114 PWE, with a significance level of p&lt;0.05. Results: There were 26 cases of COVID-19 in PWE and/or family members (22.8%). During the pandemic period, 11 PWE (9.6%) reported an increase in seizures, but unrelated to COVID-19. Also, the number of crises in PWE with previous depressive disorders increased, with differences between epilepsies. Symptoms of depression, impaired well-being, and concern for their lifestyle were significant in PWE with a previous diagnosis of depression. Impaired well-being, increased anxiety, nervousness, and tiredness, and the concern with being infected were mentioned by a high number of PWE in the pandemic. Conclusion: Seizure frequency increased during the pandemic period, a finding associated with clinical variables of epilepsy. PWE with depression had worse perceived well-being. Changes in well-being and increased anxiety and nervousness were frequent in the pandemic.
DOI: 10.6084/m9.figshare.14276028.v1
title: Long-term safety of azathioprine for treatment of neuromyelitis optica spectrum disorders

description: ABSTRACT Background: Azathioprine is a common first-line therapy for neuromyelitis optica spectrum disorder (NMOSD). Objective: The aim of this study was to determine whether long-term treatment (&gt;10 years) with azathioprine is safe in NMOSD. Methods: We conducted a retrospective medical record review of all patients at the School of Medicine of the University of São Paulo (São Paulo, Brazil) who fulfilled the 2015 international consensus diagnostic criteria for NMOSD and were treated with azathioprine for at least 10 years. Results: Out of 375 patients assessed for eligibility, 19 were included in this analysis. These patients’ median age was 44 years (range=28-61); they were mostly female (17/19) and AQP4-IgG seropositive (18/19). The median disease duration was 15 years (range=10-39) and most patients presented a relapsing clinical course (84.2%). The median duration of treatment was 11.9 years (range=10.0-23.8). The median annualized relapse rates (ARR) pre- and post-treatment with azathioprine were 1 (range=0.1-2) and 0.1 (range=0-0.35); p=0.09. Three patients (15.7%) had records of adverse events during the follow-up, which consisted of chronic B12 vitamin deficiency, pulmonary tuberculosis and breast cancer. Conclusion: Azathioprine may be considered a safe agent for long-term treatment (&gt;10 years) of NMOSD, but continuous vigilance for infections and malignancies is required.
DOI: 10.6084/m9.figshare.14276031
title: Salt stress alleviation by seed priming with silicon in lettuce seedlings: an approach based on enhancing antioxidant responses

description: ABSTRACT Seed germination and seedling growth are the stages most sensitive to salt stress, which can induce ion-specific effects and oxidative stress. Although silicon (Si) has been known to improve plant defense systems against stressful-conditions, little is known about seed priming with Si and mechanisms of Si-mediated alleviation of salt stress in seedlings. To further the modulation of salt-stress responses and their relation with Si, seed priming with calcium silicate (0, 0.05 and 0.1 mM Ca2SiO4) was used in lettuce seeds to improve seedgermination and tolerance on subsequent salt-stress exposure (0 and 50 mM NaCl). The effect of Si-priming was assessed on germination, germination rate index and mean germination time and survival of seedlings in salt-stressful conditions supported by biochemical approach involving antioxidant responses. Overall results indicated that Si plays a role in alleviating the negative effects of salt stress by improving germination performance and displayed increased SOD, CAT and GR activities and reduced malondialdehyde and H2O2 contents. Thus, our findings demonstrate that seed-priming with Si is an efficient management technique thatcan be used to alleviate deleterious effects of salt-stressful on germination of lettuce seeds and enhance salt tolerance of seedlings due the increased activity of ROS- scavenging enzymes.
DOI: 10.6084/m9.figshare.14276024.v1
title: Acute chorea: case series from the emergency room of a Brazilian tertiary-level center

description: ABSTRACT Background: Chorea is a movement disorder characterized by random, brief and migratory involuntary muscle contractions. It is defined as acute when present within hours to days. Three main causes for this scenario have emerged as most likely: vascular, toxic-metabolic and inflammatory. Objectives: To identify the prevalence of the main etiologies and major clinical findings of acute chorea in the emergency room of a tertiary-level referral center; and to suggest an approach for guiding the diagnostic workup and clinical management. Methods: We retrospectively reviewed the clinical aspects and neuroimaging data of 10 patients presenting with acute chorea at the neurological emergency room of our hospital from 2015 to 2019. Results: Stroke was the most common etiology (50% of the cases). All of them were ischemic. It was noteworthy that only one case demonstrated the classical ischemic topographic lesion at the contralateral subthalamic nuclei. Regarding nonvascular etiologies, nonketotic hyperglycemia was the major cause, followed by drug-related chorea. One patient showed inflammatory etiology, which was probably Sydenham chorea reactivation. Conclusion: Acute chorea is an uncommon and challenging problem at the emergency room, often associated with potentially treatable causes. We suggest that use of the acronym DANCE (Diagnosis of chorea, Acute stroke protocol, Normal glucose levels, Check neuroimaging, Exposure to drugs) could form a potential initial approach in the evaluation, in order to emphasize causes that require prompt proper management (e.g. thrombolysis).
DOI: 10.6084/m9.figshare.14276033
title: Evidence of accelerated and altered pollen development after Imazapyr treatment in resistant sunflower

description: ABSTRACT Imazapyr (IM) treatment applied at early reproductive developmental stages have been associated with induced male sterility in sunflower (Helianthus annuus L.). The aim of this work was to determine the effects induced by two different doses of IM-treatment (1X and 2X; 80 and 160 g a.i.·ha-1) on pollen grain yield, pollen viability and on the exine surface, in an intermediate resistant (I) and a completely resistant (R) genotype. An anatomical analysis of some stages during the microsporogenesis and microgametogenesis was also achieved by confocal microscopy. Plants of both genotypes survived the herbicide treatments and showed no external injury symptoms. Differential response to different doses of IM was obtained in R and I genotypes. The pollen grains per flower and the viability in seeds were significantly reduced in the I genotype by the 2X treatment; however, the pollen viability of the remaining grains was not altered, and the diameter of the pollen grains was increased by the herbicide treatment. Anatomical and cytological observations of the sporogenous tissue development were made via confocal microscopy. IM treatment induced alterations in sporogenous cells during the initial phases of microsporogenesis in both genotypes, and the 2X dose accelerated the microgametogenesis process, particularly in the I genotype. These findings improved the understanding of the mechanism underlying the gametocide effects of the imidazolinones in sunflower.
DOI: 10.6084/m9.figshare.14276034
title: Postharvest quality of ‘Brookfield’ apple field-treated with naphthalene acetic acid alone or combined with other growth regulators

description: ABSTRACT The Gala cultivars represent about 65% of the Brazilian apple production, however, it has a short harvest period, making necessary to use technologies that anticipate or delay fruit maturation. A widely used technology is the preharvest application of growth regulators. The objective of this investigation was to evaluate the effect of preharvest application of naphthalene acetic acid (NAA) combined with aminoethoxyvinylglycine (AVG) and 2-chloroethylphosphonic acid (Ethephon), on the quality of ‘Brookfield’ apple at harvest and after eight months controlledatmosphere (CA)(1.2 kPa O2 and 2.0 kPa CO2) storage followed by seven days of shelf life at 20 °C. The treatments were: [1] Control: application of water only; [2] NAA (40 g.ha-1); [3] NAA (40 g·ha-1) plus Ethephon (2.0 L·ha-1 24% of active ingredient), and [4] NAA (40 g·ha-1) plus AVG (0.83 kg.ha-1 15% of active ingredient). At harvest, fruit treated with NAA presented a higher level of starch degradation, ethylene production and respiration rate. Fruit treated with NAA plus AVG maintained better quality after eight months under CA storage plus seven days of shelf life, due to higher healthy fruit amount and higher flesh firmness, but this combination reduced the red skin color index. Additionally, NAA plus Ethephon may be an alternative to maintain the quality of ‘Brookfield’ apple during storage in comparison to the application of NAA isolated.
DOI: 10.6084/m9.figshare.14276036
title: Selection of promissory crops of wild cherry-type tomatoes using physicochemical parameters and antioxidant contents

description: ABSTRACT Tomato is considered a fruit with a high food demand across the world due to its high antioxidant capacity. Lycopene content in tomato has been widely studied, particularly in cherry-type tomatoes which are considered a high source of lycopene for humans. The aim of this study was to analyze the differences in terms of concentration and variation of diverse physicochemical parameters of quality (acidity, vitamin C, pH, °Brix, color coordinates L*, a* and b*) and, antioxidants like ?-carotene and lycopene in 11 accessions of cherry-type tomatoes, considering the advantages of antioxidants for human health. Multicriteria analysis and other tests showed substantial differences among the accessions. IAC401 was associated with the best physicochemical characteristics of a promissory cherry-type tomato for cultivation due to its association with functional food, followed by IAC426R and IAC1624. This study allowed an innovative association of physicochemical characteristics and antioxidant contents of cherry-type tomatoes through multicriteria analyses which enable the analysis, elimination, and proposal of promising accessions for their use in future crops which will promote the best intrinsic features of tomato to be used in the studied region. In this case accessions with the highest scores offer not only optimum amounts of antioxidants and highest levels of organic acids and sugars.
DOI: 10.6084/m9.figshare.14276030.v1
title: Silicon nutrition counteracts salt-induced damage associated with changes in biochemical responses in apple

description: ABSTRACT Salt stress is an environmental challenge that adversely influences plant responses. Silicon (Si) nutrition plays critical roles in plant tolerance to salt stress. Apple (Malus domestica Borkh.), a salt sensitive fruit species, was used in the present experiment to investigate the influences of Si on salt stress as well as on alterations of biochemical responses. Apple cv Fuji grafted on M9 clonal rootstock was exposed to salt stress for 4 months with 35 mmol·L-1 NaCl. CaSiO3 doses (0.5, 1 and 2 mmol·L-1) were applied to the roots of the salt-stressed apple plants except control. Si application resulted in mitigation of salt stress in apple plants. The highest chlorophyll a, b and a + b were obtained from the 1 mmol·L-1 Si treatment (5.37, 2.41 and 7.78 μg·g-1 fw, respectively). Moreover, Si treatment had higher chlorophyll content compared to the control as well as salt exposed plants. Silicon applications led to a reduction in malondialdehyde (MDA) content even lower than control. The 0.5 mmol·L-1 Si treatment had the highest values of ascorbate peroxidase (APX) and phenolic content. The results show that Si nutrition plays important roles in apple salt tolerance via biochemical mechanisms and that it can be used in areas subject to salt stress for apple growing.
DOI: 10.6084/m9.figshare.14276033.v1
title: Evidence of accelerated and altered pollen development after Imazapyr treatment in resistant sunflower

description: ABSTRACT Imazapyr (IM) treatment applied at early reproductive developmental stages have been associated with induced male sterility in sunflower (Helianthus annuus L.). The aim of this work was to determine the effects induced by two different doses of IM-treatment (1X and 2X; 80 and 160 g a.i.·ha-1) on pollen grain yield, pollen viability and on the exine surface, in an intermediate resistant (I) and a completely resistant (R) genotype. An anatomical analysis of some stages during the microsporogenesis and microgametogenesis was also achieved by confocal microscopy. Plants of both genotypes survived the herbicide treatments and showed no external injury symptoms. Differential response to different doses of IM was obtained in R and I genotypes. The pollen grains per flower and the viability in seeds were significantly reduced in the I genotype by the 2X treatment; however, the pollen viability of the remaining grains was not altered, and the diameter of the pollen grains was increased by the herbicide treatment. Anatomical and cytological observations of the sporogenous tissue development were made via confocal microscopy. IM treatment induced alterations in sporogenous cells during the initial phases of microsporogenesis in both genotypes, and the 2X dose accelerated the microgametogenesis process, particularly in the I genotype. These findings improved the understanding of the mechanism underlying the gametocide effects of the imidazolinones in sunflower.
DOI: 10.6084/m9.figshare.14276039
title: Characterization of tomato generations according to a three-way data analysis

description: ABSTRACT Availability of a three-way data analysis to characterize two consecutive tomato (Solanum lycopersicum) generations is necessary to continue a plant breeding program with less uncertainty. The aim of this work was to analyze tomato fruit quality from F2 and F3 populations by two three-way data analysis: multiple factorial analysis (MFA) and generalized procrustes analysis (GPA). These techniques have the same main objective, searching for a common structure, but they achieve it in different ways. This work evaluated 18 tomato genotypes, represented by individual plants in F2 and selfed families in F3. The same quantitative traits related to fruit quality were measured in both generations. The first two axes of the MFA represented 51.0% of the total variability. From the representation of the genotypes on these axes, traits differing from one generation to another were identified. The first two axes of the GPA represented 56.4% of the total variability. This analysis provided a table of analysis of variance (ANOVA), which corroborated the graphic and analytical interpretations of the MFA, a technique that provides the composition of the obtained axes. The comparison between the results obtained from these techniques indicated that both MFA and GPA allowed the detection of genotypes with discrepancies between the two generations. The MFA technique presented the advantage of studying graphically and analytically the nature and degree of phenotypic differences among genotypes in both generations, while the GPA complemented the analysis with an ANOVA, achieving the quantification of statistical significances for the discrepancies or similarities between them.