Abstract Mutations in GBA1 are a well-established risk factor for Parkinson disease (PD). GBA-associated PD (GBA-PD) may have a higher burden of nonmotor symptoms than idiopathic PD (IPD). We sought ...to characterize the relationship between GBA-PD and neuropsychiatric symptoms. Subjects were screened for common GBA1 mutations. GBA-PD (n = 31) and non-carrier (IPD; n = 55) scores were compared on the Unified Parkinson Disease Rating Scale (UPDRS), Montreal Cognitive Assessment (MoCA), Beck Depression Inventory (BDI), and the State-Trait Anxiety Index (STAI). In univariate comparisons, GBA-PD had a greater prevalence of depression (33.3%) versus IPD (13.2%) (p < 0.05). In regression models controlling for age, sex, disease duration, motor disability, and MoCA score, GBA-PD had an increased odds of depression (OR 3.66, 95% CI 1.13
–
11.8) (p = 0.03). Post-hoc analysis stratified by sex showed that, among men, GBA-PD had a higher burden of trait anxiety and depression than IPD; this finding was sustained in multivariate models. Among women, GBA-PD did not confer greater psychiatric morbidity than IPD. These results suggest that GBA1 mutations confer greater risk of neuropsychiatric morbidity in PD, and that sex may affect this association.
Familial adenomatous polyposis (FAP) is a rare genetic disorder with autosomal dominant inheritance, defined by numerous adenomatous polyps, which inevitably progress to colorectal carcinoma unless ...detected and managed early. Greater than 70% of patients with this syndrome also develop extraintestinal manifestations, such as multiple osteomas, dental abnormalities, and a variety of other lesions located throughout the body. These manifestations have historically been subcategorized as Gardner syndrome, Turcot syndrome, or gastric adenocarcinoma and proximal polyposis of the stomach. Recent studies, however, correlate the severity of gastrointestinal disease and the prominence of extraintestinal findings to specific mutations within the adenomatous polyposis coli gene (
), supporting a spectrum of disease as opposed to subcategorization. Advances in immunohistochemical and molecular techniques shed new light on the origin, classification, and progression risk of different entities associated with FAP.
To provide a comprehensive clinicopathologic review of neoplastic and nonneoplastic entities associated with FAP syndrome, with emphasis on recent developments in immunohistochemical and molecular profiles of extraintestinal manifestations in the thyroid, skin, soft tissue, bone, central nervous system, liver, and pancreas, and the subsequent changes in classification schemes and risk stratification.
This review will be based on peer-reviewed literature and the authors' experiences.
In this review we will provide an update on the clinicopathologic manifestations, immunohistochemical profiles, molecular features, and prognosis of entities seen in FAP, with a focus on routine recognition and appropriate workup of extraintestinal manifestations.
Electrospun fibers are a promising method for encapsulation of reactive agents in self-healing coatings. Healing is initiated by mechanical damage to the coating causing the fibers to rupture and ...release their core materials into the damage region. Prior work has demonstrated autonomous healing in coatings containing electrospun fibers, but full characterization of the electrospun fiber microstructure and healing performance of the coating is lacking. In this study, we utilize electrospun fibers containing liquid healing agents to achieve a crosslinking reaction of poly(dimethylsiloxane) (PDMS) to a crosslinking agent poly(diethoxysiloxane) (PDES), initiated by the catalyst dibutyltindilaurate (DBTL), to fill a damaged region and reseal the metal substrate. Fiber morphology is characterized using scanning electron microscopy (SEM), transmission electron microscopy (TEM), and confocal fluorescence microscopy (CFM). Successful delivery of healing agents to the damage region and subsequent crosslinking reaction is observed using SEM and chemically using infrared spectroscopy. The performance of the healed coating is evaluated electrochemically using linear polarization, where the coatings were subjected to a corrosive environment. The self-healing electrospun coating exhibits lower corrosion current than in control cases, resulting in an 88% corrosion inhibition efficiency.
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•Bead-free electrospun core-shell fibers containing PDMS were developed.•Electrospun fibers and coating were characterized using several microscopy methods.•Self-healing coating healed damage and exhibited 88% corrosion inhibition efficiency.
Complex multidimensional vascular polymers are created, enabled by sacrificial template materials of 0D to 3D. Sacrificial material consisting of the commodity biopolymer poly(lactic acid) is treated ...with a tin catalyst to accelerate thermal depolymerization, and formed into sacrificial templates across multiple dimensions and spanning several orders of magnitude in scale: spheres (0D), fibers (1D), sheets (2D), and 3D printed. Templates are embedded in a thermosetting polymer and removed using a thermal treatment process, vaporization of sacrificial components (VaSC), leaving behind an inverse replica. The effectiveness of VaSC is verified both ex situ and in situ, and the resulting structures are validated via flow rate testing. The VaSC platform is expanded to create vascular and porous architectures across a wide range of size and geometry, allowing engineering applications to take advantage of vascular designs optimized by biology.
Sacrificial templates of 0D to 3D are fabricated and used to create vascular and porous architectures. Embedded sacrificial templates are removed using a thermal treatment process, vaporization of sacrificial components (VaSC), leaving behind an inverse replica. The effectiveness of VaSC is verified both ex situ and in situ, and the resulting structures are validated via flow rate testing.
The quality of cardiopulmonary resuscitation (CPR) affects hemodynamics, survival, and neurological outcomes following pediatric cardiopulmonary arrest (CPA). Most health care professionals fail to ...perform CPR within established American Heart Association guidelines.
To determine whether "just-in-time" (JIT) CPR training with visual feedback (VisF) before CPA or real-time VisF during CPA improves the quality of chest compressions (CCs) during simulated CPA.
Prospective, randomized, 2 × 2 factorial-design trial with explicit methods (July 1, 2012, to April 15, 2014) at 10 International Network for Simulation-Based Pediatric Innovation, Research, & Education (INSPIRE) institutions running a standardized simulated CPA scenario, including 324 CPR-certified health care professionals assigned to 3-person resuscitation teams (108 teams).
Each team was randomized to 1 of 4 permutations, including JIT training vs no JIT training before CPA and real-time VisF vs no real-time VisF during simulated CPA.
The proportion of CCs with depth exceeding 50 mm, the proportion of CPR time with a CC rate of 100 to 120 per minute, and CC fraction (percentage CPR time) during simulated CPA.
The quality of CPR was poor in the control group, with 12.7% (95% CI, 5.2%-20.1%) mean depth compliance and 27.1% (95% CI, 14.2%-40.1%) mean rate compliance. JIT training compared with no JIT training improved depth compliance by 19.9% (95% CI, 11.1%-28.7%; P < .001) and rate compliance by 12.0% (95% CI, 0.8%-23.2%; P = .037). Visual feedback compared with no VisF improved depth compliance by 15.4% (95% CI, 6.6%-24.2%; P = .001) and rate compliance by 40.1% (95% CI, 28.8%-51.3%; P < .001). Neither intervention had a statistically significant effect on CC fraction, which was excellent (>89.0%) in all groups. Combining both interventions showed the highest compliance with American Heart Association guidelines but was not significantly better than either intervention in isolation.
The quality of CPR provided by health care professionals is poor. Using novel and practical technology, JIT training before CPA or real-time VisF during CPA, alone or in combination, improves compliance with American Heart Association guidelines for CPR that are associated with better outcomes.
clinicaltrials.gov Identifier: NCT02075450.
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