[Clinical and genetic analysis of a patient with Neuronal intranuclear inclusion body disease characterized by cortical enhancement in the posterior brain region].

OBJECTIVE: To explore the clinical, imaging, and genetic characteristics of an adult patient with sporadic Neuronal intranuclear inclusion disease (NIID). METHODS: A patient who had visited the First People's Hospital of Chenzhou on August 6, 2023 was selected as the study subject. Results of clinical examination, neuroimaging, and genetic testing were retrospectively analyzed along with a literature review. The number of GGC trinucleotide repeats in the 5'-untranslated region of the NOTCH2NLC gene was determined by GC-PCR. RESULTS: The patient had presented with episodic encephalopathy, with enhanced magnetic resonance imaging showing enhancement features of the posterior cerebral cortex during the period of acute episode. Genetic testing revealed an increased number of GGC repeats (n = 97) in the 5'- untranslated region of the NOTCH2NLC gene, which confirmed the diagnosis of NIID. CONCLUSION: Clinical attention should be paid to the enhanced MRI findings of patients with adult-onset NIID, for whom posterior cortical enhancement may be characteristic manifestation during the acute phase of encephalopathy-like episode.

Hybrid Organic-Inorganic Additive for Robust Al Anode in Alkaline Aluminum-Air Battery.

Aluminum-air batteries (AABs), known for their high energy density, environmental friendliness, and cost-effectiveness, show immense promise in the realm of energy conversion applications. Nonetheless, their commercialization has encountered inherent challenges of Al anode corrosion and material degradation. In this study, economical hybrid electrolyte additives to inhibit the Al corrosion are developed, safeguarding the integrity of the Al anode. Due to the synergistic interplay between the organic compound dithiothreitol, and inorganic compounds zinc chloride, a robust zinc film is formed on the Al surface This Zn film plays a pivotal role in quelling parasitic hydrogen evolution reactions that typically can plague the Al electrode. Consequently, the as-prepared hybrid additive culminates in a remarkable enhancement to AABs, delivering exceptional discharge capacity of 1793.37 mAh g-1 , high energy density of 2047 Wh kg-1 , and excellent battery longevity (over 20 h in on/off cycling tests). This study, therefore, introduces a novel approach in utilizing hybrid electrolyte additives to effectively counteract corrosion-related challenges and boost the stability and performance of AABs.

Procedural Promotion of Multiple Stages in the Wound Healing Process by Graphene-Spiky Silica Heterostructured Nanoparticles.

Background: Multiple stages including hemostasis, inflammation, proliferation, and remodeling were involved in the wound healing process. The increase in nanomaterials in recent years has extended the scope of tools for wound healing; however, it is still difficult to achieve the four multistage procedures simultaneously. Materials and Methods: In this study, graphene-spiky silica heterostructured nanoparticles (GS) were synthesized for the procedural acceleration of the multistage in wound healing process. The nanobridge effect of GS was analyzed through the adhesion of two skins, the antibacterial effect was assessed in Gram-negative Escherichia coli (E. coli) and Gram-positive Staphylococcus aureus (S. aureus) bacteria, cell proliferation and migration were investigated in mouse embryonic fibroblast (NIH-3T3) cells, and the in vivo wound healing effect was examined in female BALB/c mice with a cutting wound and E. coli or S. aureus bacteria infection on the back. Results: First, GS has a strong nanobridge effect on the rapid closure of wounds because the spiky architecture on the surface of GS facilitates the adhesion of skins, promoting the hemostasis stage. Second, graphene exhibits antimicrobial activities both in chemical and physical interactions, especially under simulated sunlight irradiation. Third, graphene plays an important role in scaffolding function, together with the spiky topographical architecture of GS, accelerating the proliferation and maturation stages. Conclusion: By periodically promoting every stage of wound healing, GS combined with simulated sunlight irradiation could significantly accelerate wound healing. With a simple composition and compact structure but multiple functions, this strategy will be the guideline for the development of ideal wound-healing nanomaterials.

[Optimal surgical timing and treatment of thumb duplication in children].

OBJECTIVE: To investigate the optimal surgical timing and treatment of congenital duplication of the thumb in children. METHODS: A clinical study was performed on 154 fingers of the 132 patients (including 85 males, 47 females;mean age (1.53 +/- 2.47) years; ranged from 2 months to 13 years). Duplicated thumbs were surgically treated from December 2007 to February 2012. All patients underwent detailed physical examination and radiological assessment. Surgical methods should be selected according to the age, Wassel classification and deformity. The operation steps included resection of the most hypoplastic thumb, followed by skin flap plasty, tendon shift (transplantation) , reconstruction of collateral ligament and articular capsule. 11 cases over the age of 6 underwent osteotomy discretion as appropriate. RESULTS: A total of 117 fingers of the 104 patients were followed up for an average 36. 7 months (range,6-55 months). We adopted upgrade Tada standard to evaluate the follow-up results as excellent in 77 thumbs, good in 21 thumbs, fair in 15 thumbs and poor in 4 thumbs. Three thumbs appeared secondary angular deformity 2 years after operation and one thumb appeared residual osteoepiphysis in the radial side of metacarpophalangeal joint 3 years after operation. The appearance and the function were almost normal after second operation. CONCLUSIONS: The optimal surgical timing of congenital duplication of thumb should be based on the appearance of thumb ossification center. The surgical timing of Wassel type- I and II should be chosen at 1. 5 years old when the ossification center of distal phalanx appears, Wassel type-III and IV should be chosen at 1 year old when the ossification center of proximal phalanx appears, Wassel type-V and VI should be chosen at 2. 5 years old when the ossification center of metacarpal appears, and Wassel type-VII should be chosen at 2. 5 years old. The surgical method should be selected on individualized principle. The key points are reconstruction of collateral ligament, tendon and articular capsule and correction of ulnar deviation (or radial deviation).