Real-Time Monitoring of Particle Size in Emulsion Polymerization: Simultaneous Turbidity and Photon Density Wave Spectroscopy.

Particle size evolution in seeded semibatch emulsion polymerization is monitored by two real-time monitoring techniques: online turbidity spectroscopy (TUS) and inline photon density wave spectroscopy (PDWS). An automatic dilution system that withdraws a sample from the reactor and upon dilution transfers to the measurement cell is used for the online TUS analysis. A PDWS probe is immersed in the reactor and collects inline the scattered light directly from the reacting latex.

Chromosome Transplantation: Opportunities and Limitations.

There are thousands of rare genetic diseases that could be treated with classical gene therapy strategies such as the addition of the defective gene via viral or non-viral delivery or by direct gene editing. However, several genetic defects are too complex for these approaches. These "genomic mutations" include aneuploidies, intra and inter chromosomal rearrangements, large deletions, or inversion and copy number variations.

Exonic knockout and knockin gene editing in hematopoietic stem and progenitor cells rescues RAG1 immunodeficiency.

Recombination activating genes () are tightly regulated during lymphoid differentiation, and their mutations cause a spectrum of severe immunological disorders. Hematopoietic stem and progenitor cell (HSPC) transplantation is the treatment of choice but is limited by donor availability and toxicity. To overcome these issues, we developed gene editing strategies targeting a corrective sequence into the human gene by homology-directed repair (HDR) and validated them by tailored two-dimensional, three-dimensional, and in vivo xenotransplant platforms to assess rescue of expression and function.

Unbiased assessment of genome integrity and purging of adverse outcomes at the target locus upon editing of CD4 T-cells for the treatment of Hyper IgM1.

Hyper IgM1 is an X-linked combined immunodeficiency caused by CD40LG mutations, potentially treatable with CD4 T-cell gene editing with Cas9 and a "one-size-fits-most" corrective template. Contrary to established gene therapies, there is limited data on the genomic alterations following long-range gene editing, and no consensus on the relevant assays. We developed drop-off digital PCR assays for unbiased detection of large on-target deletions and found them at high frequency upon editing.

Wavelength exponent based calibration for turbidity spectroscopy: Monitoring the particle size during emulsion polymerization reactions.

Particle size and particle size distribution (PSD) are important properties of polymer latexes because they strongly affect the film formation and the rheological properties of the latexes. Thus, monitoring the particle size is of paramount importance during the production of waterborne polymeric dispersions, for which online/inline measurements of the particle size are required. Herein, turbidity spectroscopy (TUS) is used to measure the particle size of nanoparticles in dispersed media.

Polymer Colloids: Current Challenges, Emerging Applications, and New Developments.

Polymer colloids are complex materials that have the potential to be used in a vast array of applications. One of the main reasons for their continued growth in commercial use is the water-based emulsion polymerization process through which they are generally synthesized. This technique is not only highly efficient from an industrial point of view but also extremely versatile and permits the large-scale production of colloidal particles with controllable properties.

Point-of-care lung ultrasound score for predicting escalated care in children with respiratory distress.

Purpose: Respiratory distress due to lower respiratory illnesses is a leading cause of death in children. Early recognition of high-risk populations is critical for the allocation of adequate resources. Our goal was to assess whether the lung ultrasound (US) score obtained at admission in children with respiratory distress predicts the need for escalated care.

Dysfunctional Extracellular Matrix Remodeling Supports Perianal Fistulizing Crohn's Disease by a Mechanoregulated Activation of the Epithelial-to-Mesenchymal Transition.

Background And Aims: Perianal fistula represents one of the most disabling manifestations of Crohn's disease (CD) due to complete destruction of the affected mucosa, which is replaced by granulation tissue and associated with changes in tissue organization. To date, the molecular mechanisms underlying perianal fistula formation are not well defined. Here, we dissected the tissue changes in the fistula area and addressed whether a dysregulation of extracellular matrix (ECM) homeostasis can support fistula formation.

Hematopoietic Cells from Pluripotent Stem Cells: Hope and Promise for the Treatment of Inherited Blood Disorders.

Inherited blood disorders comprise a large spectrum of diseases due to germline mutations in genes with key function in the hematopoietic system; they include immunodeficiencies, anemia or metabolic diseases. For most of them the only curative treatment is bone marrow transplantation, a procedure associated to severe complications; other therapies include red blood cell and platelet transfusions, which are dependent on donor availability. An alternative option is gene therapy, in which the wild-type form of the mutated gene is delivered into autologous hematopoietic stem cells using viral vectors.

Where Are We with RPE Replacement Therapy? A Translational Review from the Ophthalmologist Perspective.

The retinal pigmented epithelium (RPE) plays a pivotal role in retinal homeostasis. It is therefore an interesting target to fill the unmet medical need of different retinal diseases, including age-related macular degeneration and Stargardt disease. RPE replacement therapy may use different cellular sources: induced pluripotent stem cells or embryonic stem cells.

Taking Advantage of Phosphate Functionalized Waterborne Acrylic Binders to Get Rid of Inhibitors in Direct-to-Metal Paints.

In this paper, two phosphate functionalized acrylic binders are formulated to yield direct-to-metal paints without using corrosion inhibitors. The difference between both binders is the addition of polystearylacrylate crystalline nanodomains in one of them, and an amorphous methyl methacylate-co-butyl acrylate copolymer in the other. The water sensitivity, mechanical stability, adhesion, and the performance of the paints against corrosion (high humidity resistance, accelerated weathering, and salt-spray tests) are assessed and compared with a DTM paint formulated from a commercial binder.

The importance of viral load in the severity of acute bronchiolitis in hospitalized infants.

Objective: The relationship between viral load and the clinical evolution of bronchiolitis is controversial. Therefore, we aimed to analyze viral loads in infants hospitalized for bronchiolitis.

Methods: We tested for the presence of human respiratory syncytial virus (HRSV) or human rhinovirus (HRV) using quantitative molecular tests of nasopharyngeal secretions and recorded severity outcomes.

Generation and characterization of the human induced pluripotent stem cell (hiPSC) line NCUFi001-A from a patient carrying KCNQ1 G314S mutation.

In this study we describe the generation and characterization of an human induced pluripotent stem cell (hiPSC) line from a long QT syndrome type 1 (LQT1) patient carrying the KCNQ1 c.940 G > A (p.Gly314Ser) mutation.

Human iPSC-based neurodevelopmental models of globoid cell leukodystrophy uncover patient- and cell type-specific disease phenotypes.

Globoid cell leukodystrophy (GLD) is a rare neurodegenerative lysosomal storage disease caused by an inherited deficiency of β-galactocerebrosidase (GALC). GLD pathogenesis and therapeutic correction have been poorly studied in patient neural cells. Here, we investigated the impact of GALC deficiency and lentiviral vector-mediated GALC rescue/overexpression in induced pluripotent stem cell (iPSC)-derived neural progenitors and neuronal/glial progeny obtained from two GLD patients.

In Nasal Mucosal Secretions, Distinct IFN and IgA Responses Are Found in Severe and Mild SARS-CoV-2 Infection.

Likely as in other viral respiratory diseases, SARS-CoV-2 elicit a local immune response, which includes production and releasing of both cytokines and secretory immunoglobulin (SIgA). Therefore, in this study, we investigated the levels of specific-SIgA for SARS-CoV-2 and cytokines in the airways mucosa 37 patients who were suspected of COVID-19. According to the RT-PCR results, the patients were separated into three groups: negative for COVID-19 and other viruses (NEGS, = 5); negative for COVID-19 but positive for the presence of other viruses (OTHERS, = 5); and the positive for COVID-19 (COVID-19, = 27).

Point-of-care lung ultrasound imaging in pediatric COVID-19.

Background: There has been limited data regarding the usefulness of lung ultrasound (US) in children with COVID-19.

Objective: To describe lung US imaging findings and aeration score of 34 children with COVID-19.

Methods: This study included 0-16-year-old patients with confirmed COVID-19, who were admitted between April 19 and June 18, 2020 in two hospitals in the city of Sao Paulo, Brazil.

Tracking Hydroplasticization by DSC: Movement of Water Domains Bound to Poly(Meth)Acrylates during Latex Film Formation.

The film formation step of latexes constitutes one of the challenges of these environmentally friendly waterborne polymers, as the high glass transition () polymers needed to produce hard films to be used as coatings will not produce coherent films at low temperature. This issue has been dealt by the use of temporary plasticizers added with the objective to reduce the of the polymers during film formation, while being released to the atmosphere afterwards. The main problem of these temporary plasticizers is their volatile organic nature, which is not recommended for the environment.

Multisystem Inflammatory Syndrome Associated With COVID-19 With Neurologic Manifestations in a Child: A Brief Report.

Although first considered a benign infection, recent studies have disclosed severe and potentially lethal inflammatory manifestations of COVID-19 in children. We report the case of a 4-year-old child with a post-infectious multisystem inflammatory syndrome associated with COVID-19, with a Kawasaki-like shock and prominent neurologic features, for whom a cytokine storm and reduced brain-derived neurotrophic factor were well documented.

Frataxin gene editing rescues Friedreich's ataxia pathology in dorsal root ganglia organoid-derived sensory neurons.

Friedreich's ataxia (FRDA) is an autosomal-recessive neurodegenerative and cardiac disorder which occurs when transcription of the FXN gene is silenced due to an excessive expansion of GAA repeats into its first intron. Herein, we generate dorsal root ganglia organoids (DRG organoids) by in vitro differentiation of human iPSCs. Bulk and single-cell RNA sequencing show that DRG organoids present a transcriptional signature similar to native DRGs and display the main peripheral sensory neuronal and glial cell subtypes.

Lower cost alternatives for molecular diagnosis of COVID-19: conventional RT-PCR and SYBR Green-based RT-qPCR.

In March 2020, WHO declared a pandemic state due to SARS-CoV-2 having spread. TaqMan-based real-time RT-qPCR is currently the gold standard for COVID-19 diagnosis. However, it is a high-cost assay, inaccessible for the majority of laboratories around the world, making it difficult to diagnose on a large scale.

Design of Waterborne Asymmetric Block Copolymers as Thermoresponsive Materials.

AB diblock waterborne copolymers made of styrene (St) and 2-ethylhexyl acrylate (2EHA) were synthesized by means of two-step reversible addition fragmentation chain transfer (RAFT) (mini)emulsion polymerization. Monofunctional asymmetric RAFT agent was used to initiate the polymerization. The hard polystyrene "A" block was synthesized via miniemulsion polymerization followed by 2EHA pre-emulsion feeding to form the soft "B" block.

Toxicity and postwithdrawal effects of ketamine on the reproductive function of male albino rats: Hormonal, histological, and immunohistochemical study.

Ketamine is increasingly used in clinical practice, and ketamine addiction is common in young individuals. There are limited reviews on the chronic effects of ketamine on the testes. Three groups of rats received saline or ketamine 50 mg/kg/day intraperitoneally for 6 weeks with or without a subsequent 4-week drug-free period.

Chromosome Transplantation: A Possible Approach to Treat Human X-linked Disorders.

Many human genetic diseases are associated with gross mutations such as aneuploidies, deletions, duplications, or inversions. For these "structural" disorders, conventional gene therapy, based on viral vectors and/or on programmable nuclease-mediated homologous recombination, is still unsatisfactory. To correct such disorders, chromosome transplantation (CT), defined as the perfect substitution of an endogenous defective chromosome with an exogenous normal one, could be applied.