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Objective: To investigate whether a short maximal sprint can provide another means to counter the rapid fall in glycemia associated with moderate-intensity exercise in individuals with type 1 diabetes and therefore decrease the risk of early postexercise hypoglycemia.Research Design and Methods: In the study, seven male subjects with type 1 diabetes injected their normal insulin dose and ate their usual breakfast. When their postprandial glycemia fell to approximately 11 mmol/l, they pedaled at 40% Vo(2peak) for 20 min on a cycle ergometer then immediately engaged in a maximal 10-s cycling sprint (sprint trial) or rested (control trial); the sprint and rest trials were administered in a counterbalanced order.Results: Moderate-intensity exercise resulted in a significant fall (P < 0.05) in glycemia in both trials (means +/- SE: 3.6 +/- 0.5 vs. 3.1 +/- 0.5 mmol/l for sprint and control, respectively). The subsequent short cycling sprint opposed a further fall in glycemia for 120 min, whereas in the absence of a sprint, glycemia decreased further (3.6 +/- 1.22 mmol/l; P < 0.05) after exercise. The stabilization of glycemia in the sprint trial was associated with elevated levels of catecholamines, growth hormone, and cortisol. In contrast, these hormones remained at stable or near-stable levels in the control trial. Changes in insulin and free fatty acid levels were similar in the sprint and control trials.Conclusions: These results suggest that after moderate-intensity exercise, it is preferable for young individuals with insulin-treated, complication-free type 1 diabetes to engage in a 10-s maximal sprint to acutely oppose a further fall in glycemia than to only rest. The addition of the sprint after moderate-intensity exercise provides another means to reduce the risk of hypoglycemia in active individuals with type 1 diabetes. [ABSTRACT FROM AUTHOR]

Objective: Intent-to-treat analysis follows patients from listing to death, regardless of their transplant status, and aims to provide a more holistic scope of the progress made in adult solid-organ transplantation., Background: Many studies have shown progress in waitlist and post-transplant survival for adult kidney, liver, heart, and lung transplants, but there is a need to provide a more comprehensive perspective of transplant outcomes for patients and their families., Methods: Univariable and multivariable Cox regression analyses were used to analyze factors contributing to intent-to-treat survival in 813,862 adults listed for kidney, liver, heart, and lung transplants. The Kaplan-Meier method was used to examine changes in waitlist, post-transplant, and intent-to-treat survival. Transplantation rates were compared using χ 2 tests., Results: Intent-to-treat survival has steadily increased for liver, heart, and lung transplants. The percentage of patients transplanted within 1 year significantly increased for heart (57.4% from 52.9%) and lung (73.5% from 33.2%). However, the percentage of patients transplanted within 1 year significantly decreased from 35.8% to 21.2% for kidney transplant. Notably, intent-to-treat survival has decreased for kidneys despite increases in waitlist and post-transplant survival, likely because of the decreased transplant rate., Conclusion: Intent-to-treat survival steadily improved for liver, heart, and lung transplant over the 30-year study period. Continued advancements in allocation policy, immunosuppression, and improved care of patients on the waitlist may contribute to further progress in outcomes of all organs, but the increasing discrepancy in supply and demand of donor kidneys is alarming and has impeded the progress of kidney intent-to-treat survival., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

Epilepsy surgery provides excellent benefits in post-traumatic epilepsy of the temporal lobe (PTE-TL), but outcomes relative to non-traumatic epilepsy of the temporal lobe (NTE-TL) are less favorable. Large well-designed studies are recommended to further clarify the role of epilepsy surgery in PTE. It is unclear whether epilepsy surgery outcomes in PTE are as robust as described for drug resistant epilepsy (DRE) in general. Prior outcome studies in PTE are limited by small numbers, lack of a control group, or both. We performed a meta-analysis of studies in temporal lobe epilepsy (TLE) to evaluate post-surgical outcomes in those with PTE-TL and compare outcomes to those with NTE-TL. PubMed, EMBASE, and Web of Science databases were queried for studies reporting epilepsy surgery outcomes separately for PTE-TL and NTE-TL. Outcomes were divided into favorable (Engel Class I) or unfavorable (Engel Class II-IV) for comparison. Meta-analyses were performed to evaluate: 1) the proportion of Class I outcomes following epilepsy surgery in PTE-TL; and 2) calculate the odds of Class I surgical outcomes in PTE-TL compared with NTE-TL. Of 3669 articles that reported surgical outcomes in epilepsy, nine studies ( n  = 886) were identified that reported outcomes for both PTE-TL ( n  = 219) and NTE-TL ( n  = 667). The weighted proportion of favorable outcomes (Engel Class I) were high for both PTE-TL (70.1%, 95% CI 61.9%-78.3%) and NTE-TL (75.2%, 95% CI 69.4%-80.2%). Patients with PTE-TL were at greater risk of unfavorable (Engel Class II-IV) outcomes (relative risk 1.36, 95% CI 1.04-1.78) compared with NTE-TL.

Purpose: Patients often have basic audiometry (BA) but not objective diagnostic tests of the vestibular system (VNG) when complaining of symptoms of a vestibular disorder. The relationship of BA results to VNG results is unknown. This study sought to determine if BA scores are related to impaired VNG scores., Materials and Methods: We reviewed electronic medical records at a tertiary care center, for patients seen between 2015 and 2021 who had had both a BA and a VNG (n = 651). BA subtests were pure tone averages, word recognition, and tympanogram. VNG subtests were cervical vestibular evoked myogenic potentials, Dix-Hallpike maneuvers, and bi-thermal caloric tests. All tests were summarized as normal/abnormal., Results: More subjects had abnormal BA than abnormal VNG scores. Age but not sex was significantly related to abnormal scores. High BP was a significant comorbidity in 15 % of the sample, more in patients with abnormal than normal VNG scores. Although the abnormal BA and abnormal VNG were significantly related, pure tone averages and tympanogram scores were not related to VNG subtests. Abnormal word recognition with both ears combined was significantly related to normal and abnormal bi-thermal caloric tests., Conclusions: If the clinician needs to know of any VNG impairment, in general, then performing a BA without a VNG might suffice. If the clinician needs information about the details of possible vestibular impairment, then a VNG should be performed., Competing Interests: Declaration of competing interest There are no conflicts of interest, financial or otherwise., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

The purpose of this study was to determine the effect of exercise intensity on the proportion and rate of carbohydrate oxidation and glucoregulatory hormone responses during recovery from exercise. Six physically active participants completed 1 hr of low-intensity (LI; 50% lactate threshold) or moderate-intensity (MI; 100% lactate threshold) exercise on separate days following a randomized counterbalanced design. During exercise and for 6 hr of recovery, samples of expired air were collected to determine oxygen consumption, respiratory exchange ratio, energy expenditure, and substrate oxidation rates. Blood samples were also collected to measure glucoregulatory hormones (catecholamines, GH) and metabolites (glucose, free fatty acids, lactate, pH, and bicarbonate). During exercise, respiratory exchange ratio, energy expenditure, and the proportion and rate of carbohydrate (CHO) oxidation were higher during MI compared with LI. However, during recovery from MI, respiratory exchange ratio and the proportion and rate of CHO oxidation were lower than preexercise levels and corresponding LI. During exercise and early recovery, catecholamines and growth hormone were higher in MI than LI, and there was a trend for higher levels of free fatty acids in the early recovery from MI compared with LI. In summary, CHO oxidation during exercise increases with exercise intensity but there is a preference for CHO sparing (and fat oxidation) during recovery from MI exercise compared with LI exercise. This exercise intensity-dependent shift in substrate oxidation during recovery is explained, in part, by the pattern of change of key glucoregulatory hormones including catecholamines and growth hormone and plasma fatty acid concentrations.

Introduction: In the U.S., sepsis afflicts 1.7 million adults, causing 270,000 deaths each year. Early detection of sepsis could decrease the number of deaths by 92,000 annually and decrease hospital expenditures by 1.5 billion USD. Few prior studies and reviews have presented a holistic understanding of the relationship between machine learning and existing process improvement measures. This study, in addition to discussing machine learning and existing process improvements measures, elaborates on the disadvantages and the barriers to integrating machine learning into the clinic. This article synthesizes previous studies to educate healthcare professionals on effectively managing sepsis by leveraging the benefits of machine learning., Methods: This study used the PubMed database. Search terms include sepsis antibiotics, sepsis process improvement, sepsis machine learning. Our search criteria included previous studies published between January 1, 2017, and February 1, 2022., Results/discussion: Although machine learning algorithms have better predictive capabilities, their effectiveness in the clinical setting is limited as studies show mixed results because the medical staff often fails to intervene. To overcome poor interventional response, clinicians need to work with the facility's IT department to ensure integration into clinical workflow and minimize alert-fatigue. Algorithms should enhance the productivity of clinical teams, not attempt to replace them entirely., Conclusion: Hospitals can employ process improvement measures that effectively utilize machine learning algorithms to ensure integration into clinical workflows. Healthcare professionals can utilize workflow tools in addition to the predictive capabilities of machine learning to enhance clinical decisions in sepsis., (Copyright © 2022 FECA. Publicado por Elsevier España, S.L.U. All rights reserved.)

Importance: Transplantation has transformed into a burgeoning field that is rapidly evolving to optimize organ distribution and survival outcomes. The years since 2012 (the last comprehensive study) have seen changes in transplantation, such as advances in immunotherapy and novel indices, that necessitate an updated analysis of survival benefit., Design: Our goal was to determine the survival benefit for solid-organ transplants in the United Network for Organ Sharing (UNOS) database for a three decade period and provide updates on advancements since 2012. Our retrospective analysis examined data containing U.S. patient records from September 1, 1987, to September 1, 2021., Results: We found that 3,430,272 life-years were saved over our transplant period (4.33 life-years saved per patient); kidney-1,998,492 life-years; liver -767,414; heart-435,312; lung-116,625; pancreas-kidney-123,463; pancreas-30,575; intestine-7901. After matching, 3,296,851 life-years were saved. Life-years saved and median survival increased for all organs between 2012 and 2021. Compared to 2012, median survival increased in kidney (from 12.4 to 14.76 years), liver (from 11.6 to 14.59), heart (9.5 to 11.73), lung (5.2 to 5.63), pancreas-kidney (from 14.5 to 16.88), pancreas (from 13.3 to 16.10). When compared to 2012, the percent transplanted increased in kidney, liver, heart, lung, and intestine, while pancreas-kidney and pancreas show decreased percent transplanted., Conclusion: Our study underscores the tremendous survival benefits of solid organ transplantation (over 3.4 million life-years saved) and shows improvements since 2012. Our study also highlights areas of transplantation, notably pancreas transplants, that may necessitate reinvigorated attention., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Introduction: Review of videos (without electroencephalography) to differentiate epileptic seizures (ES) from non-epileptic spells (NES) may be helpful where epilepsy monitoring is not feasible. Previous studies of video-based diagnosis have suffered from variable accuracy, sensitivity, and specificity., Methods: We systematically reviewed relevant literature in PubMed, Embase, and Web of Science from inception to September 2022, identifying articles that reported on the video-based diagnosis of ES and NES. In primary analysis, for each study, the most expert group was chosen when different groups of reviewers classified the videos (e.g., epilepsy specialists and general neurologists). In secondary analysis, we compared the diagnostic accuracy of different expertise levels (e.g., epileptologists, general neurologists, residents, medical students). Meta-analysis was performed to obtain pooled estimates of reliability measures., Results: From 5245 articles identified, 13 met the inclusion criteria, with cumulative data from 683 patients (696 videos) reviewed by 95 independent reviewers in primary analysis. Video alone had a strong ability to differentiate ES from NES as evidenced by the following metrics- area under the curve- 0.9 (considered "outstanding"), sensitivity- 82.2% (95% Confidence Interval [C.I], 80.2%-84.0%), specificity- 84.7% (C.I., 82.8%-86.5%), and diagnostic odds ratio- 24.7 (C.I., 11.5-52.9). The secondary analysis showed reviewer-dependent accuracy with epileptologists showing the highest accuracy (DOR 81.2, C.I., 90.0%-94.6%)., Conclusions: Video alone has reliable diagnostic performance for differentiating ES from NES. Meta-analysis limitations include inter-study heterogeneity including variable video quality and reviewer expertise. Combined video-EEG remains the gold standard for the diagnosis of epilepsy and NES., Competing Interests: Declaration of Competing Interest All authors declare that they have no conflict of interests., (Copyright © 2023 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Background: The Kidney Donor Risk Index (KDRI) by Rao et al. was developed to measure the quality of kidney allografts. While Rao's KDRI has been found to be a robust measure of kidney allograft survival for adult kidney transplant recipients, many studies have indicated the need to create a distinct pediatric KDRI., Methods: Our retrospective study utilized data from the United Network for Organ Sharing database. We examined 9295 deceased donor recipients' data for age < 18 years from 1990 to 2020. We performed a multivariate Cox regression to determine the significant recipient and transplant factors impacting pediatric kidney allograft survival., Results: Multivariate analysis found 5 donor factors to be independently associated with graft failure or recipient death: age, female sex, anoxia as the cause of death, history of cigarette use, and cold ischemia time. Using receiver operator characteristic (ROC) curve analysis and analyzing the predictive value of each KDRI at 1, 5, and 10 years, the proposed pediatric KDRI had a statistically significant and higher predictive value for pediatric recipients at 5 (0.60 versus 0.57) and 10 years (0.61 versus 0.57) than the Rao KDRI., Conclusions: The proposed pediatric KDRI may provide a more accurate and simpler index to assess the quality of kidney allografts for pediatric recipients. However, due to the mild increase in predictive capabilities over the Rao index, the study serves as a proof of concept to develop a pediatric KDRI. Further studies should focus on increasing the index's predictive capabilities. A higher resolution version of the Graphical abstract is available as Supplementary information., (© 2022. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Background: Hypertension treatment with renin-angiotensin system inhibitors (RASi) presents contradictions about the recovery of damage in cardiovascular autonomic modulation characterized by reduced heart rate variability (HRV) and increased blood pressure variability (BPV). Conversely, the association of RASi with physical training can influence achievements in cardiovascular autonomic modulation., Objective: To investigate the effects of aerobic physical training on hemodynamics and cardiovascular autonomic modulation in hypertensive volunteers untreated and treated with RASi., Methods: A non-randomized controlled trial in which 54 men (≅ 40-60 years old) with a history of hypertension for >2 years were allocated in accordance with their characteristics into three groups: untreated (Control; n=16), treated with type 1 angiotensin II (AT 1 ) receptor blocker (losartan; n=21), and treated with angiotensin-converting enzyme inhibitor (enalapril; n=17). All participants underwent hemodynamic, metabolic, and cardiovascular autonomic evaluation using baroreflex sensitivity (BRS) and spectral analysis of HRV and BPV, before and after 16 weeks of supervised aerobic physical training., Results: The volunteers treated with RASi had lower BPV and HRV, both in the supine position and in the tilt test, with the losartan group having the lowest values. Aerobic physical training increased HRV and BRS in all groups. However, the association of enalapril with physical training appears to be more prominent., Conclusion: Long-term treatment with enalapril and losartan may harm the autonomic modulation of HRV and BRS. Aerobic physical training is essential to promote positive adjustments in the autonomic modulation of HRV and BRS in hypertensive patients treated with RASi, especially with enalapril., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Introduction: Although lung demand continues to outpace supply, 75% of potential donor lungs are discarded without being transplanted in the United States. To identify the discarded cohorts best suited to alleviate the lung shortage and reduce waitlist mortality, we explored changes in survival over time for five marginal donor definitions: age >60 years, smoking history >20 pack-years, PaO 2 /FiO 2  < 300 mmHg, purulent bronchoscopic secretions, and chest radiograph infiltrates., Methods: Our retrospective cohort study separated 27 803 lung recipients in the UNOS Database into three 5-year eras by transplant date: 2005-2009, 2010-2014, and 2015-2019. Multivariable Cox proportional hazards regression and Kaplan-Meier analysis with log-rank test were used to compare survival across the eras., Results: Three definitions-low PaO 2 /FiO 2 , purulent bronchoscopic secretions, and abnormal chest radiographs-did not bear out as truly marginal, demonstrating lack of significantly elevated risk. Advanced donor age demonstrated considerable survival improvement (HR (95% CI): 1.47 (1.26-1.72) in 2005-2009 down to 1.14 (.97-1.35) for 2015-2019), with protective factors being recipients <60 years, moderate recipient BMI, and low Lung Allocation Score (LAS). Donors with smoking history failed to demonstrate any significant improvement (HR (95% CI): 1.09 (1.01-1.17) in 2005-2009 increasing to 1.22 (1.08-1.38) in 2015-2019)., Conclusions: Advanced donor age, previously the most significant risk factor, has improved to near-benchmark levels, demonstrating the possibility for matching older donors to healthier non-elderly recipients in selected circumstances. Low PaO 2 /FiO 2 , bronchoscopic secretions, and abnormal radiographs demonstrated survival on par with standard donors. Significant donor smoking history, a moderate risk factor, has failed to improve., (© 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Our objective was to determine the effects of feeding 25-hydroxyvitamin D 3 [25(OH)D 3 ], or vitamin D 3 (cholecalciferol) on plasma, mineral, and metabolite concentrations, mineral balance, mineral excretion, rumination, energy balance, and milk production of dairy cows. We hypothesized that supplementing 3 mg/d of 25(OH)D 3 during the prepartum period would be more effective than supplementing vitamin D 3 at the National Research Council (2001) levels to minimize calcium imbalance during the transition period and improve milk production of dairy cows. Forty multiparous, pregnant nonlactating-Holstein cows were enrolled in this study. Body weight, body condition score, parity, and milk yield in the previous lactation (mean ± standard deviation) were 661 ± 59.2, 3.46 ± 0.35, 1.79 ± 0.87, and 33.2 ± 6.43 kg/d, respectively. Cows were enrolled into the blocks (n = 20 for each treatment) at 30 d of the expected day of calving to receive an acidogenic diet (373 g/kg of neutral detergent fiber and 136 g/kg of crude protein, dry matter basis; -110 mEq/kg) associated with the treatments: (1) control (CTRL), vitamin D 3 at 0.625 mg/d (equivalent to 25,000 IU of vitamin D 3 /d) or (2) 25(OH)D 3 at 3 mg/d (equivalent to 120,000 IU of vitamin D 3 /d). All cows were fed with the base ration for 49 d after calving. Blood samples were taken on d 7, 0, 1, 2, 21, and 42, relative to calving. No effect of treatment was observed for prepartum dry matter intake or body condition score. A trend for increase of ionized Ca was observed for the cows fed 25(OH)D 3 , compared with the CTRL, but no effect of treatment was detected for total Ca or total P. Feeding 25(OH)D 3 increased colostrum yield. The plasmatic concentration of 25-hydroxyvitamin D 3 was increased with 25(OH)D 3 supplementation. 25-Hydroxyvitamin D 3 supplementation increased plasma glucose concentration at parturition. The postpartum dry matter intake was not influenced by treatments. Feeding 25(OH)D 3 increases milk yield, 3.5% fat-corrected milk, and energy-corrected milk and improves milk yield components in early lactation. Overall, these findings suggest that 25(OH)D 3 at 3 mg/d can improve the energy metabolism and lactation performance, compared with the current-feeding practice of supplementing vitamin D 3 at 0.625 mg/d., (The Authors. Published by Elsevier Inc. and Fass Inc. on behalf of the American Dairy Science Association®. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).)

Bovine anaplasmosis causes considerable economic losses in dairy cattle production systems worldwide, ranging from $300 million to $900 million annually. It is commonly detected through rectal temperature, blood smear microscopy, and packed cell volume (PCV). Such methodologies are laborious, costly, and difficult to systematically implement in large-scale operations. The objectives of this study were to evaluate (1) rumination and activity data collected by Hr-Tag sensors (SCR Engineers Ltd.) in heifer calves exposed to anaplasmosis; and (2) the predictive ability of recurrent neural networks in early identification of anaplasmosis. Additionally, we aimed to investigate the effect of time series length before disease diagnosis (5, 7, 10, or 12 consecutive days) on the predictive performance of recurrent neural networks, and how early anaplasmosis disease can be detected in dairy calves (5, 3, and 1 d in advance). Twenty-three heifer calves aged 119 ± 15 (mean ± SD) d and weighing 148 ± 20 kg of body weight were challenged with 2 × 10 7 erythrocytes infected with UFMG1 strain (GenBank no. EU676176) isolated from Anaplasma marginale. After inoculation, animals were monitored daily by assessing PCV. The lowest PCV value (14 ± 1.8%) and the finding of rickettsia on blood smears were used as a criterion to classify an animal as sick (d 0). Rumination and activity data were collected continuously and automatically at 2-h intervals, using SCR Heatime Hr-Tag collars. Two time series were built including last sequence of -5, -7, -10, or -12 d preceding d 0 or a sequence of 5, 7, 10, or 12 d randomly selected in a window from -50 to -15 d before d 0 to ensure a sequence of days in which PCV was considered normal (32 ± 2.4%). Long short-term memory was used as a predictive approach, and a leave-one-animal-out cross-validation (LOAOCV) was used to assess prediction quality. Anaplasmosis disease reduced 34 and 11% of rumination and activity, respectively. The accuracy, sensitivity, and specificity of long short-term memory in detecting anaplasmosis ranged from 87 to 98%, 83 to 100%, and 83 to 100%, respectively, using rumination data. For activity data, the accuracy, sensitivity, and specificity varied from 70 to 98%, 61 to 100%, and 74 to 100%, respectively. Predictive performance did not improve when combining rumination and activity. The use of longer time-series did not improve the performance of models to predict anaplasmosis. The accuracy and sensitivity in predicting anaplasmosis up to 3 d before clinical diagnosis (d 0) were greater than 80%, confirming the possibility for early identification of anaplasmosis disease. These findings indicate the great potential of wearable sensors in early identification of anaplasmosis diseases. This could positively affect the profitability of dairy farmers and animal welfare., (The Authors. Published by Elsevier Inc. and Fass Inc. on behalf of the American Dairy Science Association®. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).)

Cryptosporidium infections are one of the most prevalent causes of diarrhea in calves and considered to be one of the major sources of economic loss in livestock production. A global trend is currently underway, in identifying natural and sustainable alternatives to support animal husbandry and production. Isoquinoline alkaloids are known for their anti-inflammatory, antimicrobial, and antioxidant properties in the promotion of gut health. Thus, an experiment was designed to evaluate the effects of natural, herbal-based feed isoquinoline alkaloids to support calves experimentally inoculated with Cryptosporidium parvum. Twenty-six calves were randomly divided into control (CN) (n = 13) and treatment (SG) (n = 13) groups. The SG group received 5 g of feed additive in every milk feeding from 1 to 21 days of age. The CN group received milk without any additives. All calves were orally inoculated on the third day of life with 1 × 10 6 Cryptosporidium parvum oocysts. The animals were evaluated daily, from 3 to 30 days of age, for the occurrence, duration, and intensity of diarrhea. Calves with a base deficit of ≥ 9 mEq/L were hydrated to aid recovery. The SG calves showed a higher average weight gain between 14 and 21 days of age, without mortality and with reduced intensity and duration of diarrhea. In contrast, calves in the CN group showed more serious acid-base disorders, required more hydration support, and had a mortality rate of 15.4 %. These results showed that calves supplemented with isoquinoline alkaloids had decreased intensity and duration of symptoms, reduced requirement for supportive therapy, and prevented mortality among animals., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Raine syndrome is a rare, often lethal autosomal recessive condition marked by congenital malformations that range in severity. Considering that several case reports of this syndrome describe cases of stillbirth or perinatal death, information about the clinical presentation and development of this condition in mild, non-lethal cases is lacking. With that in mind, in this case report, we describe the clinical, oro-dental, and skeletal findings of a 14-year-old Brazilian patient diagnosed with a mild form of non-lethal Raine syndrome. This patient has very mild facial dysmorphia, not displaying hypoplastic nose, micrognathia, low set ears or depressed nasal bridge, which is uncommon even in other mild, non-lethal cases of RS. Furthermore, this patient has bilateral brain calcifications and a series of oro-dental abnormalities, such as amelogenesis imperfecta and recurrent periodontal abcesses. Sanger sequencing of genomic DNA identified a homozygous missense variant c.1487C > T at exon 9 of FAM20C (NM&#95;020223.4) in the patient. The patient's mother carries the same variant but is heterozygous. This variant predicts a proline to leucine substitution in position 496 (p.P496L, NP&#95;064608.2) previously reported, which allows for the phenotypic comparison between these cases. This way, this case report calls attention to how differently RS can appear, highlighting the importance of new non-lethal Raine syndrome case reports to help further determine the phenotypic spectrum of this condition., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Previous studies demonstrated that anti-hyperlipidemic drug gemfibrozil acts as NO- and heme-independent activator of NO receptor soluble guanylyl cyclase. A series of new gemfibrozil derivatives were synthesized and evaluated for sGC activation. The structure-activity relationship study identified the positions in gemfibrozil's scaffold that are detrimental for sGC activation and those that are amendable for optimizing modifications. Compared with gemfibrozil, compounds 7c and 15b were more potent activators of cGMP-forming activity of purified sGC and exhibited enhanced relaxation of preconstricted mouse thoracic aorta rings. These studies established the overall framework needed for futher improvement of sGC activators based on gemfibrozil scaffold., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Background: Serious games (SGs) are used as complementary approaches to stimulate patients with dementia. However, many of the SGs use out-of-the-shelf technologies that may not always be suitable for such populations, as they can lead to negative behaviors, such as anxiety, fatigue, and even cybersickness., Objective: This study aims to evaluate how patients with dementia interact and accept 5 out-of-the-shelf technologies while completing 10 virtual reality tasks., Methods: A total of 12 participants diagnosed with dementia (mean age 75.08 [SD 8.07] years, mean Mini-Mental State Examination score 17.33 [SD 5.79], and mean schooling 5.55 [SD 3.30]) at a health care center in Portugal were invited to participate in this study. A within-subject experimental design was used to allow all participants to interact with all technologies, such as HTC VIVE, head-mounted display (HMD), tablet, mouse, augmented reality (AR), leap motion (LM), and a combination of HMD with LM. Participants' performance was quantified through behavioral and verbal responses, which were captured through video recordings and written notes., Results: The findings of this study revealed that the user experience using technology was dependent on the patient profile; the patients had a better user experience when they use technologies with direct interaction configuration as opposed to indirect interaction configuration in terms of assistance required (P=.01) and comprehension (P=.01); the participants did not trigger any emotional responses when using any of the technologies; the participants' performance was task-dependent; the most cost-effective technology was the mouse, whereas the least cost-effective was AR; and all the technologies, except for one (HMD with LM), were not exposed to external hazards., Conclusions: Most participants were able to perform tasks using out-of-the-shelf technologies. However, there is no perfect technology, as they are not explicitly designed to address the needs and skills of people with dementia. Here, we propose a set of guidelines that aim to help health professionals and engineers maximize user experience when using such technologies for the population with dementia., (©Luis Duarte Andrade Ferreira, Henrique Ferreira, Sofia Cavaco, Mónica Cameirão, Sergi Bermúdez i Badia. Originally published in JMIR Serious Games (http://games.jmir.org), 05.08.2020.)

Primary familial brain calcification (PFBC) is a well-known genetic condition that has recently had a surge of autosomal recessive cases. We recently reported a case of autosomal recessive PFBC on a 54-year-old Brazilian patient with a novel homozygous variant on MYORG. Interestingly, that patient also had a series of uncommon signs and symptoms, including Hashimoto's thyroiditis, polyneuropathy, optic nerve head drusen (ONHD), and persistent anemia. We chose to perform whole exome sequencing (WES) to possibly detect other unknown genetic conditions that could explain the extra-neurological findings reported. WES confirmed the presence of the MYORG variant previously reported by us, and determined the presence of a heterozygous nonsense variant on HBB (c.118C > T, p.Q40*), defining a diagnosis of beta-thalassemia. Based on literature review, the new WES finding explains the persistent anemia and polyneuropathy shown by the patient, while still leaving the ONHD and autoimmune thyroiditis without a clear genetic link. This way, we propose that these novel clinical findings could be linked to MYORG, but still encourage further studies to evaluate this possibility.

Purpose: Collagen deposition and myofibroblast differentiation are critical factors related to excessive scarring in ocular surgeries. This study evaluated the anti-fibrotic activity of rosmarinic acid on rabbit Tenon's capsule fibroblasts stimulated with transforming growth factor- β2., Methods: Primary cultures of rabbit Tenon's capsule fibroblasts were treated with various concentrations of rosmarinic acid for 12 h, in the presence and absence of transforming growth factor-β2. After 48 h, the proliferation index of rabbit Tenon's capsule fibroblasts and the differentiation of myofibroblasts were investigated through immunofluorescence staining for proliferating cell nuclear antigen and alpha smooth muscle actin. An automated cell counter and colorimetric metabolic activity assay were used to evaluate cell number and viability. Collagen expression and production were determined by quantitative real-time polymerase chain reaction and hydroxyproline assay, respectively., Results: Unstimulated rabbit Tenon's capsule fibroblasts treated with any concentration of rosmarinic acid exhibited diminished collagen expression (p<0.01) but showed no differences in proliferation index. Transforming growth factor-β2 exposure induced myofibroblast differentiation and increased collagen production. Exposure to rosmarinic acid at 1.0 and 3.0 µM concentrations reduced the proliferation index (p<0.02), as well as the collagen expression and hydroxyproline content (p<0.05). Exposure to 3.0 µM rosmarinic acid reduced viability (p=0.035) in unstimulated rabbit Tenon's capsule fibroblasts and cell numbers (p=0.001) in both stimulated and unstimulated rabbit Tenon's capsule fibroblast cultures., Conclusions: Exposure to 1.0 µM rosmarinic acid was noncytotoxic and led to reduced collagen expression and proliferation of stimulated rabbit Tenon's capsule fibroblasts. These findings suggest that rosmarinic acid is a relatively non-injurious anti-fibrotic compound to rabbit Tenon's capsule fibroblasts, with potential application as an adjunctive agent in ocular procedures, particularly in glaucoma surgeries.

Primrose syndrome (PRIMS), a rare genetic disorder with several clinical findings including intellectual disability, macrocephaly, typical facial features, and muscle wasting, is caused by heterozygous variants in the ZBTB20 gene. We report the cases of two males diagnosed with PRIMS at different ages, emphasizing the likely progressive nature of the disorder, as well as the differences and similarities of presentation during infancy and adulthood. Patient 1 is a 2-year-old American male with a medical history marked by impaired hearing, developmental delays, and fainting spells. Patient 2 is a 28-year-old Brazilian male, who presents with a phenotype similar to that seen in Patient 1 with additional features of ectopic calcifications and prominent muscular and skeletal abnormalities. Additionally, Patient 2 has a history of fainting spells and diminished body height and weight, with the latter features having only been reported in one PRIMS patient so far. Both Patients 1 and 2 were found to carry heterozygous likely pathogenic missense variants, detected in the last coding exon of ZBTB20 (c.1822T>C, p.Cys608Arg, de novo, and c.1873A>G, p.Met625Val, respectively), consistent with PRIMS. Overall, these case reports highlight PRIMS's likely progressive nature and contribute to the understanding of the natural history of this condition., (© 2019 Wiley Periodicals, Inc.)

Introduction: Pseudomonas aeruginosa, an important pathogen globally, presents several resistance mechanisms. This study aimed to investigate the presence of bla GES in clinical isolates of Pseudomonas aeruginosa obtained from various clinical specimens from patients admitted to three different hospitals in Recife, Brazil. The Guiana extended spectrum beta-lactamase (GES) enzymes are responsible for conferring broad spectrum resistance to beta-lactam drugs, including the carbapenems., Methods: A total of 100 carbapenem-resistant P. aeruginosa isolates underwent polymerase chain reaction (PCR) testing to identify bla GES, bla KPC, bla SPM-1, bla IMP, and bla VIM. Additionally, PCR products positive for bla GES were sequenced. The clonal profiles of these same isolates were then determined by means of enterobacterial repetitive intergenic consensus (ERIC)-PCR analysis., Results: PCR analysis revealed that four isolates harbored bla GES; DNA sequencing showed that two harbored bla GES-1 and two bla GES-11. Beta-lactamase genes bla SPM-1, bla IMP, bla VIM, and bla KPC were investigated; none of these genes was detected. Automated susceptibility testing methods (Vitek®2, bioMérieux) showed that the bla GES-1-positive isolates were only susceptible to polymyxin B. The patterns obtained with ERIC-PCR methods showed clonal relationship between the two isolates that harbored bla GES-11, whereas different clonal profiles were found in the isolates harboring bla GES-1., Conclusions: We detected the presence of bacterial isolates positive for two different variants of the enzyme GES in three different hospitals from Recife, Brazil. These enzymes have a great capacity for dissemination among Gram-negative bacteria and confer broad-spectrum resistance to beta-lactam antibiotics and to the carbapenems.

The effect of electric field on ascorbic acid and carotenoid degradation in acerola pulp during ohmic heating was evaluated. Ascorbic acid kinetic degradation was evaluated at 80, 85, 90 and 95°C during 60 min of thermal treatment by ohmic and conventional heating. Carotenoid degradation was evaluated at 90 and 95°C after 50 min of treatment. The different temperatures evaluated showed the same effect on degradation rates. To investigate the influence of oxygen concentration on the degradation process, ohmic heating was also carried out under rich and poor oxygen modified atmospheres at 90°C. Ascorbic acid and carotenoid degradation was higher under a rich oxygen atmosphere, indicating that oxygen is the limiting reagent of the degradation reaction. Ascorbic acid and carotenoid degradation was similar for both heating technologies, demonstrating that the presence of the oscillating electric field did not influence the mechanisms and rates of reactions associated with the degradation process., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

This study evaluated the characteristics of rice starch and protein obtained by a fast alkaline extraction method on rice flour (RF) derived from broken rice. The extraction was conducted using 0.18% NaOH at 30°C for 30min followed by centrifugation to separate the starch rich and the protein rich fractions. This fast extraction method allowed to obtain an isoelectric precipitation protein concentrate (IPPC) with 79% protein and a starchy product with low protein content. The amino acid content of IPPC was practically unchanged compared to the protein in RF. The proteins of the IPPC underwent denaturation during extraction and some of the starch suffered the cold gelatinization phenomenon, due to the alkaline treatment. With some modifications, the fast method can be interesting in a technological point of view as it enables process cost reduction and useful ingredients obtention to the food and chemical industries., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Visceral Leishmaniasis (VL) has spread to many urban centers worldwide. Dogs are considered the main reservoir of VL, because canine cases often precede the occurrence of human cases. Detection and euthanasia of serologically positive dogs is one of the primary VL control measures utilized in some countries, including Brazil. Using accurate diagnostic tests can minimize one undesirable consequence of this measure, culling false-positive dogs, and reduce the maintenance of false-negative dogs in endemic areas. In December 2011, the Brazilian Ministry of Health replaced the ELISA (EIE CVL) screening method and Indirect Immunofluorescence Test (IFI CVL) confirmatory method with a new protocol using the rapid DPP CVL screening test and EIE CVL confirmatory test. A study of diagnostic accuracy of these two protocols was done by comparing their performance using serum samples collected from a random sample of 780 dogs in an endemic area of VL. All samples were evaluated by culture and real time PCR; 766 out of the 780 dogs were tested using the previous protocol (IFI CVL + EIE CVL) and all 780 were tested using the current protocol (DPP CVL + EIE CVL). Performances of both diagnostic protocols were evaluated using a latent class variable as the gold standard. The current protocol had a higher specificity (0.98 vs. 0.95) and PPV (0.83 vs. 0.70) than the previous protocol, although sensitivity of these two protocols was similar (0.73). When tested using sera from asymptomatic animals, the current protocol had a much higher PPV (0.63 vs. 0.40) than the previous protocol (although the sensitivity of either protocol was the same, 0.71). Considering a range of theoretical CVL prevalences, the projected PPVs were higher for the current protocol than for the previous protocol for each theoretical prevalence value. The findings presented herein show that the current protocol performed better than previous protocol primarily by reducing false-positive results.

The objective of this study was to use pinhão derivatives, starch and coat extract, as new natural ingredients to develop cosmetic formulations. Two types of formulation, gel and emulgel, and their controls were developed. The formulations were characterized by stability studies using thermal stress. The parameters analyzed were resistance to centrifugation, pH, spreadability, rheology, content of phenolic compounds and antioxidant activity. Sensory analysis was also performed to verify the acceptability of the ingredients to potential consumers. The pH was kept the same after heating/freezing cycles for all formulations, and the formulations showed stability by resistance to centrifugation. The formulations did not induce any skin irritation or cutaneous pH alteration. The pinhão starch addition improved spreadability stability and increased viscosity when compared with control formulations. The pinhão coat extract used in these formulations is a good source of phenolic compounds and antioxidant activity. Moreover, sensory analysis indicates that the emulgel formulation is the best vehicle for adding pinhão starch and pinhão coat extract., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

This study investigated the non-thermal effects of electricity on anthocyanin degradation during ohmic heating of jaboticaba juice. For this, temperature profiles during conventional and ohmic heating processes were matched, and the degradation kinetics of anthocyanins were compared at temperatures ranging from 70 to 90°C. The monomeric anthocyanin content was quantified by UV-Visible spectroscopy using the pH-differential method. Anthocyanin degradation was fitted to a first-order model. The rate constants ranged from 1.7 to 7.5 × 10(-3)min(-1) and from 1.8 to 7.6 × 10(-3)min(-1) for ohmic and conventional heating, respectively. The analysis of variance (α=0.05) showed no significant differences between rate constants of the ohmic and conventional heating at the same temperatures. All kinetic and thermodynamic parameters evaluated showed similar values for both technologies. These results indicate that the presence of the oscillating electric field did not affect the degradation rates of anthocyanins during ohmic heating., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Host tissues affected by Leishmania infantum have differing degrees of parasitism. Previously, the use of different biological tissues to detect L. infantum DNA in dogs has provided variable results. The present study was conducted to evaluate the accuracy of molecular diagnostic testing (qPCR) in dogs from an endemic area for canine visceral leishmaniasis (CVL) by determining which tissue type provided the highest rate of parasite DNA detection. Fifty-one symptomatic dogs were tested for CVL using serological, parasitological and molecular methods. Latent class analysis (LCA) was performed for accuracy evaluation of these methods. qPCR detected parasite DNA in 100% of these animals from at least one of the following tissues: splenic and bone marrow aspirates, lymph node and skin fragments, blood and conjunctival swabs. Using latent variable as gold standard, the qPCR achieved a sensitivity of 95.8% (CI 90.4-100) in splenic aspirate; 79.2% (CI 68-90.3) in lymph nodes; 77.3% (CI 64.5-90.1) in skin; 75% (CI 63.1-86.9) in blood; 50% (CI 30-70) in bone marrow; 37.5% (CI 24.2-50.8) in left-eye; and 29.2% (CI 16.7-41.6) in right-eye conjunctival swabs. The accuracy of qPCR using splenic aspirates was further evaluated in a random larger sample (n = 800), collected from dogs during a prevalence study. The specificity achieved by qPCR was 76.7% (CI 73.7-79.6) for splenic aspirates obtained from the greater sample. The sensitivity accomplished by this technique was 95% (CI 93.5-96.5) that was higher than those obtained for the other diagnostic tests and was similar to that observed in the smaller sampling study. This confirms that the splenic aspirate is the most effective type of tissue for detecting L. infantum infection. Additionally, we demonstrated that LCA could be used to generate a suitable gold standard for comparative CVL testing.

In this work, the influence of the electric field frequency and solids content on the degradation kinetics of ascorbic acid during ohmic heating of acerola pulp and acerola serum was investigated. The degradation percentage of ascorbic acid in the pulp after 120 min of heating varied between 12 and 17%. For the serum, the degradation percentage was in the range of 13 and 18%. The results were fitted to the first-order model, and the kinetic rate constants ranged from 1.1 to 1.6×10(-3) min(-1) and from 1.1 to 1.5×10(-3) min(-1) for pulp and serum, respectively. D values ranged between 1480 and 2145 min for the pulp and between 1524 and 1951 min for the serum. A distinct behavior between the kinetic parameters of the pulp and serum in electric field frequencies ranging from 10 to 1000 Hz indicates that the presence of distinct amounts and types of solids might affect the rate of the electron transfer in electrochemical reactions. These variables may also affect the polarization process stimulated by the oscillating electric field. The non-achievement of the equilibrium of the polarization process may have an influence on oxidation reactions, affecting the predisposition to hydrogen donation from the ascorbic acid molecule.

The aim of this study is to estimate the prevalence of HIV/HBV and HIV/HCV coinfections among AIDS cases reported in Brazil, and to describe the epidemiological profile of these cases. Coinfection was identified through probabilistic record linkage of the data of all patients carrying the HIV virus recorded as AIDS patients and of those patients reported as carriers of hepatitis B or C virus in various databases from the Brazilian Ministry of Health from 1999 to 2010. In this period 370,672 AIDS cases were reported, of which 3,724 were HIV/HBV coinfections. Women are less likely to become coinfected than men and the chance of coinfection increases with age. This study allowed an important evaluation of HBV/HIV and HCV/HIV coinfections in Brazil using information obtained via merging secondary databases from the Ministry of Health, without conducting seroprevalence research. The findings of this study might be important for planning activities of the Brazilian epidemiologic surveillance agencies.

Objective: To determine whether performing a 10-s sprint after moderate-intensity exercise increases the amount of carbohydrate required to maintain euglycemia and prevent late-onset postexercise hypoglycemia relative to moderate-intensity exercise alone., Research Design and Methods: Seven individuals with type 1 diabetes underwent a hyperinsulinemic-euglycemic clamp and performed 30 min of moderate-intensity exercise on two separate occasions followed by either a 10-s maximal sprint effort or no sprint. During the following 8 h, glucose infusion rate to maintain euglycemia and rates of glucose appearance and disappearance were measured continuously., Results: In response to exercise and throughout the 8-h recovery period, there were no differences in glucose infusion rate, blood glucose levels, plasma insulin concentrations, and rates of glucose appearance and disappearance between the two experimental conditions (P > 0.05)., Conclusions: A 10-s sprint performed after 30 min of moderate-intensity exercise does not affect the amount of carbohydrate required to maintain euglycemia postexercise in individuals with type 1 diabetes.

Context: Exercise increases the risk of hypoglycemia in type 1 diabetes., Objective: Recently we reported a biphasic increase in glucose requirements to maintain euglycemia after late-afternoon exercise, suggesting a unique pattern of delayed risk for nocturnal hypoglycemia. This study examined whether this pattern of glucose requirements occurs if exercise is performed earlier in the day., Design, Participants, and Intervention: Ten adolescents with type 1 diabetes underwent a hyperinsulinemic euglycemic glucose clamp on 2 different occasions during which they either rested or performed 45 minutes of moderate-intensity exercise at midday. Glucose was infused to maintain euglycemia for 17 hours after exercise., Main Outcome Measures: The glucose infusion rate (GIR) to maintain euglycemia, glucose rates of appearance and disappearance, and levels of counterregulatory hormones were compared between conditions., Results: GIRs to maintain euglycemia were not significantly different between groups at baseline (9.8 ± 1.4 and 9.5 ± 1.6 g/h before the exercise and rest conditions, respectively) and did not change in the rest condition throughout the study. In contrast, GIR increased more than 3-fold during exercise (from 9.8 ± 1.4 to 30.6 ± 4.7 g/h), fell within the first hour of recovery, but remained elevated until 11 hours after exercise before returning to baseline levels., Conclusions: The pattern of glucose requirements to maintain euglycemia in response to moderate-intensity exercise performed at midday suggests that the risk of exercise-mediated hypoglycemia increases during and for several hours after moderate-intensity exercise, with no evidence of a biphasic pattern of postexercise risk of hypoglycemia.

Degradation kinetics of monomeric anthocyanins in acerola pulp during thermal treatment by ohmic and conventional heating was evaluated at different temperatures (75-90°C). Anthocyanin degradation fitted a first-order reaction model and the rate constants ranged from 5.9 to 19.7 × 10(-3)min(-1). There were no significant differences between the rate constants of the ohmic and the conventional heating processes at all evaluated temperatures. D-Values ranged from 116.7 to 374.5 for ohmic heating and from 134.9 to 390.4 for conventional heating. Values of the free energy of inactivation were within the range of 100.19 and 101.35 kJ mol(-1). The enthalpy of activation presented values between 71.79 and 71.94 kJ mol(-1) and the entropy of activation ranged from -80.15 to -82.63 J mol(-1)K(-1). Both heating technologies showed activation energy of 74.8 kJ mol(-1) and close values for all thermodynamic parameters, indicating similar mechanisms of degradation., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Native and hydrolyzed pinhão starches were used as coating materials for β-carotene microencapsulation by freeze-drying. The purpose of the present study was to evaluate the stability of β-carotene encapsulated under three different conditions: in the presence of ultraviolet light at 25±2 °C, in the dark at 25±2 °C and in the dark at 10.0±0.2 °C. The color of the samples was also analyzed. Microcapsules prepared with native starch showed the lowest stability during storage. In contrast, microcapsules encapsulated with 12 dextrose equivalent (DE) hydrolyzed starch exhibited the highest stability. First-order kinetic and Weibull models were applied to describe the degradation of β-carotene over time. The R(2) values of the Weibull model were greater than those of the first-order kinetic model. Moreover, multivariate analyses (principal component and cluster analyses) were also conducted., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

The objective of this study was to learn the level of satisfaction that nurses have about their work. Participants were 34 nurses, who answered the Professional Satisfaction Index (PSI), translated and validated for the Portuguese language and adapted to home care. The following six components were analyzed: Interaction, Autonomy, Remuneration, Professional Status, Organizational Norms, and Working Requirements. The PSI found in the study was 13.19. This was a low value, considering the possible variation from 0.9 to 37.1, though it was greater than the satisfaction indexes found in other nurse studies.

Different vascular abnormalities have been reported under the denomination of "persistence of the fifth aortic arch." Detailed studies on experimental embryology raised the discussion about the existence of the fifth aortic arch as an embryological structure, both in humans and mammals. In 1969 the Van Praaghs described the occurrence of double left aortic arch, denominating such anomaly as persistence of the fifth arch. We describe here a female patient showing the presence of an anomalous vessel in parallel with the aortic arch. The finding was occasional, during a preoperative evaluation for cholecystectomy., (© 2011, Wiley Periodicals, Inc.)