Evolution of the retinal function by flash-ERG in one child suffering from neuronal ceroid lipofuscinosis CLN2 treated with cerliponase alpha: case report.

Introduction: Neuronal ceroid lipofuscinoses (CLN) are neurodegenerative disorders among the most frequent, inherited as an autosomal recessive trait. Affected patients can present with progressive decline in cognitive and motor functions, seizures, a shortened life span and visual deficiency. CLN2 is one of the rare CLN that benefits from treatment by cerliponase alpha an enzyme replacement therapy.

Preparation and Characterization of Highly Elastic Foams with Enhanced Electromagnetic Wave Absorption Based on Ethylene-Propylene-Diene-Monomer Rubber Filled with Barium Titanate/Multiwall Carbon Nanotube Hybrid.

Hybrid ethylene-propylene-diene-monomer (EPDM) nanocomposite foams were produced via compression molding with enhanced electromagnetic wave absorption efficiency. The hybrid filler, consisting of 20 phr ferroelectric barium titanate (BT) and various loading fractions of multi-wall carbon nanotubes (MWCNTs), synergistically increased the electromagnetic (EM) wave absorption characteristics of the EPDM foam. Accordingly, while the EPDM foam filled with 20 phr BT was transparent to the EM wave within the frequency range of 8.

Pulmonary Alterations Among Workers in a Dental Prosthesis Laboratory: Exploring High Dust Concentrations and Novel Findings of Bacterial Genera in the Workplace to Achieve Improved Control.

Objective: To evaluate the pulmonary alterations in workers from a dental prosthesis laboratory and explore dust and bacterial dissemination generated in the laboratory.

Methods: Spirometry and computerized axial tomography were performed on 67 workers. Dust in workplace air was determined using the filtration-gravimetric method, and bacterial detection was explored using 16S rDNA gene sequencing.

Highly Deformable Porous Electromagnetic Wave Absorber Based on Ethylene-Propylene-Diene Monomer/Multiwall Carbon Nanotube Nanocomposites.

The need for electromagnetic interference (EMI) shields has risen over the years as the result of our digitally and highly connected lifestyle. This work reports on the development of one such shield based on vulcanized rubber foams. Nanocomposites of ethylene-propylene-diene monomer (EPDM) rubber and multiwall carbon nanotubes (MWCNTs) were prepared via hot compression molding using a chemical blowing agent as foaming agent.

Hafnium(IV) Chloride Catalyzes Highly Efficient Acetalization of Carbonyl Compounds.

Background: Hafnium(IV) tetrachloride efficiently catalyzes the protection of a variety of aldehydes and ketones, including benzophenone, acetophenone, and cyclohexanone, to the corresponding dimethyl acetals and 1,3-dioxolanes, under microwave heating. Substrates possessing acid-labile protecting groups (TBDPS and Boc) chemoselectively generated the corresponding acetal/ketal in excellent yields.

Aims And Objectives: In this study.