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Bone defect is a common clinical disease. Due to the uncertainty of trauma or infection areas, customized size features are often required for bone substitutes. By inspiration of the natural bone structure, this study designs porous scaffolds with a biomimetic design perspective by using different inner and outer pore units. The outer pore units adopt body‐centered cubic (BCC) structure to simulate the weight‐bearing function of human cortical bone, while inner pore units using I‐Wrapped Package structure, a kind of three periods minimum surface, to obtain a good permeability and simulates the inner layer of cancellous bone. To further regulate the overall modulus of the scaffold within the range of natural bone modulus in the human body, the scaffold was designed to axial gradient structure. Compression experiments were conducted, and the results indicated that when the volume fraction linearly increased from 20% to 50%, the Young's modulus was close to the cortical bone modulus in the human body. In vitro cell experiments further proved that osteoblasts have good cellular activity and spreading morphology on the surface of this scaffold. The customized 3D‐printed heterogeneous porous titanium scaffold has great application potential in bone tissue engineering. [ABSTRACT FROM AUTHOR]

Background: Congenital disorders of glycosylation (CDG) are a type of inborn error of metabolism (IEM) resulting from defects in glycan synthesis or failed attachment of glycans to proteins or lipids. One rare type of CDG is caused by homozygous or compound heterozygous loss‐of‐function variants in mannosidase alpha class 2B member 2 (MAN2B2). To date, only two cases of MAN2B2‐CDG have been reported worldwide. Methods: Trio whole‐exome sequencing (Trio‐WES) was conducted to screen for candidate variants. N‐glycan profiles were measured by liquid chromatography–tandem mass spectrometry (LC–MS/MS). MAN2B2 expression was evaluated by western blotting. MX dynamin like GTPase 1 (MX1) function was estimated via Thogoto virus (THOV) minireplicon assay. Results: Trio‐WES identified compound heterozygous MAN2B2 (hg19, NM_015274.1) variants (c.384G>T; c.926T>A) in a CDG patient. This patient exhibited metabolic abnormalities, symptoms of digestive tract dysfunction, infection, dehydration, and seizures. Novel immune dysregulation characterized by abnormal lymphocytes and immunoglobulin was observed. The MAN2B2 protein level was not affected, while LC–MS/MS showed obvious disruption of N‐glycans and N‐linked glycoproteins. Conclusion: We described a CDG patient with novel phenotypes and disruptive N‐glycan profiling caused by compound heterozygous MAN2B2 variants (c.384G>T; c.926T>A). Our findings broadened both the genetic and clinical spectra of CDG. [ABSTRACT FROM AUTHOR]

Introduction: Watchkeeping is a significant activity during maritime operations, and failures of sustained attention and decision‐making can increase the likelihood of a collision. Methods: A study was conducted in a ship bridge simulator where 40 participants (20 experienced/20 inexperienced) performed: (1) a 20‐min period of sustained attention to locate a target vessel and (2) a 10‐min period of decision‐making/action selection to perform an evasive maneuver. Half of the participants also performed an additional task of verbally reporting the position of their vessel. Activation of the prefrontal cortex (PFC) was captured via a 15‐channel functional near‐infrared spectroscopy (fNIRS) montage, and measures of functional connectivity were calculated frontal using graph‐theoretic measures. Results: Neurovascular activation of right lateral area of the PFC decreased during sustained attention and increased during decision‐making. The graph‐theoretic analysis revealed that density declined during decision‐making in comparison with the previous period of sustained attention, while local clustering declined during sustained attention and increased when participants prepared their evasive maneuver. A regression analysis revealed an association between network measures and behavioral outcomes, with respect to spotting the target vessel and making an evasive maneuver. Conclusions: The right lateral area of the PFC is sensitive to watchkeeping and decision‐making during operational performance. Graph‐theoretic measures allow us to quantify patterns of functional connectivity and were predictive of safety‐critical performance. [ABSTRACT FROM AUTHOR]