Comparative effects of glazing versus polishing on mechanical, optical, and surface properties of zirconia ceramics with different translucencies.

OBJECTIVES: This study compared the effects of glazing versus polishing on mechanical, optical, and surface properties of zirconia ceramics with different translucencies. MATERIALS AND METHODS: In this in vitro study, 120 bar-shaped specimens (25 × 4 × 1.2 mm) were fabricated from three different types of zirconia with different translucencies (n = 40, DD Bio ZW, ZX2, and Cube X2). After sintering, each zirconia group was randomly divided into five subgroups of control (glazing), glazing + bur abrasion, glazing + bur abrasion + polishing with EVE Diacera® kit, glazing + bur abrasion + reglazing, and glazing + bur abrasion + polishing with EVE Diacera® kit + reglazing. The specimens underwent surface roughness, hardness, flexural strength, and translucency tests, as well as X-ray diffraction (XRD) and scanning electron microscopy (SEM) for assessment of surface topography. Data were analyzed by one-way analysis of variance, Tukey test, and Pearson test (α = .05). RESULTS: Flexural strength, surface hardness, and translucency were significantly correlated with zirconia type. ZW zirconia had significantly higher flexural strength and surface hardness and significantly lower translucency than Cube X2 and ZX2 (p < .001). Surface roughness had no significant correlation with zirconia type (p = .274). Polishing created the smoothest, and bur abrasion created the roughest surface (p < .001). Flexural strength and hardness in most experimental groups were significantly lower than in the control group (p < .001). Translucency was not significantly different in bur abrasion and polishing groups, compared with the control group; however, reglazing significantly increased the translucency (p < .001). SEM micrographs confirmed the surface roughness results. XRD showed monoclinic phase only in reglazed groups. CONCLUSION: Of different surface treatments, polishing improved the surface properties and caused the smallest change in mechanical properties of zirconia with different translucencies.

Effect of polishing versus glazing of CAD-CAM ceramics on wear and surface roughness of opposing composite resin.

Background: This study aimed to assess the effect of polishing versus glazing of computer-aided design-computer-aided manufacturing (CAD-CAM) ceramics on depth of wear and surface roughness of opposing composite resin. Materials and Methods: This in vitro study was conducted on 40 Z250 composite and 40 CAD-CAM ceramic specimens including Celtra Duo, Vita Mark II, e.max CAD, and Vita Suprinity ceramics. All ceramic specimens were roughened by a fine-grit bur after primary glazing to simulate an adjusted surface in the clinical setting. They were then randomly assigned to two subgroups and underwent reglazing or polishing. All composite and ceramic specimens underwent profilometry after surface treatment and prior to the wear test, and the results were recorded quantitatively. Composite specimens were then subjected to 120,000 wear cycles against ceramic specimens in a chewing simulator, and the depth of wear was measured by a scanner. Data were statistically analyzed by repeated measures two-way analysis of variance (ANOVA) and one-way ANOVA (α = 0.05). Results: Comparison of the surface roughness of composite specimens before and after the wear test revealed significant differences in both glazed Suprinity (P = 0.048) and Vita Mark II (P = 0.026) ceramics groups. The change in surface roughness after the wear test (compared with baseline) was significant in glazed (P = 0.000) and polished (P = 0.013) Vita Mark II and polished Suprinity (P = 0.037) ceramics, but this change was not significant in other ceramics (P > 0.05). The depth of wear after the wear test was not significantly different among the ceramic and composite subgroups (P > 0.05). Conclusion: Assessment of depth of wear and surface roughness of composite specimens showed that the polishing kits of CAD-CAM ceramics can serve as a suitable alternative to reglazing.

Cycle Threshold Values Predict COVID-19 Severity and Mortality but Are not Correlated with Laboratory Markers.

Many studies have evaluated the possible utility of cycle threshold (Ct) values as a predictor of Coronavirus disease 2019 (COVID-19) severity and patient outcome. Given the inconsistent results, we aimed to evaluate the association between severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) Ct values and disease severity, inflammatory markers, and outcomes in Iranian patients with COVID-19. A retrospective study of 528 patients with COVID-19 hospitalized from September 2020 to October 2021 was conducted. Demographic, clinical, and laboratory data of patients were retrieved from electronic medical records. Ct values were analyzed as a continuous variable after subcategorizing into 3 groups: low (Ct values<20), medium (Ct values 20 to 30), and high (Ct values>30). Of the 528 patients (45.1% female) aged 13 to 97 years, 109 patients had low Ct values, 312 patients had medium, and 107 patients had high Ct values. Patients with low Ct values were more likely to present with critical COVID-19, require invasive mechanical ventilation and develop complications such as acute respiratory distress syndrome and pneumonia. Furthermore, patients with low or medium Ct values were more likely to die compared to patients with high Ct values. Multivariate analysis showed that patients with low or medium Ct values were more likely to have severe COVID-19 compared with patients with high Ct values. The multivariate analysis also showed a higher risk of mortality in patients with low Ct values compared to patients with high Ct values, although this was not statistically significant. Our findings revealed that Ct values were an independent predictor of COVID-19 severity. The risk of mortality was higher in patients with low Ct values. However, further investigation is needed to address the correlation between Ct values and inflammatory factors.

World human neutrophil antigens investigation survey.

BACKGROUND AND OBJECTIVES: Human neutrophil antigens (HNAs) are categorized into five systems: HNA-1 to HNA-5. Given the importance of neutrophils in immunity, we sought to create awareness of the role of HNA diagnostic services in managing immune neutropenia and transfusion-related acute lung injury. To provide health communities all around the world with access to these services, we conducted a survey to create a directory of these HNA diagnostic services. MATERIALS AND METHODS: An Excel table-based survey was created to capture information on the laboratory's location and was emailed to 55 individuals with known or possible HNA investigation activity. The collected data were then summarized and analysed. RESULTS: Of contacted laboratories, the surveys were returned from 23 (38.2%) laboratories; 17 have already established HNA diagnostic (of them 12 were regular participants of the International Granulocyte Immunobiology Workshop [ISBT-IGIW]), 4 laboratories were in the process of establishing their HNA investigation and the remaining 2 responder laboratories, did not conduct HNA investigations. In established laboratories, investigation for autoimmune neutropenia (infancies and adults) was the most frequently requested, and antibodies against HNA-1a and HNA-1b were the most commonly detected. CONCLUSION: The directory of survey respondents provides a resource for health professionals wanting to access HNA diagnostic services. The present study offers a comprehensive picture of HNA diagnostics (typing and serology), identifying weak points and areas for improvement for the first time. Identifying more laboratories involved in HNA diagnostics with limited access to international societies in the field will globally improve HNA diagnostics.

Clinical and immunological characteristics of 69 leukocyte adhesion deficiency-I patients.

BACKGROUND: In order to support the comprehensive classification of Leukocyte Adhesion Deficiency-I (LAD-I) severity by simultaneous screening of CD11a/CD18, this study assessed clinical, laboratory, and genetic findings along with outcomes of 69 LAD-I patients during the last 15 years. METHODS: Sixty-nine patients (40 females and 29 males) with a clinical phenotype suspected of LAD-I were referred to Immunology, Asthma, and Allergy research institute, Tehran, Iran between 2007 and 2022 for further advanced immunological screening and genetic evaluations as well as treatment, were enrolled in this study. RESULTS: The diagnosis median age of the patients was 6 months. Delayed umbilical cord separation was found in 25 patients (36.2%). The median diagnostic delay time was 4 months (min-max: 0-82 months). Forty-six patients (66.7%) were categorized as severe (CD18 and/or CD11a: below 2%); while 23 children (33.3%) were in moderate category (CD18 and/or CD11a: 2%-30%). During the follow-ups, 55.1% of children were alive with a mortality rate of 44.9%. Skin ulcers (75.4%), omphalitis (65.2%), and gingivitis (37.7%) were the most frequent complaints. Genetic analysis of the patients revealed 14 previously reported and three novel pathogenic mutations in the ITGB2 gene. The overall survival of patients with and without hematopoietic stem cell transplantation was 79.3% and 55.6%, respectively. CONCLUSION: Physicians' awareness of LAD-I considering delayed separation of umbilical cord marked neutrophilic leukocytosis, and variability in CD11 and CD18 expression levels, and genetic analysis leads to early diagnosis and defining disease severity. Moreover, the prenatal diagnosis would benefit families with a history of LAD-I.

Microtensile Bond Strength of Different Bonding Agents to Superficial and Deep Dentin in Etch-and-Rinse and Self-Etch Modes.

Objectives: This study aimed to assess the microtensile bond strength (µTBS) of etch-and-rinse (E&R), self-etch (SE), and universal adhesives to superficial and deep dentin. Materials and Methods: In this in vitro study, 40 sound third molars were randomly divided into two main groups of superficial and deep dentin. Based on our classification, superficial dentin was right beneath the deepest occlusal groove, and deep dentin was 2mm beneath the deepest occlusal groove. Each group was divided into 4 subgroups (n=20) for application of Adper Single Bond 2 (ASB), Clearfil SE Bond (CSE), and Scotchbond Universal (SBU) in E&R and SE modes along with Charisma Smart composite resin on dentin. The specimens were incubated in distilled water at 37°C for 24 hours and their µTBS was then measured. The mode of failure was determined under a stereomicroscope at ×40 magnification. Data were analyzed by one-way ANOVA (alpha=0.05). Results: The highest µTBS belonged to the superficial dentin/SBU/E&R group. The µTBS was significantly higher in superficial dentin than deep dentin for all adhesives (P=0.005). There was no significant difference in mode of failure among the groups. Conclusion: Based on the results obtained in the present study, type of bonding agent and application mode affected µTBS. In use of universal adhesive, E&R mode can improve µTBS.

Surface Micro-Hardness and Wear Resistance of a Self-Adhesive Flowable Composite in Comparison to Conventional Flowable Composites.

Objectives: The durability of composite restorations is directly affected by the mechanical properties of the composite. The aim of this study was to evaluate the hardness and wear resistance of self-adhesive flowable composite (SAF) in comparison with conventional flowable composites. Materials and Methods: In this in vitro study, 50 composite specimens were prepared in brass molds with 10mm ×10mm ×2mm and divided into five groups (n=10). Specimens included three conventional flowable composites (Grandio flow, Filtek flow and Admira fusion flow), one self-adhering flowable composite (SAF, Vertise flow) and a microhybrid composite (filtek z250). After polishing, the micro-hardness of the specimens was measured in a Vickers hardness device, and the specimens were then subjected to 5000, 10000, 20000, 40000, 80000 and 120000 wear cycles in a wear tester. One-way ANOVA/Games-Howell, Kruskal Wallis, and Friedman tests were used for statistical analysis. The significance level was set at P<0.05. Results: The surface micro-hardness of the SAF was significantly lower than that of the microhybrid composite (P=0.01). There was no significant difference between the surface hardness of the different tested flowable composites (P>0.05). Also, the wear resistance of the studied composites was not significantly different in various cycles (P>0.05). Conclusion: Based on our results, SAF would not be an ideal substitute for conventional flowable composites in high-stress areas.

Epilepsy-Related Mortality After Laser Interstitial Thermal Therapy in Patients With Drug-Resistant Epilepsy.

OBJECTIVES: The purpose of this report was to study the incidence of sudden unexpected death in epilepsy (SUDEP) after laser interstitial thermal therapy (LITT) for drug-resistant epilepsy (DRE). METHODS: A prospective observational study of consecutive patients treated with LITT between 2013 and 2021 was conducted. The primary outcome was the occurrence of SUDEP during postoperative follow-up. Surgical outcome was classified according to the Engel scale. RESULTS: There were 5 deaths, including 4 SUDEPs, among 135 patients with a median follow-up duration of 3.5 (range 0.1-9.0) years and a total of 501.3 person-years at risk. The estimated incidence of SUDEP was 8.0 (95% CI 2.2-20.4) per 1,000 person-years. Three SUDEPs occurred in patients with poor seizure outcomes, whereas 1 patient was seizure-free. Compared with pooled historical data, SUDEP occurred at a higher rate than in cohorts treated with resective surgery and at a rate similar to nonsurgical controls. DISCUSSION: SUDEP occurred early and late after mesial temporal LITT. The SUDEP rate was comparable with rates reported in epilepsy surgery candidates who did not receive intervention. These findings reinforce targeting seizure freedom to decrease SUDEP risk, including early consideration for further intervention. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that LITT is not effective in reducing SUDEP incidence in patients with DRE.

Association between postictal EEG suppression, postictal autonomic dysfunction, and sudden unexpected death in epilepsy: Evidence from intracranial EEG.

OBJECTIVE: The association between postictal electroencephalogram (EEG) suppression (PES), autonomic dysfunction, and Sudden Unexpected Death in Epilepsy (SUDEP) remains poorly understood. We compared PES on simultaneous intracranial and scalp-EEG and evaluated the association of PES with postictal heart rate variability (HRV) and SUDEP outcome. METHODS: Convulsive seizures were analyzed in patients with drug-resistant epilepsy at 5 centers. Intracranial PES was quantified using the Hilbert transform. HRV was quantified using root mean square of successive differences of interbeat intervals, low-frequency to high-frequency power ratio, and RR-intervals. RESULTS: There were 64 seizures from 63 patients without SUDEP and 11 seizures from 6 SUDEP patients. PES occurred in 99% and 87% of seizures on intracranial-EEG and scalp-EEG, respectively. Mean PES duration in intracranial and scalp-EEG was similar. Intracranial PES was regional (<90% of channels) in 46% of seizures; scalp PES was generalized in all seizures. Generalized PES showed greater decrease in postictal parasympathetic activity than regional PES. PES duration and extent were similar between patients with and without SUDEP. CONCLUSIONS: Regional intracranial PES can be present despite scalp-EEG demonstrating generalized or no PES. Postictal autonomic dysfunction correlates with the extent of PES. SIGNIFICANCE: Intracranial-EEG demonstrates changes in autonomic regulatory networks not seen on scalp-EEG.

Mapping evidence of Iran diabetes research: protocol for a scoping review.

Background: A considerable amount of research funding goes to diabetes management strategies to improve therapeutic goals and reduce the burden of diabetes. A vast amount of the budget is wasted due to unnecessary studies. A scoping review is a pivotal study to overview the available evidence and avoid research waste. In this review, we will try to find out the scope of available studies on diabetes management interventions, identify associated research gaps, and prioritize future studies. Method: We will carry out a study using Arksey and O'Malley's scoping review framework. We will search the Scopus and PubMed databases from 01/01/2015 till 01/01/2020. Only original articles related to pharmacological and non-pharmacological management interventions will be included. These interventional studies should be conducted on the Iranian population. After data extraction, a descriptive data analysis will be used to present information in different charts or tables. We will evaluate related published articles based on their document type, level of evidence, type of diabetes, subject area, interventions types, main findings and outcomes. Discussion: This study represents the first attempt to sum up available studies related to diabetes management interventions performed in Iran. The results of this study will be useful for all the stakeholders and policy-makers involved in diabetes research. It can help clinicians to be informed about studies on management interventions and can guide scientists eager to diabetes research to choose their future research plans based on diabetes research requirements and gaps.

The Potential of Wearable Devices and Mobile Health Applications in the Evaluation and Treatment of Epilepsy.

Wearable devices and mobile health software applications have a great potential for improving epilepsy-related health outcomes and contributing to personalized medical care for persons with epilepsy. With limitations and challenges, they can be used for tracking seizure occurrence and for seizure detection, prediction, and forecasting in hospital and ambulatory settings. They can also help promote self-monitoring and self-management and thereby contribute to patient empowerment. In this review, we provide an overview of current wearable devices and mobile health software applications for epilepsy. We focus on clinically validated devices, their clinical applications, the challenges faced when using these devices in real-world settings, and how these devices may be optimized in the future.

Evaluation of the effects of different concentrations of bleaching agents on flexural strength and microhardness of VITA ENAMIC.

Background: Considering the studies on the effects of bleaching materials on properties of dental materials, The aims of this in vitro study were to evaluate the effects of two different concentrations of bleaching agents on flexural strength and microhardness of VITA ENAMIC. Materials and Methods: In this experimental in vitro study, 30 rectangular-shaped specimens (2 mm width × 2 mm height × 12 mm length) for flexural strength and 30 specimens (5 mm width × 5 mm length × 2 mm height) for microhardness tests were prepared from VITA ENAMIC blocks 12 × 14 × 18 mm. The specimens were polished using silicon-carbide sandpapers 400, 600, 800, 1200, 2000 under flow of water for 60 s each. The prepared samples for flexural strength and microhardness were divided into 3 subgroups (n = 10): control group (C), samples bleached using Opalescence PF 15% (B15), and samples bleached with Opalescence Xtra Boost 40% (B40). Flexural strength measurement was done using a universal testing machine, and microhardness test was done using Vickers. Data were analyzed using analysis of variance and post hoc tests and P < 0.05 was considered significant. Results: The mean microhardness values of C, B15, and B40 groups were 255.46 ± 3.02, 249.86 ± 4.18, and 235.53 ± 4.61 kgf/mm2. Opalescence PF 15% and Opalescence Xtra Boost 40% affected microhardness of ENAMIC significantly (P < 0.05). The mean flexural strength values of C, B15, and B40 groups were 155.26 ± 16.13, 142.14 ± 11.52, and 133.39 ± 16.13 MPa. A significant decrease in flexural strength was found between the C and B40 groups (P = 0.007). However, the difference between flexural strength of the C and B15 groups was not significant (P > 0.05). Conclusion: Our study showed that both concentrations of bleaching agents can affect microhardness of ENAMIC. Moreover, hydrogen peroxide 40% has a negative effect on the flexural strength of ENAMIC.

Knowledge gaps in diabetes research: an evidence mapping of the literature.

Background: Mapping the available evidence can be used to inform current diabetes research, identify relevant gaps, and prioritize future research. In this regard, we mapped diabetes research performed in Iran. Method: We searched the Scopus and PubMed databases from 01/01/2015 till 01/01/2020 using keywords such as diabetes and Iran. The included articles were classified according to their document types, level of evidence, and subject areas. Results: The majority of the included articles (53%) were related to diabetes types, followed by complications (28%). Most of the documents were original articles (82%), and reviews were 18% of the publications. Systematic reviews constitute only 6% of the total documents. Observational studies were the most common types of study designs (26%), followed by clinical trials (20%). Moreover, topics on control and management of diabetes were the most prevalent subject areas (58%), and fewer studies were on preventive strategies (6%). In diabetes management studies, less attention has been paid to evaluate psychological (10%), educational (9%), and physical activity-related (7%) interventions. There was a shortage of secondary studies related to physical activity, psychology, diagnostic, and screening-related studies. Conclusion: To fill diabetes research gaps, more investment in cost-effectiveness interventions, such as preventive strategies and behavioral self-management programs, need. Moreover, we need to pay more attention on applied sciences and real world evidence to bridge translational gaps from bench to bedside. In this regard, further data synthesis can be helpful in evaluating the effectiveness of the available studies and avoiding unnecessary investigations.

The Immunogenetics of Lichen Planus.

Lichen planus (LP) is a multifaceted autoimmune disease affecting the skin, nails, hair, and mucous membranes, with several clinical subgroups. Cell-mediated immunity plays a key role in its progression. This chapter reviews the known genetic associations of lichen planus including HLA as well as non-HLA genes.

Human Neutrophil Antigen Genotype and Allele Frequencies in Iranian Blood Donors.

OBJECTIVE: Human neutrophil antigens (HNAs) can be targeted by HNA-allo antibodies and cause a variety of clinical conditions such as transfusion-related acute lung injury (TRALI) and neonatal alloimmune neutropenia (NAIN). The current study is aimed at identifying the genotype and allele frequencies of HNAs in Iranian blood donors. METHODS: A total of 150 blood samples were obtained from healthy blood donors. HNA-1, HNA-3, HNA-4, and HNA-5 were genotyped, using the polymerase chain reaction sequence-specific primer (PCR-SSP) technique. The expression of the HNA-2 antigen on the neutrophil surface was evaluated by flow cytometry. RESULTS: The allele frequencies of FCGR3B∗1 (encoding HNA-1a), FCGR3B∗2 (encoding HNA-1b), and FCGR3B∗3 (encoding HNA-1c) were 0.34, 0.63, and 0.03, respectively. For HNA-3, the allele frequencies for SLC44A2∗1 (encoding HNA-3a) and SLC44A2∗2 (encoding HNA-3b) were 0.63 and 0.37, respectively. The frequencies of ITGAM∗1 (encoding HNA-4a) and ITGAM∗2 (encoding HNA-4b) alleles were 0.85 and 0.15, respectively. Furthermore, the frequencies of ITGAL∗1 (encoding HNA-5a) and ITGAL∗2 (encoding HNA-5b) alleles were 0.72 and 0.28, respectively. In the studied population, HNA-2 antigen was present on the neutrophil surface in 97.3% of the individuals, while no detectable HNA-2 expression was observed in 2.7% of the individuals. However, no significant difference in HNA-2 expression between different age groups was found. CONCLUSION: The present study provides the first report of the HNA allele and genotype frequencies among the Iranian population. All HNAs (HNA-1 to HNA-5) were typed using the PCR-SSP and flow cytometer. In the current cohort study, the determined HNA allele frequencies were similar to the previous reports from British, German, and Danish populations. Considering the presence of different Iranian ethnic groups, further studies with a larger sample size are needed to draw a total picture for HNA allele frequencies.

Identification of Antibodies against Neutrophil Surface Antigens in Two Iranian Patients with Autoimmune Neutropenia.

Autoimmune neutropenia is a type of immune-mediated neutropenia, caused by antibody-induced neutrophil destruction. Here we report two cases (3-year-old boy and 9-year-old girl) with suspected autoimmune neutropenia. The presence of neutrophil antibodies in sera of these patients was investigated using standard neutrophil antibody screening tests such as granulocyte immunofluorescence test (GIFT), granulocyte agglutination test (GAT), and lymphocyte immunofluorescence test (LIFT). A positive reactivity with two panel cells was found in GIFT. No reactivities with panel cells were observed in GAT and LIFT. To the best of our knowledge, this is the first report for detecting the neutrophil reactive antibodies using genotyped neutrophils in patients with autoimmune neutropenia in Iran. The final diagnosis of our patients was primary autoimmune neutropenia for the boy and autoimmune neutropenia associated with familial Mediterranean fever for the girl.

Immunological effects of convalescent plasma therapy for coronavirus: a scoping review.

BACKGROUND: Preliminary studies revealed the safety and effectiveness of convalescent plasma (CP) therapy for patients with coronavirus. In this study, we aimed to evaluate and summarize the available evidence on CP therapy, identify the research gap regarding the immunological response to CP therapy and pave the road for future studies. METHODS: This study was conducted according to the Hilary Arksey and Lisa O'Malley framework. To find out the relevant studies, we searched PubMed, Scopus and Embase databases up to 30th May 2021. Data have been extracted according to three categories: (1) patients' characteristics, (2) clinical and immunological responses to CP therapy and (3) pre-infusion screening of the CP samples. RESULTS: A total of 12,553 articles were identified. One hundred fifty-four studies met the inclusion criteria for full-text review. More than half of the included studies (112 studies, (75.6%)) concluded satisfactory outcomes and or safety of CP infusion in patients. Results of studies showed the efficacy of CP therapy in clinical improvement (101 studies), decreasing in the level of inflammatory factors (62 studies), elimination or decreasing in viral load (60 studies), and induction or increase in antibody response (37 studies). Despite these promising results, the results of the 49 studies revealed that CP therapy was ineffective in the survival of patients, clinical improvement, viral infection elimination or decrease in the inflammatory factor levels. Furthermore, the adaptive immune response was evaluated in 3 studies. Information related to the pre-infusion screening for human leukocyte antigen/human neutrophil antigen (HLA/HNA) antibodies was not reported in most of the studies. Our gap analysis revealed that the influence of the CP infusion on the adaptive immune and inflammatory responses in patients with coronavirus needs further investigation. CONCLUSIONS: Based on the results of most included studies, CP infusion was safe and resulted in clinical improvement of patients and decreasing the viral load. The effect of the CP infusion on adaptive immune response and inflammatory cytokines in patients with coronavirus needs further investigation.

Investigating the Variation of TREC/KREC in Combined Immunodeficiencies.

T-cell receptor excision circles (TREC)/Kappa-deleting recombination excision circles (KREC) assay has been recently recognized for detecting patients with primary (T- and/or B-cell) immunodeficiency (PID). We aimed to investigate the alterations of these biomarkers in some combined immunodeficiency patients compared to the healthy controls in different age groups. TREC and KREC were assessed in a total of 82 PID patients, most of them with exact genetic diagnosis (3 months to 42 years); using quantitative real-time-polymerase chain reaction (PCR). Patients had a final diagnosis of common variable immunodeficiency (n=23), ataxia-telangiectasia (AT) (n=17), hyper-IgE syndrome (HIES) (7 with DOCK8 deficiency, 4 with signal transducer and activator of transcription 3 (STAT3) deficiency, and 8 children with unknown genetic defects), Wiskott-Aldrich syndrome (WAS) (n=20), purine nucleoside phosphorylase (PNP)deficiency(n=1), dedicator of cytokinesis2 (DOCK2) deficiency (n=1), recombinase activating gene1 (RAG1) deficiency (n=1). Very low to zero amounts of TREC and/or KREC were detected in 14 out of 23 cases of common variable immunodeficiency (CVID), 14 out of 17 cases of AT, 8 out of 20 cases of WAS, 6 out of 7 cases of DOCK8-deficiency patients, 4 out of 8 cases of HIES with unknown genetic defects and all patients with defects in DOCK2, PNP, and RAG1. STAT3-deficient patients were normal for both biomarkers. All patients showed a significant difference in both markers compared to age-matched healthy controls. Our findings highlight that apart from severe types of T/B cell defects, this assay can also be used for early diagnosis the patients with late-onset of disease and even PIDs without a positive family history.

The probability of sudden unexpected death in epilepsy given postictal prone position.

Sudden Unexpected Death in Epilepsy (SUDEP) is strongly linked to prone position in the immediate aftermath of a generalized tonic-clonic seizure (GTCS). The risk of SUDEP after a GTCS resulting in prone position has not yet been estimated. We calculated the probability of SUDEP given prone position after a GTCS using Bayesian analysis with inputs obtained from known risk of SUDEP and probability of prone position after a GTCS. The risk for SUDEP given the prone position is estimated to be 0.41% (95% Credible Interval 0.13-0.69%). The relative risk of SUDEP in prone vs. non-prone position following a GTCS is estimated to be 63 (95% Credible Interval 30-96). Sudden Unexpected Death in Epilepsy might be prevented by repositioning the patient after a seizure to avoid the prone position.