Caucasian Infants’ Attentional Orienting to Own- and Other-Race Faces

Infants show preferential attention toward faces and detect faces embedded within complex naturalistic scenes. Newborn infants are insensitive to race, but rapidly develop differential processing of own- and other-race faces. In the present study, we investigated the development of attentional orienting toward own- and other-race faces embedded within naturalistic scenes. Infants aged six-, nine- and twelve-months did not show differences in the speed of orienting to own- and other race faces, but other-race faces held infants’ visual attention for longer. We also found a clear developmental progression in attentional capture and holding, with older infants orienting to faces faster and fixating them for longer. Results are interpreted within the context of the two-process model of face processing

Infants show pupil dilatory responses to happy and angry facial expressions

Facial expressions are one way in which infants and adults communicate emotion. Infants scan expressions similarly to adults, yet it remains unclear whether they are receptive to the affective information they convey. The current study investigates six-, nine- and twelve-month infants’ (N = 146) pupillary responses to the six ‘basic’ emotional expressions (happy, sad, surprise, fear, anger, and disgust). To do this we use dynamic stimuli and gaze-contingent eye-tracking to simulate brief interactive exchanges, alongside a static control condition. Infants’ arousal responses were stronger for dynamic compared to static stimuli. And for dynamic stimuli we found that, compared to neutral, infants showed dilatory responses for happy and angry expressions only. Although previous work has shown infants can discriminate perceptually between facial expressions, our data suggest that sensitivity to the affective content of all six basic emotional expressions may not fully emerge until later in ontogeny

Method for Maintaining One-Dimensional Temperature Gradients in Unsaturated, Closed Soil Cells

One-dimensional temperature gradients are difficult to achieve in nonisothermal laboratory studies because, in addition to desired axial temperature gradients, ambient temperature interference (ATI) creates a radial temperature distribution. Our objective was to develop a closed soil cell with limited ATI. The cell consists of a smaller soil column, the control volume, surrounded by a larger soil column, which provides radial insulation. End boundary temperatures are controlled by a new spiral-circulation heat exchanger. Four cell size configurations were tested for ATI under varying ambient temperatures. Results indicate that cells with a 9-cm inner column diameter, 5-cm concentric soil buffer, and either 10- or 20-cm length effectively achieved one-dimensional temperature conditions. At 30°C ambient temperature, and with axial temperature gradients as large as 1°C cm−1, average steady-state radial temperature gradients in the inner soil columns were−1 Thus, these cell configurations meet the goal of maintaining a one-dimensional temperature distribution. These cells provide new opportunities for improving the study of coupled heat and water movement in soil

Direct replacement of oral sodium benzoate with glycerol phenylbutyrate in children with urea cycle disorders

Long-term management of urea cycle disorders (UCDs) often involves unlicensed oral sodium benzoate (NaBz) which has a high volume and unpleasant taste. A more palatable treatment is licenced and available (glycerol phenylbutyrate [GPB], Ravicti) but guidance on how to transition patients from NaBz is lacking. A retrospective analysis of clinical and biochemical data was performed for eight children who transitioned from treatment with a single ammonia scavenger, NaBz, to GPB at a single metabolic centre; UCDs included arginosuccinic aciduria (ASA) (n = 5), citrullinaemia type 1 (n = 2) and carbamoyl phosphate synthetase I deficiency (CPS1) (n = 1). Patients transitioned either by gradual transition over 1–2 weeks (n = 3) or direct replacement of NaBz with GPB (n = 5). Median initial dose of GPB was 8.5 mL/m2/day based on published product information; doses were revisited subsequently in clinic and titrated individually (range 4.5–11 mL/m2/day). Pre-transition and post-transition mean ammonia levels were 37 μmol/L (SD 28 μmol/L) and 29 μmol/L (SD 22 μmol/L), respectively (p = 0.09), and mean glutamine levels were 664 μmol/L (SD 225 μmol/L) and 598 μmol/L (SD 185 μmol/L), respectively (p = 0.24). There were no reductions in levels of branched chain amino acids. No related adverse drug reactions were reported. Patients preferred GPB because of its lower volume and greater palatability. Direct replacement of NaBz with GPB maintained metabolic control and was simple for the health service and patients to manage. A more cautious approach with additional monitoring would be warranted in brittle patients and patients whose ammonia levels are difficult to control

Understanding face detection with visual arrays and real-world scenes

Face detection has been studied by presenting faces in blank displays, object arrays, and real-world scenes. This study investigated whether these display contexts differ in what they can reveal about detection, by comparing frontal-view faces with those shown in profile (Experiment 1), rotated by 90º (Experiment 2), or turned upside-down (Experiment 3). In blank displays, performance for all face conditions was equivalent, whereas upright frontal faces showed a consistent detection advantage in arrays and scenes. Experiment 4 examined which facial characteristics drive this detection advantage by rotating either the internal or external facial features by 90º while the other features remained upright. Faces with rotated internal features were detected as efficiently as their intact frontal counterparts, whereas detection was impaired when external features were rotated. Finally, Experiment 5 applied Voronoi transformations to scenes to confirm that complexity of stimulus displays modulates the detection advantage for upright faces. These experiments demonstrate that context influences what can be learned about the face detection process. In complex visual arrays and natural scenes, detection proceeds more effectively when external facial features are preserved in an upright orientation. These findings are consistent with a cognitive detection template that focuses on general face-shape information

Proposed therapeutic range of treosulfan in reduced toxicity pediatric allogeneic hematopoietic stem cell transplant conditioning: results from a prospective trial

Treosulfan is given off‐label in pediatric allogeneic hematopoietic stem cell transplant. This study investigated treosulfan's pharmacokinetics (PKs), efficacy, and safety in a prospective trial. Pediatric patients (n = 87) receiving treosulfan‐fludarabine conditioning were followed for at least 1 year posttransplant. PKs were described with a two‐compartment model. During follow‐up, 11 of 87 patients died and 12 of 87 patients had low engraftment (≤ 20% myeloid chimerism). For each increase in treosulfan area under the curve from zero to infinity (AUC(0‐∞)) of 1,000 mg hour/L the hazard ratio (95% confidence interval) for mortality increase was 1.46 (1.23–1.74), and the hazard ratio for low engraftment was 0.61 (0.36–1.04). A cumulative AUC(0‐∞) of 4,800 mg hour/L maximized the probability of success (> 20% engraftment and no mortality) at 82%. Probability of success with AUC(0‐∞) between 80% and 125% of this target were 78% and 79%. Measuring PK at the first dose and individualizing the third dose may be required in nonmalignant disease

Identifying the unmet information and support needs of women with autoimmune rheumatic diseases during pregnancy planning, pregnancy and early parenting: mixed-methods study

Background Autoimmune rheumatic diseases (ARDs) such as inflammatory arthritis and Lupus, and many of the treatments for these diseases, can have a detrimental impact on fertility and pregnancy outcomes. Disease activity and organ damage as a result of ARDs can affect maternal and foetal outcomes. The safety and acceptability of hormonal contraceptives can also be affected. The objective of this study was to identify the information and support needs of women with ARDs during pregnancy planning, pregnancy and early parenting. Methods This mixed methods study included a cross-sectional online survey and qualitative narrative interviews. The survey was completed by 128 women, aged 18–49 in the United Kingdom with an ARD who were thinking of getting pregnant in the next five years, who were pregnant, or had young children (< 5 years old). The survey assessed quality-of-life and information needs (Arthritis Impact Measurement Scale Short Form and Educational Needs Assessment Tool), support received, what women found challenging, what was helpful, and support women would have liked. From the survey participants, a maximum variation sample of 22 women were purposively recruited for qualitative interviews. Interviews used a person-centered participatory approach facilitated by visual methods, which enabled participants to reflect on their experiences. Interviews were also carried out with seven health professionals purposively sampled from primary care, secondary care, maternity, and health visiting services. Results Survey findings indicated an unmet need for information in this population (ENAT total mean 104.85, SD 30.18). Women at the pre-conception stage reported higher needs for information on pregnancy planning, fertility, giving birth, and breastfeeding, whereas those who had children already expressed a higher need for information on pain and mobility. The need for high quality information, and more holistic, multi-disciplinary, collaborative, and integrated care consistently emerged as themes in the survey open text responses and interviews with women and health professionals. Conclusions There is an urgent need to develop and evaluate interventions to better inform, support and empower women of reproductive age who have ARDs as they navigate the complex challenges that they face during pregnancy planning, pregnancy and early parenting

Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene transfer

Argininosuccinate lyase (ASL) belongs to the hepatic urea cycle detoxifying ammonia, and the citrulline-nitric oxide (NO) cycle producing NO. ASL-deficient patients present argininosuccinic aciduria characterised by hyperammonaemia, multiorgan disease and neurocognitive impairment despite treatment aiming to normalise ammonaemia without considering NO imbalance. Here we show that cerebral disease in argininosuccinic aciduria involves neuronal oxidative/nitrosative stress independent of hyperammonaemia. Intravenous injection of AAV8 vector into adult or neonatal ASL-deficient mice demonstrates long-term correction of the hepatic urea cycle and the cerebral citrulline-NO cycle, respectively. Cerebral disease persists if ammonaemia only is normalised but is dramatically reduced after correction of both ammonaemia and neuronal ASL activity. This correlates with behavioural improvement and reduced cortical cell death. Thus, neuronal oxidative/nitrosative stress is a distinct pathophysiological mechanism from hyperammonaemia. Disease amelioration by simultaneous brain and liver gene transfer with one vector, to treat both metabolic pathways, provides new hope for hepatocerebral metabolic diseases