Severe combined immunodeficiency (SCID) is fatal unless treated with hematopoietic stem cell transplant. Delay in diagnosis is common without newborn screening. Family history of infant death due to ...infection or known SCID (FH) has been associated with earlier diagnosis.
The aim of this study was to identify the clinical features that affect age at diagnosis (AD) and time to the diagnosis of SCID.
From 2005 to 2016, 147 SCID patients were referred to the Asian Primary Immunodeficiency Network. Patients with genetic diagnosis, age at presentation (AP), and AD were selected for study.
A total of 88 different SCID gene mutations were identified in 94 patients, including 49
mutations, 12
mutations, 8
mutations, 7
mutations, 4
mutations, 4
mutations, 2
mutations, and 2
mutations. A total of 29 mutations were previously unreported. Eighty-three of the 94 patients fulfilled the selection criteria. Their median AD was 4 months, and the time to diagnosis was 2 months. The commonest SCID was X-linked (
= 57). A total of 29 patients had a positive FH. Candidiasis (
= 27) and bacillus Calmette-Guérin (BCG) vaccine infection (
= 19) were the commonest infections. The median age for candidiasis and BCG infection documented were 3 months and 4 months, respectively. The median absolute lymphocyte count (ALC) was 1.05 × 10
/L with over 88% patients below 3 × 10
/L. Positive FH was associated with earlier AP by 1 month (
= 0.002) and diagnosis by 2 months (
= 0.008), but not shorter time to diagnosis (
= 0.494). Candidiasis was associated with later AD by 2 months (
= 0.008) and longer time to diagnosis by 0.55 months (
= 0.003). BCG infections were not associated with age or time to diagnosis.
FH was useful to aid earlier diagnosis but was overlooked by clinicians and not by parents. Similarly, typical clinical features of SCID were not recognized by clinicians to shorten the time to diagnosis. We suggest that lymphocyte subset should be performed for any infant with one or more of the following four clinical features: FH, candidiasis, BCG infections, and ALC below 3 × 10
/L.
•DC transmission lines can produce severe corona discharge and audible noise (AN) at high altitudes.•A climate tank test platform with adjustable air pressure and humidity was built in the paper.•The ...corona current pulse and AN data in the humidity range of 45 % to 90 % and the air pressure range of 78.9 to 56.04 kPa were obtained.•AN level increased significantly with decreasing air pressure and with increasing relative humidity, the changes were small.
DC transmission lines can produce more severe corona discharge and audible noise (AN) at high altitudes than that in low elevation areas, which is an important factor to be considered in line erection and selection. Besides, the humidity can also affect the performance of corona discharge and AN level. In this paper, a climate tank test platform with adjustable air pressure and humidity was built, and the characteristics of positive DC corona discharge and AN level of stranded wire conductor were investigated. The corona current pulse and AN data in the humidity range of 45 % to 90 % and the air pressure range of 78.9 to 56.04 kPa were obtained, and the variation were analyzed. It is found that, as the air pressure decrease, the A-weighted AN level increase clearly. With the increase of relative humidity, the A-weighted AN level fluctuated, but the change is relatively small.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Abstract
With the vigorous growth of UHV transmission projects, transmission lines crossing high-altitude areas are inevitable, and the degree of corona discharge in high-altitude areas is more ...intense. The electromagnetic environmental problems caused by corona discharge have gradually become one of the key technical problems restricting UHV transmission in high-altitude areas. To investigate how height affects the positive polarity corona’s corona characteristics, using the movable small corona cage to carry out experiments at four altitude points in the range of 1120 m–4320 m, and measures the corona current pulse and radio interference level of 0.8 mm diameter fine copper wire under different positive voltages. Several conclusions can be drawn from the data analysis, including the following: The average current pulse amplitude grows with altitude, first before decreasing, and the current pulse repetition frequency rises along with altitude. The major conclusions of this essay can serve as a starting point for the study of the corona effect.
In this study, complicated model sulfur compounds in bunker oil and de-asphalted bunker oil were biodesulfurized in a batch process by microbial consortium enriched from oil sludge. Dibenzothiophene ...(DBT) and benzobnaphtho1,2-dthiophene (BNT1) were selected as model sulfur compounds. The results show that the mixed culture was able to grow by utilizing DBT and BNT1 as the sole sulfur source, while the cell density was higher using DBT than BNT1 as the sulfur source. GC-MS analysis of their desulfurized metabolites indicates that both DBT and BNT1 could be desulfurized through the sulfur-specific degradation pathway with the selective cleavage of carbon–sulfur bonds. When DBT and BNT1 coexisted, the biodesulfurization efficiency of BNT1 decreased significantly as the DBT concentrations increased (>0.1 mmol/L). BNT1 desulfurization efficiency also decreased along with the increase of 2-hydroxybiphenyl as the end product of DBT desulfurization. For real bunker oil, only 2.8 % of sulfur was removed without de-asphalting after 7 days of biotreatment. After de-asphalting, the biodesulfurization efficiency was significantly improved (26.2–36.5 %), which is mainly attributed to fully mixing of the oil and water due to the decreased viscosity of bunker oil.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Abstract
Because of the low normal atmospheric pressure and frequent, more severe corona discharges in high-altitude regions. The electromagnetic environment problems caused by the corona discharge ...restrict the line design in high-altitude areas. The variance in the law of negative DC corona characteristics under different air pressure conditions is derived using an artificial climate tank with adjustable air pressure. The characteristics of the negative DC needle-plate corona discharge were measured experimentally. The negative DC corona discharge pulses at 89.4 kPa–56.04 kPa were obtained, and the characteristics of the current pulses were statistically examined. The study of the mechanism of the DC corona discharge can benefit from the research findings as essential reference data.
Abdominal aortic aneurysms (AAAs) elicit massive inflammatory leukocyte recruitment to the aorta. CD4+ T cells, which include regulatory T cells (Tregs) and conventional T cells (Tconvs), are ...involved in the progression of AAA. Tregs have been reported to limit AAA formation. However, the function and phenotype of the Tconvs found in AAAs remain poorly understood. We characterized aortic Tconvs by bulk RNA sequencing and discovered that Tconvs in aortic aneurysm highly expressed Cxcr6 and Csf2. Herein, we determined that the CXCR6/CXCL16 signaling axis controlled the recruitment of Tconvs to aortic aneurysms. Deficiency of granulocyte‐macrophage colony‐stimulating factor (GM‐CSF), encoded by Csf2, markedly inhibited AAA formation and led to a decrease of inflammatory monocytes, due to a reduction of CCL2 expression. Conversely, the exogenous administration of GM‐CSF exacerbated inflammatory monocyte infiltration by upregulating CCL2 expression, resulting in worsened AAA formation. Mechanistically, GM‐CSF upregulated the expression of interferon regulatory factor 5 to promote M1‐like macrophage differentiation in aortic aneurysms. Importantly, we also demonstrated that the GM‐CSF produced by Tconvs enhanced the polarization of M1‐like macrophages and exacerbated AAA formation. Our findings revealed that GM‐CSF, which was predominantly derived from Tconvs in aortic aneurysms, played a pathogenic role in the progression of AAAs and may represent a potential target for AAA treatment.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Immune cell infiltration is a significant pathological process in abdominal aortic aneurysms (AAA). T cells, particularly CD4+ T cells, are essential immune cells responsible for substantial ...infiltration of the aorta. Regulatory T cells (Tregs) in AAA have been identified as tissue‐specific; however, the time, location, and mechanism of acquiring the tissue‐specific phenotype are still unknown. Using single‐cell RNA sequencing (scRNA‐seq) on CD4+ T cells from the AAA aorta and spleen, we discovered heterogeneity among CD4+ T cells and identified activated, proliferating and developed aorta Tregs. These Tregs originate in the peripheral tissues and acquire the tissue‐specific phenotype in the aorta. The identification of precursors for Tregs in AAA provides new insight into the pathogenesis of AAA.
This study utilised scRNA‐seq of CD4+ T cells in the AAA aorta and spleen to reveal the complex infiltration of CD4+ T cells in the AAA aorta and the process by which Tregs acquire a tissue‐specific phenotype in AAA. Aorta Tregs originate in the spleen and develop the tissue Treg phenotype in the aorta.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
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