Miracle-dz/UniFAR-FTUs huggingface dataset:
Clinical characteristics and outcomes in childhood-onset hypertrophic cardiomyopathy

Abstract Aims Childhood-onset hypertrophic cardiomyopathy (HCM) is far less common than adult-onset disease, thus natural history is not well characterized. We aim to describe the characteristics and outcomes of childhood-onset HCM. Methods and results We performed an observational cohort study of 7677 HCM patients from the Sarcomeric Human Cardiomyopathy Registry (SHaRe). Hypertrophic cardiomyopathy patients were stratified by age at diagnosis [<1 year (infancy), 1–18 years (childhood), >18 years (adulthood)] and assessed for composite endpoints reflecting heart failure (HF), life-threatening ventricular arrhythmias, atrial fibrillation (AF), and an overall composite that also included stroke and death. Stratifying by age of diagnosis, 184 (2.4%) patients were diagnosed in infancy; 1128 (14.7%) in childhood; and 6365 (82.9%) in adulthood. Childhood-onset HCM patients had an ∼2%/year event rate for the overall composite endpoint, with ventricular arrhythmias representing the most common event in the 1st decade following baseline visit, but HF and AF becoming more common by the end of the 2nd decade. Sarcomeric variants were more common in childhood-onset HCM (63%) and carried a worse prognosis than non-sarcomeric disease, including a greater than two-fold increased risk of HF [HRadj 2.39 (1.36–4.20), P = 0.003] and 67% increased risk of the overall composite outcome [HRadj 1.67 (1.16–2.41), P = 0.006]. When compared with adult-onset HCM, childhood-onset was 36% more likely to develop life-threatening ventricular arrhythmias [HRadj 1.36 (1.03–1.80)] and twice as likely to require transplant or ventricular assist device [HRadj 1.99 (1.23–3.23)]. Conclusion Patients with childhood-onset HCM are more likely to have sarcomeric disease, carry a higher risk of life-threatening ventricular arrythmias, and have greater need for advanced HF therapies. These findings provide insight into the natural history of disease and can help inform clinical risk stratification.
PCIT engagement and persistence among child welfare-involved families: Associations with harsh parenting, physiological reactivity, and social cognitive processes at intake

Abstract Parent-Child interaction therapy (PCIT) has been shown to improve positive, responsive parenting and lower risk for child maltreatment (CM), including among families who are already involved in the child welfare system. However, higher risk families show higher rates of treatment attrition, limiting effectiveness. In N = 120 child welfare families randomized to PCIT, we tested behavioral and physiological markers of parent self-regulation and socio-cognitive processes assessed at pre-intervention as predictors of retention in PCIT. Results of multinomial logistic regressions indicate that parents who declined treatment displayed more negative parenting, greater perceptions of child responsibility and control in adult–child transactions, respiratory sinus arrhythmia (RSA) increases to a positive dyadic interaction task, and RSA withdrawal to a challenging, dyadic toy clean-up task. Increased odds of dropout during PCIT's child-directed interaction phase were associated with greater parent attentional bias to angry facial cues on an emotional go/no-go task. Hostile attributions about one's child predicted risk for dropout during the parent-directed interaction phase, and readiness for change scores predicted higher odds of treatment completion. Implications for intervening with child welfare-involved families are discussed along with study limitations.
Reliability–Based Optimization using Surface Response Methodology to Split Heavy Petroleum Fractions by Centrifugal Molecular Distillation Process

The present work aimed to develop an experimental and computational study for optimizing the centrifugal molecular distillation process to split heavy petroleum fractions. On the basis of the balance equations and Langmuir–Knudsen equation, a mathematical model was proposed. The influence of the evaporator temperature (EVT), the feed flow rate (Q) and the interactions between them, on the overall distillate mass flow rate (D) and the distillate yield (%D) was analyzed. A full 22 factorial central plus star rotatable (α = ±√2) composite design was performed in the experimental range from 423.15 to 603.15 K for EVT and from 1.473 to 4.418 kg · h−1 for Q. The optimized conditions, using response surface methodology, established that the EVT must range from 540 to 600 K and the Q from 1.5 to 3.5 kg · h−1. The comparison of the experiment results with the predicted model results shows an acceptable qualitative agreement between the experiment and simulated data.
The Relationship Between Body Awareness and Self-care in Patients With Heart Failure

Background Recognizing changing symptoms is challenging for patients with heart failure (HF), especially older patients. Body awareness involves an attentional focus on and awareness of internal bodily changes. Patients with poor body awareness are unlikely to recognize subtle bodily changes, which may result in improper self-care. Despite the potential benefits of body awareness, its contribution to HF management has not been examined. Aims The aims of this study were to examine the relationship between body awareness and self-care in patients with HF and explore whether this relationship varies by age. Methods Patients (N = 136) completed the Body Awareness Questionnaire and 2 scales of the Self-care of HF Index (maintenance and management). Linear regression models were constructed to explore the relationship between body awareness and self-care after controlling for covariates. The moderation effect of age on the relationship between body awareness and self-care was examined using the PROCESS macro. Results Body awareness was associated with self-care maintenance (unstandardized coefficient = 0.19; 95% confidence interval, 0.05–0.34) but not management. When the interaction effect of body awareness and age was entered in the regression model, neither self-care maintenance nor management was related to this interaction term. Conclusion We found that body awareness is conducive to self-care maintenance but not self-care management in patients with HF, and the relationship between body awareness and self-care did not vary by age. Our findings suggest that relying on internal body sensations may be insufficient to facilitate self-care management. Thus, other strategies along with the strategies to enhance body awareness are necessary to improve the full process of self-care in HF patients.
Alcohol Reduction to Reduce Relapse in Acute Alcoholic Pancreatitis—Missed Opportunities

Abstract Aim Resuming drinking is a main contributant to recurrence in alcoholic pancreatitis. We assessed current clinical practice in the Netherlands regarding alcohol in managing patients with a first episode of acute alcoholic pancreatitis. Methods A survey was distributed to 35 hospitals affiliated with the Dutch Pancreatitis Study Group. We evaluated current support based on various components of brief interventions, the participation of psychosocial healthcare providers, the cooperation with the primary care physicians and the presence of a protocol and its implementation. Results The response rate was 100% (n = 35). Psychoeducation is the most frequently performed intervention in current support treatment (97% of hospitals). In 17% of hospitals, healthcare providers with a psychosocial background routinely participate in current support treatment; 37% of hospitals create an individual treatment plan in which goals regarding alcohol cessation are specified and only 46% of hospitals provide the primary care physician with specific discharge information; 31% of hospitals indicate that the treatment is uniformly performed within their division of Gastroenterology. Protocols are available in 3% of the hospitals surveyed. Opportunities to involve the patient’s social network were not given sufficient priority. Conclusion Among Dutch hospitals, there is no routine management strategy with regard to enhancing treatment for heavy alcohol use in alcoholic pancreatitis patients. There is a need to test a validated support program in randomized studies. Meanwhile, possible opportunities for effecting change are often missed.
Emerging infectious encephalitides

Purpose of review The COVID-19 pandemic has cast increased attention on emerging infections. Clinicians and public health experts should be aware of emerging infectious causes of encephalitis, mechanisms by which they are transmitted, and clinical manifestations of disease. Recent findings A number of arthropod-borne viral infections -- transmitted chiefly by mosquitoes and ticks -- have emerged in recent years to cause outbreaks of encephalitis. Examples include Powassan virus in North America, Chikungunya virus in Central and South America, and tick-borne encephalitis virus in Europe. Many of these viruses exhibit complex life cycles and can infect multiple host animals in addition to humans. Factors thought to influence emergence of these diseases, including changes in climate and land use, are also believed to underlie the emergence of the rickettsial bacterium Orientia tsutsugamushi, now recognized as a major causative agent of acute encephalitis syndrome in South Asia. In addition, the COVID-19 pandemic has highlighted the role of bats as carriers of viruses. Recent studies have begun to uncover mechanisms by which the immune systems of bats are poised to allow for viral tolerance. Several bat-borne infections, including Nipah virus and Ebola virus, have resulted in recent outbreaks of encephalitis. Summary Infectious causes of encephalitis continue to emerge worldwide, in part because of climate change and human impacts on the environment. Expansion of surveillance measures will be critical in rapid diagnosis and limiting of outbreaks in the future.
Influence of iodine cell quality onto the stability and absolute frequency shifts of laser etalons

Frequency doubled Nd:YAG lasers are often the option with stabilization to the saturated absorption in molecular iodine featuring good signal-to-noise ratio at the 532 nm. Purity of iodine in the absorption cell is one of the most important factor how to achieve results in optical frequencies corresponding to theoretical values. We present results of measurement of purity of sets of iodine cells made at our institute. The purity was tested by improved method based on measurement of induced fluorescence and evaluation by the Stern-Volmer formula. Frequency-doubled Nd:YAG lasers stabilized with these cells were compared to evaluate their frequency shifts. The absolute frequencies of selected iodine hyperfine transitions were measured in direct laser frequency comparison with the reproducibility well below the kHz level. The results indicating the iodine cell purity are presented with relation to the absolute frequency shifts. This not only highlights the influence of iodine cell quality onto the stability and absolute frequency of lasers etalons but also shows the way towards improvements of the iodine cell manufacturing technology.
WWTR1(TAZ)-CAMTA1 gene fusion is sufficient to dysregulate YAP/TAZ signaling and drive epithelioid hemangioendothelioma tumorigenesis

Here, Seavey et al. developed a conditional epithelioid hemangioendothelioma (EHE) mouse model whereby Wwtr1-Camta1 is controlled by the endogenous transcriptional regulators upon Cre activation by using an approach to target the Wwtr1-Camta1 gene fusion to the Wwtr1 locus. Their results demonstrate that TAZ-CAMTA1 is sufficient to drive EHE formation and that this EHE mouse model is a valid preclinical model for understanding the role of TAZ dysregulation in cancer.
Ethics Consultation in U.S. Hospitals: Opinions of Ethics Practitioners

Abstract To design effective strategies to improve ethics consultation (EC) practices, it is important to understand the views of ethics practitioners. Previous U.S. studies of ethics practitioners have overrepresented the views of academic bioethicists. To help inform EC improvement efforts, we surveyed a random stratified sample of U.S. hospitals, examining ethics practitioners’ opinions on EC in general, on their own EC service, on strategies to improve EC, and on ASBH practice standards. Respondents across all categories of hospitals had very positive perceptions of their own ethics consultation service (ECS) and few concerns about quality. Our findings suggest that the ethics-related needs of small, rural, non-teaching hospitals may be very different from those of academic medical centers, and therefore, different approaches to addressing ethical issues might be warranted.
Mudanças nos compostos bioativos e atividade antioxidante de pimentas da região amazônica

Embrapa Amazonia Oriental owns and active bank of peppers with different genotypes of the genus Capsicum , which have not been analyzed for their functional characteristics and antioxidant capacity yet. This study aimed to determine the contents of ascorbic acid, phenolic compounds, total carotenoids and total antioxidant activity in immature and mature fruits of Capsicum peppers genotypes. The levels of vitamin C (100.76-361.65 mg 100 g -1 in immature fruits and 36.70-157.76 mg 100 g -1 in mature fruits) decreased with fruit maturity. Total carotenoids were not detected in immature fruits, however, values of 73.80-1349.97 m g g -1 were observed for mature fruits, according to the genotype. The contents of phenolic compounds increased in mature fruits (147.40-718.64 mg GAE 100 g -1 ) for eight out of nine genotypes evaluated. The pepper fruits showed significant antioxidant activity (55.02-92.03 m M trolox g -1 in immature fruits and 39.60-113.08 m M trolox g -1 in mature fruits). It was concluded that the degree of fruit maturation affected the contents of bioactive compounds of the genotypes evaluated. IAN-186301 and IAN-186324, for total carotenoids; IAN-186301, IAN-186311, IAN-186312 and IAN-186313, for ascorbic acid; IAN-186304 and IAN-186311, for phenolic compounds; and IAN-186311, for antioxidant activity, were considered the most promising genotypes, with potential to be used in breeding programs.
NonSurgical Embryo Recovery from Estrus-Synchronized or Superovulated Morada Nova Ewes: A Feasible Strategy for Sheep Embryo Banking.

This study assessed the feasibility of in vivo embryo production and nonsurgical embryo recovery (NSER) in Morada Nova ewes (an endangered native Brazilian breed of sheep) subjected to different estrus synchronization and/or superovulation protocols. Ewes received intravaginal sponges soaked with 60 mg medroxyprogesterone acetate (MAP), which were kept in place for six (G6; n = 12), nine (G9; n = 12), or 12 (G12; n = 12) days. Half of the ewes in each group remained estrus synchronized only (SYNCH) and the other half was superovulated (SOV) with 133 mg porcine follicle-stimulating hormone (pFSH). There were no differences (p > 0.05) in antral follicle counts determined with ultrasonography 60 hours before MAP sponge removal (or at the time of the first pFSH dose) among G6 (6.4 ± 0.9), G9 (6.2 ± 0.7), and G12 (5.5 ± 0.6). Estrus responses and NSER success rates did not vary (p > 0.05) among the three progestin-treatment groups of ewes for either estrus-induced or superovulated animals. The onset of estrus occurred 10-12 hours later (p < 0.01) in G9SYNCH ewes compared with G6SYNCH and G12SYNCH, and the duration of estrus was ∼19 hours greater (p < 0.01) in G9SOV than in G6SOV. The average duration of the NSER procedure was 32.6 ± 1.3 minutes. At least one structure was recovered in 85.7% of synchronized and in 87.5% of superovulated ewes. Viable embryo recovery rates were also similar (p > 0.05) for G6 (1.0 ± 0.3 and 2.5 ± 1.5), G9 (1.3 ± 0.5 and 4.8 ± 2.0), and G12 groups (1.0 ± 0.3 and 4.8 ± 2.3; estrus-synchronized and superovulated ewes, respectively). In conclusion, progestogen pretreatment of different durations and NSER can be employed in Morada Nova ewes, resulting in reasonable viable embryo recovery rates in both estrus-synchronized and superovulated animals. Therefore, both techniques are suitable for use in commercial settings as well as small ruminant conservation programs.
Cardiovascular and metabolic consequences in patients with asymptomatic adrenal adenomas

Purpose of review The incidence of adrenal tumors has increased over the past 20 years, most of which are incidentally discovered nonfunctioning adenomas (NFA) and tumors with mild autonomous cortisol secretion (MACS). This review aimed to summarize recent progress in understanding cardiometabolic risk in patients with NFA and MACS and to provide updates on the effect of treatment on improving outcomes in this population. Recent findings NFA and MACS are associated with adverse cardiovascular risk factors and metabolic derangements, which are likely mediated by excessive glucocorticoid secretion. Recent studies showed significantly higher prevalence of hypertension, impaired glucose metabolism, obesity, and dyslipidemia in patients with NFA and MACS. Adrenalectomy may improve comorbidities in selected patients. Summary Asymptomatic adrenal adenomas are common and are associated with adverse cardiometabolic changes. In selected patients, adrenalectomy may reduce cardiometabolic risk and improve clinical outcomes.
Practical Aspects of Transitioning from Intravenous to Subcutaneous Immunoglobulin Therapy in Neuromuscular Disorders

ABSTRACT: Recent evidence shows that subcutaneous immunoglobulin (SCIG) is as efficacious as intravenous immunoglobulin (IVIG) and has a better safety profile and acceptance rate among patients with neuromuscular disorders who require maintenance IVIG treatment. Awareness of the practical aspects of patient selection, enrollment, dose calculation, administration, and follow-up would help physicians coordinate a smooth and seamless transition from IVIG to SCIG. SCIG is ideally offered to patients having intolerable side effects during IVIG or wearing-off effect and in those keen for treatment autonomy. The weekly dose of SCIG is calculated by multiplying the maintenance dose of IVIG by the dose adjustment factor and dividing by the interval between IVIG in weeks and is initiated 1 week after the last dose of IVIG. The physician places the order for the SCIG and the clinic nurse or the physician refers the patient to the home care nursing program for further education and training. The necessary supplies are dispatched to the patient who would also collect the SCIG from the transfusion center of the nearest hospital. The patient is educated on assembling and administering the infusion, and home visits are continued until the patient or caregiver is confident. Regular follow-up with the patient is maintained to assess treatment response and side effects if any. With a smooth transition, most patients have excellent tolerance to SCIG and in our experience seldom request switching back to IVIG. Transitioning patients from IVIG to SCIG offers several advantages and thus, in general, is preferable for multiple stakeholders.
Use of different chelating agents for heavy metal extraction from contaminated harbour sediment

This study presents the results of laboratory-scale experimental tests of contaminant extraction from marine sediment slurries. The sediment was collected in a harbour situated in a high-density industrial area, characterised by a high pollution level of heavy metals. The objective of this study was to investigate the effectiveness of selected chelating agents (rhamnolipids, EDDS, EDTA and citric acid) in heavy metal removal from contaminated sediments. To this aim, contamination of the sediment used in this experiment was artificially increased to reach final values of 1250 mg·kg−1 Cu and 2026 mg·kg−1 Pb. The investigated parameters during the washing tests were chelant concentration and overall washing time. To evaluate the sediment characteristics, COD and acid digestion were performed, whereas total organic carbon and heavy metal concentration were determined on the solutions extracted. The results show that the use of EDDS and EDTA gave good extraction efficiencies (up to 95%), unlike the lower removal rates achieved by using citric acid and rhamnolipids. In addition, major cations, mainly Fe and Ca, were found to be strong competitors with the target metals for metal–ligand complex formation.
Measurements of ClO and O3 from 21°N to 61°N in the lower stratosphere during February 1988: Implications for heterogeneous chemistry

The decline in stratospheric ozone at northern midlatitudes in wintertime may be caused by chlorine photochemistry that has been enhanced by heterogeneous reactions. We examine the possibility that the heterogeneous reaction of N2O5 on sulfate aerosols is the cause of this decadal ozone decline by comparing ClO and O3 measurements made in the lower stratosphere during February, 1988, with results from a two-dimensional model. At midlatitudes, the abundances, latitudinal, and seasonal gradients of the observed ClO are similar to the results of a model with heterogeneous chemistry, but are in strong disagreement with the results from the model with only gas-phase chemistry. At low latitudes, agreement is best with the results of the model with only gas-phase chemistry. These limited observations indicate that the amount of reactive chlorine is being enhanced, and that heterogeneous chemistry is a likely cause, but the details of the heterogeneous chemistry and the possibility of other mechanisms still need to be considered.
New Fossil Histerid Species from Cretaceous Burmese Amber (Coleoptera: Histeridae)

Abstract Four newly discovered Cretaceous genera and species are established in the family Histeridae. Phasmister cristatus Caterino, new genus, new species and Carinumerus maddisoni Caterino, new genus, new species are described in the Onthophilinae. Yethiha peregrina Caterino, new genus, new species and Druantia aeterna Caterino, new genus, new species are described in the Dendrophilinae. Additionally, Onthophilus yingae Jiang et al. is reassigned to Carinumerus. The new onthophiline genera share characters with the previously described Cretaceous histerid Cretonthophilus Caterino et al. and lack characters typical of extant Onthophilinae, and the three together may represent an early diverging lineage of this subfamily. The species of Druantia shares some features with extant Dendrophilinae, and some with Yethiha, so their systematic placement is less clear. A brief diagnostic catalog of all known Cretaceous Histeridae is provided.