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Summary: Atoh7 is transiently expressed in retinal progenitor cells (RPCs) and is required for retinal ganglion cell (RGC) differentiation. In humans, a deletion in a distal non-coding regulatory region upstream of ATOH7 is associated with optic nerve atrophy and blindness. Here, we functionally interrogate the significance of the Atoh7 regulatory landscape to retinogenesis in mice. Deletion of the Atoh7 enhancer structure leads to RGC deficiency, optic nerve hypoplasia, and retinal blood vascular abnormalities, phenocopying inactivation of Atoh7. Further, loss of the Atoh7 remote enhancer impacts ipsilaterally projecting RGCs and disrupts proper axonal projections to the visual thalamus. Deletion of the Atoh7 remote enhancer is also associated with the dysregulation of axonogenesis genes, including the derepression of the axon repulsive cue Robo3. Our data provide insights into how Atoh7 enhancer elements function to promote RGC development and optic nerve formation and highlight a key role of Atoh7 in the transcriptional control of axon guidance molecules.

Background The interaction between right ventricular (RV) function and pulmonary hypertension is crucial for prognosis of patients with severe functional tricuspid regurgitation. RV free wall longitudinal strain (RVFWLS) has been reported to detect RV systolic dysfunction earlier than other conventional parameters. Although pulmonary artery systolic pressure measured by Doppler echocardiography is often underestimated in severe functional tricuspid regurgitation, right atrial pressure (RAP) estimated by echocardiography may be viewed as a prognostic factor. Impact of RAP and RVFWLS on outcome in patients with severe functional tricuspid regurgitation remains unclear. The aim of the present study was to investigate prognostic implication of RAP, RVFWLS, and their combination in this population. Methods and Results We retrospectively examined 377 patients with severe functional tricuspid regurgitation. RAP, pulmonary artery systolic pressure, RV fractional area change, and RVFWLS were analyzed. RAP of 15 mm Hg was classified as elevated RAP. All‐cause death at 2‐year follow‐up was defined as the primary end point. RVFWLS provided better prognostic information than RV fractional area change by receiver operating characteristic curve analysis. In the multivariable Cox regression analysis, elevated RAP and RVFWLS of ≤18% were independent predictors of clinical outcome. Patients with RVFWLS of ≤18% had higher risk of all‐cause death than those without by Kaplan–Meier curve analysis. Furthermore, when patients were stratified into 4 groups by RAP and RVFWLS, the group with elevated RAP and RVFWLS of ≤18% had the worst outcome. Conclusions Elevated RAP and RVFWLS of ≤18% were independent predictors of all‐cause death. The combination of elevated RAP and RVFWLS effectively stratified the all‐cause death.

Soft robotics is a breakthrough technology to support human–robot interactions. The soft structure of a soft robot can increase safety during human and robot interactions. One of the promising soft actuators for soft robotics is dielectric elastomer actuators (DEAs). DEAs can operate silently and have an excellent energy density. The simple structure of DEAs leads to the easy fabrication of soft actuators. The simplicity combined with silent operation and high energy density make DEAs interesting for soft robotics researchers. DEAs actuation follows the Maxwell-pressure principle. The pressure produced in the DEAs actuation depends much on the voltage applied. Common DEAs requires high voltage to gain an actuation. Since the power consumption of DEAs is in the milli-Watt range, the current needed to operate the DEAs can be neglected. Several commercially available DC-DC converters can convert the volt range to the kV range. In order to get a voltage in the 2–3 kV range, the reliable DC-DC converter can be pricy for each device. This problem hinders the education of soft actuators, especially for a newcomer laboratory that works in soft electric actuators. This paper introduces an entirely do-it-yourself (DIY) Ultrahigh voltage amplifier (UHV-Amp) for education in soft robotics. UHV-Amp can amplify 12 V to at a maximum of 4 kV DC. As a demonstration, we used this UHV-Amp to test a single layer of powdered-based DEAs. The strategy to build this educational type UHV-Amp was utilizing a Cockcroft-Walton circuit structure to amplify the voltage range to the kV range. In its current state, the UHV-Amp has the potential to achieve approximately 4 kV. We created a simple platform to control the UHV-Amp from a personal computer. In near future, we expect this easy control of the UHV-Amp can contribute to the education of soft electric actuators.

Li metal is a promising negative-electrode material for high-energy-density all-solid-state batteries. However, the surface of Li metal is prone to oxidation, which results in the formation of a contamination layer at the Li metal–solid electrolyte interface. This interfacial contamination layer is the root cause of short-circuiting and poor cycle stability, thus hindering the development of all-solid-state batteries. Prior studies have not quantitatively assessed the effect of the above layer on battery performance. Herein, the degradation mechanisms affecting the interface are investigated using alternating-current impedance measurements and Li plating–stripping cycle tests for a symmetric cell. A thin contamination layer results in a Li–electrolyte interface with a low resistance of 0.20 kΩ cm2 and stable Li plating–stripping behavior at a current density of 3 mA cm−2, whereas a thick contamination layer results in a high interfacial resistance of 2.0 kΩ cm2. The thinning of the contamination layer on Li metal enhances the stability of the Li–electrolyte interface and Li plating–stripping kinetics. [ABSTRACT FROM AUTHOR]

Goltz syndrome is a rare X-linked dominant multisystem disorder that presents with ectoderm and mesoderm-derived symptoms. Skin manifestations including congenital patchy skin aplasia, congenital nodular fat herniation, congenital hypo- or hyperpigmentation along Blaschko's lines, telangiectasia, and congenital ridged dysplastic nails are typical in this disorder. Almost all cases of Goltz syndrome correspond to female newborns and that hemizygosis makes the syndrome fetal in males. Triple X syndrome is a relatively common congenital disorder that presents with mild to no symptoms in the developmental and psychiatric realm. The patient reported here was born with multisystem anomaly affecting the eyes, craniofacial region, cardiovascular system, skin, and limbs. A G-banding chromosomal study revealed 47, XXX. She was diagnosed with Goltz syndrome owing to her distinctive skin manifestations. The congenital cervical skin defect healed with conservative treatment. The facial cleft, cleft lip-palate, and syndactyly were successfully treated with multiple surgical treatments. The combination of triple X syndrome and Goltz syndrome is very rare. We describe the expression of presenting with both syndromes simultaneously. [ABSTRACT FROM AUTHOR]

Objectives: Right ventricular (RV)–pulmonary arterial (PA) coupling is important in various cardiac diseases. Recently, several echocardiographic surrogates for RV–PA coupling have been proposed and reported to be useful in predicting outcomes. However, it remains unclear which surrogate is the most clinically relevant. This study aimed to comprehensively compare the prognostic value of different echocardiographic RV–PA coupling surrogates. Methods: We retrospectively reviewed 242 patients with various cardiac conditions who underwent comprehensive transthoracic echocardiography with three‐dimensional RV data. In addition to conventional parameters including tricuspid annular plane systolic excursion (TAPSE), fractional area change (FAC), and PA systolic pressure (PASP), we analyzed RV free wall and global longitudinal strain (FWLS and GLS). We also obtained RV ejection fraction (RVEF), stroke volume (SV), and end‐systolic volume (ESV) using three‐dimensional RV analysis. RV–PA coupling surrogates were calculated as TAPSE/PASP, FAC/PASP, FWLS/PASP, GLS/PASP, RVEF/PASP, and SV/ESV. The study endpoint was a composite outcome of all‐cause death or cardiovascular hospitalization within 1 year. Results: In multivariable analysis, all the RV–PA coupling surrogates were independent predictors of the outcome. Among the surrogates, the model with TAPSE/PASP showed the lowest prognostic value in model fit and discrimination ability, whereas the model with RVEF/PASP exhibited the highest prognostic value. The partial likelihood ratio test indicated that the model with RVEF/PASP was significantly better than the model with TAPSE/PASP (p <.024). Conclusion: All the RV–PA coupling surrogates were independent predictors of the outcome. Notably, RVEF/PASP had the highest prognostic value among the surrogates. [ABSTRACT FROM AUTHOR]

Background: Minimally invasive endoscopic and stereotactic surgery have been established as surgical treatments for putaminal hemorrhage. However, facilities that do not have equipment for endoscopic or stereotactic surgery will likely have to perform conventional craniotomy. Using a tubular retractor, we were able to perform minimally invasive surgery, such as endoscopic surgery. Methods: A craniotomy was performed for left putaminal hemorrhage after cerebral infarction treatment. A 3-4 cm craniotomy centered at Kocher's point was performed under general anesthesia. A 2 cm incision was made in the cortex, and a tubular retractor was inserted under a microscope. The hematoma was reached at a position 4-5 cm from the cortex. Results: Thanks to the tubular retractor, it was relatively easy to observe the hematoma, and it was possible to remove it and confirm hemostasis without difficulty. Brain injury caused by the retractor insertion cavity was small, and no hemostasis was required. The surgery was completed by dura mater closure, bone flap fixation, and wound closure as per the standard. Most of the putaminal hemorrhage could be removed, and there was no rebleeding after the operation. The patient is still undergoing rehabilitation because of aphasia and muscle weakness. Manual Muscle Testing was at three points in the upper limb, and four points in the lower limb remained. Conclusion: For putaminal hemorrhage, microscopic craniotomy was performed using a tubular retractor and an approach such as endoscopic surgery. Craniotomy, hematoma removal, and hemostasis operations are also considered to be minimally invasive surgeries. [ABSTRACT FROM AUTHOR]