Clinical and Treatment History of Patients with Partial DiGeorge Syndrome and Autoimmune Cytopenia at Multiple Centers.

BACKGROUND: Patients with partial DiGeorge syndrome (pDGS) can present with immune dysregulation, the most common being autoimmune cytopenia (AIC). There is a lack of consensus on the approach to type, combination, and timing of therapies for AIC in pDGS. Recognition of immune dysregulation early in pDGS clinical course may help individualize treatment and prevent adverse outcomes from chronic immune dysregulation. OBJECTIVES: Objectives of this study were to characterize the natural history, immune phenotype, and biomarkers in pDGS with AIC. METHODS: Data on clinical presentation, disease severity, immunological phenotype, treatment selection, and response for patients with pDGS with AIC were collected via retrospective chart review. Flow cytometric analysis was done to assess T and B cell subsets, including biomarkers of immune dysregulation. RESULTS: Twenty-nine patients with the diagnosis of pDGS and AIC were identified from 5 international institutions. Nineteen (62%) patients developed Evan's syndrome (ES) during their clinical course and twenty (69%) had antibody deficiency syndrome. These patients demonstrated expansion in T follicular helper cells, CD19hiCD21lo B cells, and double negative cells and reduction in CD4 naïve T cells and regulatory T cells. First-line treatment for 17/29 (59%) included corticosteroids and/or high-dose immunoglobulin replacement therapy. Other overlapping therapies included eltrombopag, rituximab, and T cell immunomodulators. CONCLUSIONS: AIC in pDGS is often refractory to conventional AIC treatment paradigms. Biomarkers may have utility for correlation with disease state and potentially even response to therapy. Immunomodulating therapies could be initiated early based on early immune phenotyping and biomarkers before the disease develops or significantly worsens.

Quantitative Real-Time Analysis of Living Materials by Stimulated Raman Scattering Microscopy.

Composite materials built in part from living organisms have the potential to exhibit useful autonomous, adaptive, and self-healing behavior. The physicochemical, biological, and mechanical properties of such materials can be engineered through the genetic manipulation of their living components. Successful development of living materials will require not only new methods for design and preparation but also new analytical tools that are capable of real-time noninvasive mapping of chemical compositions. Here, we establish a strategy based on stimulated Raman scattering microscopy to monitor phosphatase-catalyzed mineralization of engineered bacterial films in situ. Real-time label-free imaging elucidates the mineralization process, quantifies both the organic and inorganic components of the material as functions of time, and reveals spatial heterogeneity at multiple scales. In addition, we correlate the mechanical performance of films with the extent of mineralization. This work introduces a promising strategy for quantitatively analyzing living materials, which should contribute to the accelerated development of such materials in the future.

Nanofibrous PAN-PDMS Films-Based High-Performance Triboelectric Artificial Whisker for Self-Powered Obstacle Detection.

Avoiding collisions is a key necessity for any autonomous mobile robot, and obstacle mapping enables them to maneuver in an uncharted area. In this era of the Internet of Things, with the emerging need for a multitude of sensors, adopting self-powered technologies is more practically viable than batteries for powering the same. Herein, with the fabrication of a triboelectric artificial whisker (TAW), a self-powered obstacle detection is demonstrated via tactile perception. The mechanical contact with the obstacle gives rise to an electrical signal from the TAW owing to the embedded triboelectric sensor. In addition, the triboelectric nanogenerator (TENG) based on electrospun polyacrylonitrile (PAN) nanofibers and polydimethylsiloxane film, which facilitates this self-powered artificial sensation, generates an output voltage of 720 V and current density of 5 mA m-2 with 1.7 W m-2 of maximum power delivery from a force of 10 N. The electro-spinning aided enhancement in contact area of the PAN is responsible for the remarkable improvement in the performance of the TENG, 3.4 times enhancement in power density, when compared to the nonsurface-modified ones. In addition, the TENG is able to charge commercial capacitors up to appreciable values and demonstrates powering different electronic gadgets such as calculators and thermometers.

Analysing the role of Fe (II) on flocculation of sand-clay mixtures under estuarine mixing.

Estuaries are fragile environment that are most affected by climate change. One of the major consequences of climate change on estuarine processes is the enhancement in salt intrusion leading to higher salinity values. This has several implications on the estuarine sediment dynamics. Of the various factors that affect the flocculation of cohesive sediments, salinity and turbulence have been recognized as to have great significance. Many of the estuaries are contaminated with heavy metals, of which, the concentration of Iron (Fe (II)) are generally on the higher range. However, the influence of Fe (II) on the flocculation of cohesive sediments at various estuarine mixing conditions is not well known. The present study investigated the influence of Fe (II) on the flocculation of kaolin at various concentration of Fe (II), salinity and turbulence shear. The results indicated that Fe (II) and salinity have a positive influence on kaolin flocculation. The increase in turbulence shear caused an initial increase and then a decrease in floc size. In case of sand-clay mixtures, that are observed in mixed sediment estuarine environments, a reduction in the floc size was observed, which is attributed to the breakage of flocs induced by the shear of sand. Breakage coefficient, which is a measure of break-up of flocs, is generally adopted as 0.5 assuming binary breakage. The present study revealed that the breakage coefficient can take values from 0 to 1 and is a direct function of Fe (II) and salinity and an inverse function of turbulence and sand concentration. Thus, a new model for breakage coefficient with the influencing parameters has been proposed, which is an improvement of existing model that is expressed in terms of turbulence alone. Sensitivity analysis showed that the proposed model can very well predict the breakage coefficient of Fe (II) - kaolin flocs. Thus, the model can quantify the breakage coefficient of flocs in estuaries contaminated with Fe (II) that is a vital parameter for population balance models.

Analysing the performance of the NARX model for forecasting the water level in the Chikugo River estuary, Japan.

Estuaries are dynamic environments which are driven by various natural processes like river discharge, tides, waves, influx of saline water and sediments, etc. These ecosystems are the most sensitive to sea level rise and fluctuations in river discharge associated with climate change. A direct response of sea level rise and river discharge can be observed in the water level of estuaries. However, existing models have not considered these parameters for forecasting water level. This paper focuses on developing a water level forecast model for the Chikugo River estuary in Japan using Nonlinear Autoregressive with Exogenous inputs (NARX Model). NARX neural network was used to do the one-step-ahead prediction of water level considering the various parameters that can very well be influenced by climate change: previous water level, river discharge, and salinity. Accordingly, three models were developed: (i) Model I considering previous water level; (ii) Model II additionally considering river discharge; and (iii) Model III additionally considering salinity. All the models showed appreciable performance in forecasting the water level. Model III had the best correlation with the water level with a cross-correlation value of 0.6030, while the river discharge had only a cross-correlation of 0.1113 indicating that the Chikugo River estuary is tide-dominated. The model was trained using different combinations of available data - previous water level, river discharge, and salinity. Cross-correlation results showed a better correlation between water level and salinity than various other combinations trained. Therefore, tidal intrusion influences the water level in the estuary, thereby depicting that sea level rise can affect the water level, and its influence can be well predicted by the developed model. The water level significantly affects the flora and fauna and the predictability of future estuarine floods can help in taking necessary mitigation strategies.

Adult self-reported childhood maltreatment types are associated with treatment satisfaction and alcohol relapse in patients with comorbid substance use and mental health disorders.

BACKGROUND AND OBJECTIVES: Individuals with comorbid substance use and mental health disorders (concurrent disorders; CD) report poor treatment outcomes, high prevalence of childhood maltreatment, and mostly negative experiences with treatment. No studies to date have examined childhood maltreatment and treatment outcomes in CD. This study investigated self-reported childhood maltreatment as it relates to treatment satisfaction and substance use relapse among CD patients. METHODS: The 258 CD inpatients completed a self-report questionnaire package, comprising the Childhood Trauma Questionnaire and the Inpatient Consumer Survey (ICS). Childhood maltreatment was assessed according to five subtypes and self-perceived treatment satisfaction was rated across six ICS domains. Psychiatric diagnoses, substance use status and relapse data were retrieved via patient medical charts. RESULTS: Emotional neglect was associated with lower ratings across all ICS domains and physical neglect was associated with a lower rating for 'outcome of care'. Childhood sexual abuse was associated with a greater likelihood of alcohol relapse. No other relationships were statistically significant. DISCUSSION AND CONCLUSIONS: The presence of childhood neglect (but not abuse) was more associated with overall treatment dissatisfaction, and sexual abuse alone increased the likelihood of alcohol relapse. These findings suggest some early adverse experiences in CD patients may increase negative experiences in treatment while others contribute to the risk of substance use. Broader longitudinal research is needed to examine the trajectory leading to negative outcomes. SCIENTIFIC SIGNIFICANCE: This is the first study to report differential patterns of association by type of childhood maltreatment on negative outcomes in treatment among CD patients.

Evaluation of a scoring system for vein suitability in platelet apheresis donors.

INTRODUCTION: Donor vein assessment for the selection of good quality veins is crucial for a successful apheresis procedure. This study intends to find out the effectiveness of a vein assessment scoring tool (VST) used and found to be effective in selecting whole blood donors to reduce the difficulty in identifying good quality veins for the plateletpheresis procedure. MATERIALS AND METHODS: This was a prospective observational study on platelet apheresis donors with the application of a VST consisting of three vein descriptor parameters (vein visibility, vein palpability, and vein size) with 5 Likert-type responses constituting a score of 0-12 for each arm. Two vein assessors independently evaluated the vein in both arms and marked their responses blinded from each other as well from the principal investigator. The scores were then calculated and analyzed at the end of the study for their association with phlebotomy and procedural outcomes. RESULTS: A total of 190 donors were recruited. The mean scores for the arms with successful and failed phlebotomy were 9.1 and 9.4 (SD 2.3), respectively. The intra-class correlation Alpha Cronbach value was 0.834 and 0.837 for total scoring in the left arm and right arm, respectively, between the two assessors. Scores neither showed a correlation with other outcomes like low flow alarms, hematoma formation, number of phlebotomy attempts, and procedure completion. CONCLUSION: The study showed that the vein score tool did not truly predict the phlebotomy outcome in apheresis donors, though there was a good degree of inter-assessor reliability.

Mechanical properties of tissue adhesives used for retaining extraoral silicone prostheses in maxillofacial defects: A systematic review.

STATEMENT OF PROBLEM: Different tissue adhesives are available for retaining extraoral silicone prostheses in maxillofacial defects. Comparative assessment of their mechanical properties will help the clinician select the right product for a specific clinical situation, but a systematic review is lacking. PURPOSE: The purpose of this systematic review was to analyze the existing data in the literature regarding 5 mechanical properties of tissue adhesives for extraoral silicone prostheses: peel strength, tensile strength, shear strength, torsional strength, and tack. MATERIAL AND METHODS: A manual and electronic search was performed in appropriate databases to identify relevant publications with specific inclusion and exclusion criteria. The retrieved studies were screened for eligibility using the title, abstract, and published full texts. To evaluate the risk of bias, a methodological quality assessment was performed using the Joanna Briggs Institute Critical Appraisal Checklists for Randomized Controlled Trials. A custom data extraction template was used, and the results pooled using descriptive methods. RESULTS: After screening, 15 articles, 5 in vitro studies, and 10 clinical studies were eligible for data extraction. In vitro studies assessed tack and peel bond strength. In contrast, clinical studies assessed peel strength, tensile strength, shear strength, and torsional strength through direct comparisons and in diverse settings. CONCLUSIONS: The assessed studies showed considerable methodological heterogeneity. When silicone-based tissue adhesives (Secure2 Medical Adhesive; Factor II, Hollister Colostomy Adhesive; Hollister Inc, Dow Corning 355 Medical adhesives; Dow Corning Europe Inc) were compared with water-based adhesives (Pros-Aide Adhesive; ADM Tronics Inc, PSA 1; Cosmedica Ltd, Daro adhesive; Factor II, Epithane-3; Daro Products), the silicone-based adhesives showed a higher peel bond strength (PBS), while double-sided medical adhesive tapes showed lower PBS. A few studies evaluated variations in the PBS as being affected by the addition of stone wool fibers, immersion in water, application of skin protective dressings and adhesive removers, application of multiple layers of adhesive, and usage of urethane liner.

Implications of solid waste dumps on the microplastic abundance in groundwater in Kollam, India.

Water pollution caused by microplastics has garnered increasing attention in recent years due to its potential ecological and human health risks. However, there are very limited studies and a general lack of consensus regarding the presence and negative impacts of microplastics in groundwater. Due to their small size, microplastics can easily be transported at surface as well as subsurface levels, potentially reaching the groundwater table and contaminating the groundwater system This contamination is expected to occur more rapidly in landfill areas and other locations where plastic waste is dumped. In this study, we examined well water samples collected from areas near various dumping sites to assess the abundance and characteristics of microplastics. An average of 12 items/L of microplastics were found in groundwater wells near eight dumping sites in Kollam Corporation. The predominant shape of microplastics in the groundwater samples was fibres, followed by flakes, with black being the predominant colour. The areal extent of solid waste dumping was observed to have an influence on the abundance of microplastics. Additionally, the pH of groundwater near heavy dumping sites was found to be in the acidic range, indicating the intrusion of leachate from dumps into groundwater. The study revealed that the leachate from solid waste dumps is the primary source of microplastics in groundwater. Furthermore, a risk assessment of the microplastic pollution was carried out using an index namely Microplastic Pollution Index and the areas of high risks were identified. The locations having heavy solid waste dumping and those near coastal areas were observed to be at high risk, thereby indicating that both the leachate from dumps and sea water intrusion can cause higher microplastic pollution risk in the groundwater system. The findings of this study are expected to support managers in formulating and implementing effective solid waste management plans to mitigate microplastic pollution in the groundwater system.

Fibroblast Activation Protein Regulates Lesion Burden and the Fibroinflammatory Response in Apoe-Deficient Mice in a Sexually Dimorphic Manner.

Fibroblast activation protein (FAP) has been established as an inducible and mesenchymal cell-specific mediator of disease progression in cancer and fibrosis. Atherosclerosis is a fibroinflammatory disease, and FAP was previously reported to be up-regulated in human atherosclerotic plaques compared with normal vessel. We investigated the spatial and temporal distribution of Fap-expressing cells in a murine model of atherosclerosis and used a genetic approach to determine if and how Fap affected disease progression. Fap was found to be expressed predominantly on vascular smooth muscle cells in lesions of athero-prone Apoe-/- mice. Global deletion of Fap (Fap-/-) in Apoe-/- mice accelerated atherosclerotic disease progression in both males and females, with the effect observed earlier in males. Sex-specific effects on lesion morphology were observed. Relative levels of extracellular matrix, fibrotic, and inflammatory cell content were comparable in lesions in male mice regardless of Fap status. In contrast, lesions in Fap-/- female mice were characterized by a more fibrotic composition due to a reduction in inflammation, specifically a reduction in Mox macrophages. Combined, these data suggest that Fap restrains the progression of atherosclerosis and may contribute to the sexually dimorphic susceptibility to atherosclerosis by regulating the balance between inflammation (an indicator of vulnerability to plaque rupture) and fibrosis (an indicator of plaque stability).

Effect of preventive measures in the containment of SARS-CoV-2 epidemic: a comparative study.

From the time when the first cases of COVID-19 were reported in Wuhan City, China, in December 2019, strict regulations are being implemented by policy makers to contain the spread of the virus. The measures taken in different countries spanned from complete isolation and lockdown to different degrees of restrictions to people's movement, contact between people, hygiene and sanitation. Accordingly, the success in containing the virus also differed. Italy was one among the worst-affected countries in the world despite the lockdown measures adopted. A combination of lockdown and Level-3 State of Emergency measures were adopted in Portugal and South Africa, which helped to delay and flatten the epidemic curve. The timely application of Level-3 State of Emergency in Mozambique resulted in recording low infection rates. Above all the tripod, orderly movement of people, social distance and hygiene and sanitation is the keystone measure to prevent spread of the virus. However, for successful outcome, the measures have to be tailored to the local context.

Validation of the Hubli Grading System for Assessing Unilateral and Bilateral Cleft Lip Outcomes.

BACKGROUND: When determining whether a cleft lip repair outcome is acceptable, the severity of a patient's cleft at initial presentation must be considered. Currently, there is not a widely agreed upon scale for grading the pre-operative severity or post-operative surgical outcome for patients with unilateral and/or bilateral cleft lip. The aim of this study is to validate the Hubli grading system as a reliable and reproducible system for characterizing cleft lip repair outcomes. METHODS: Craniofacial surgeon graders independently evaluated 2489 patients from 56 different countries undergoing both unilateral and bilateral cleft lip repair from April 2004 to December 2018. Preoperative severity and postoperative surgical results were assessed, and these grades were combined to determine surgical outcome acceptability based on initial cleft severity. Intrarater and interrater reliability were calculated as percentages of agreement. RESULTS: Intrarater and interrater acceptability scores had an agreement of 94.50% and 87.04%, respectively. These results indicate that using the Hubli grading system, independent raters are able to reliably and reproducibly measure the acceptability of a cleft lip repair. CONCLUSIONS: This study validates the use of the Hubli grading system to evaluate the acceptability of unilateral and bilateral cleft lip repairs. This is the largest study to date to evaluate cleft lip severity and repair outcomes, and the Hubli grading system is the only scale to determine the acceptability of surgical outcomes based on initial cleft severity. This system is designed to identify surgeons with sub-optimal outcomes in order to implement timely, individualized training on a global scale.

A Global Evaluation of Surgical Techniques and Results of Unilateral Cleft Lip Repairs.

BACKGROUND: Variations in the clinical presentation of the unilateral cleft lip resulted in numerous surgical repair techniques used around the world. No universally accepted method exists to evaluate aesthetic surgical results. The purpose of this study is to use the Hubli lip grading system, developed by Smile Train, to evaluate the aesthetic results of unilateral cleft lip repairs based on the surgical technique used. METHODS: A retrospective review was conducted of unilateral cleft lip repair operations performed by Smile Train surgeons throughout Africa, Americas, Asia Pacific, and Europe and Middle East between 2014 and 2018. Using the Hubli lip grading criteria, factoring in cleft severity, acceptable outcomes were identified and compared with the surgical technique used. RESULTS: Eight thousand forty-one unilateral cleft lip repairs were reviewed. The majority utilized the rotational advancement technique (n = 5541, 68.9%) with a statistically significant percentage of acceptable outcomes (92.1%), followed by an acceptance rate of 91.9% with variations of the z-plasty technique, 90.5% with the straight-line repair and its variations, and 88.4% using "other" techniques (P < 0.05). The rotational advancement had the highest acceptable outcomes for complete cleft lips (94.7%, P < 0.0001), associated alveolar clefting (94.2%, P < 0.05), and concomitant clefting of the hard and/or soft palate (94.3%, P < 0.05). CONCLUSION: Regardless of surgical technique used, Smile Train surgeons maintain high rates of successful surgical outcomes. Results of our study will help complement and support existing medical infrastructure and training systems Smile Train employs for surgeons working with comprehensive cleft care teams around the world.

Postoperative Imaging After Lobectomy: Predicting the Displacement and Change in Orientation of Nonresected Lung Nodules.

OBJECTIVES: The objective of this study was to determine the effect of a lobectomy to the location and orientation of nonresected lung nodule and its corresponding airway. METHODS: We reviewed preoperative and postoperative computed tomography of patients who underwent lobectomies and have a separate nonresected nodule in the ipsilateral lung. Displacement of the nonresected nodule and angulation of its corresponding segmental bronchus were measured. RESULTS: Fifty nodules from 40 patients (30 females, 10 male; mean ± SD age, 67 ± 7 years) were assessed. Nodules are displaced clockwise after right upper, right middle, and left lower lobectomies and counterclockwise after right lower and left upper lobectomies. Displacement of the remaining nodules was greater in the craniocaudal plane, followed by anteroposterior and transverses planes (mean, 3.7, 2.5, and 1.9 cm, respectively). CONCLUSIONS: Remaining ipsilateral nodules and their associated segmental airways are displaced in a predictable fashion after lobectomy. This may help in the assessment of follow-up imaging.

Effect of Acidic Agents on Microhardness and Surface Morphology of Two Metal Ceramic Materials: An In Vitro Study.

AIM: The aim of the present study was to evaluate the effect of acidic agents on microhardness and surface morphology of two metal ceramic materials at various intervals. MATERIALS AND METHODS: A total of 120 metal ceramic disks were prepared and divided into two categories with 60 each. Each category was divided into two groups, group I (Made of IPS d.SIGN) and group II (made of VITA VMK), containing 30 samples each, which was subdivided into three subgroups each containing 10 samples A(i) B(i) acting as control, A(ii) B(ii) immersed in 0.5% HCl, and A(iii) B(iii) immersed in soft cola drink. Group I was tested for microhardness with Vickers microhardness testing machine at an interval of 0 minute, 24 hours, and 168 hours after immersing in acidic agents. Group II was observed for surface morphology changes with scanning electron microscope before immersion, and at an interval of 24 hours and 168 hours after immersing in acidic agents. The results were analyzed statistically, tabulated, and compared. RESULTS: Results revealed that there was a significant decrease in microhardness of IPS d.SIGN and VITA VMK when immersed in acidic agents at an interval of 0 minute, 24 hours, and 168 hours. There were significant surface morphological changes too of both the ceramics before and after immersing in acidic agents at 24 hours and 168 hours. CONCLUSION: Acidic agents evaluated in the present study affected microhardness and surface morphology of both the ceramics, and IPS d.SIGN is the most suitable and would be recommended in people who consume acidic beverages. CLINICAL SIGNIFICANCE: Durability of dental ceramics is very good, but degradation occurs when exposed to acidic agents. All the properties of ceramics are to be known in depth as they are widely used to construct various types of prostheses, and this has to be considered especially in people who consume acidic foods and drinks. How to cite this article: Priya KS, Kumar MP, Krishna VP, et al. Effect of Acidic Agents on Microhardness and Surface Morphology of Two Metal Ceramic Materials: An In Vitro Study. J Contemp Dent Pract 2019;20(11):1329-1334.

Differential arterial and venous endothelial redox responses to oxidative stress.

OBJECTIVE: Oxidative stress is a central event linked with endothelial dysfunction and inflammation in several vascular pathologies, marked by over-production of ROS and concomitant decreases in antioxidants, for example GSH. Here, we distinguish endothelial oxidative stress regulation and associated functional disparities in the two main vascular conduits, (arteries and veins) following decreases in GSH. METHODS: MAECs and VCECs were used as models of arterial and venular endothelium, respectively, and BSO (0-100 µmol/L) was used to indirectly increase cellular oxidative stress. Inflammatory responses were measured using immune cell attachment and immunoblotting for endothelial cell adhesion molecule (ICAM-1, VCAM-1) expression, altered cell proliferation, and wound healing. RESULTS: MAECs and VCECs exhibited differential responses to oxidative stress produced by GSH depletion with VCECs exhibiting greater sensitivity to oxidative stress. Compared to MAECs, VCECs showed a significantly increased inflammatory profile and a decreased proliferative phenotype in response to decreases in GSH levels. CONCLUSIONS: Arterial and venous endothelial cells exhibit differential responses to oxidant stress, and decreases in GSH:GSSG are more exacerbated in venous endothelial cells. Specific pathogenesis in these vascular conduits, with respect to oxidant stress handling, warrants further study, especially considering surgical interventions such as Coronary artery bypass grafting that use both interchangeably.

Stereotactic Ablative Body Radiotherapy for Primary Non-Small-Cell Lung Cancer: Achieving Local Control with a Lower Biologically Effective Dose.

We conducted a retrospective study of stereotactic ablative radiotherapy (SABR) for 94 patients with non-small-cell lung cancer at our institution. The patients were treated with either 50 Gy in five treatments or 48 Gy in four treatments, corresponding to biologically effective doses (BED) of 100 Gy or 105.6 Gy, respectively. The results demonstrate that, with relatively low BEDs, we can achieve excellent local control with minimal toxicity.