ICON: The Early Diagnosis of Congenital Immunodeficiencies

Primary immunodeficiencies are intrinsic defects in the immune system that result in a predisposition to infection and are frequently accompanied by a propensity to autoimmunity and/or immunedysregulation. Primary immunodeficiencies can be divided into innate immunodeficiencies, phagocytic deficiencies, complement deficiencies, disorders of T cells and B cells (combined immunodeficiencies), antibody deficiencies and immunodeficiencies associated with syndromes. Diseases of immune dysregulation and autoinflammatory disorder are many times also included although the immunodeficiency in these disorders are often secondary to the autoimmunity or immune dysregulation and/or secondary immunosuppression used to control these disorders. Congenital primary immunodeficiencies typically manifest early in life although delayed onset are increasingly recognized. The early diagnosis of congenital immunodeficiencies is essential for optimal management and improved outcomes. In this International Consensus (ICON) document, we provide the salient features of the most common congenital immunodeficiencies.     

伊朗2009-2016年间传统医学使用及家庭费用支出情况概述

目的:分析2009-2016年间伊朗传统医学使用及家庭费用支出情况的影响因素.方法:基于伊朗家庭收入和支出调查(HIES),采用伪面板数据的Tobit模型分析家庭收入、家庭财富等级、家庭规模、男性数量、识字成员数量、家庭成员平均年龄对传统医学使用及经费支出的影响.结果:研究结果表明,越富裕的家庭使用传统医学的频率越高;...     

Comprehensive meta-analysis of associations between temperament and character traits in Cloninger’s psychobiological theory and mental disorders

ObjectiveTo explore the relationship between temperament and character traits in Cloninger’s psychobiological theory and mental disorders.Methods: A systematic literature search was conducted of five international databases for all articles published in English between January 1990 and April 2019 (PROSPERO-CRD42019133214). Owing to heterogeneity, pooled estimates of correlations for personality disorders and standardized mean differences for case–control studies related to other mental disorders were calculated using the random-effects method.ResultsThe pooled effect sizes obtained from 149 studies showed that high harm avoidance (related to 22/24 diagnostic categories), low self-directedness (21/23), low cooperativeness (17/23), high self-transcendence (14/23), low reward dependence (11/24), high novelty-seeking (10/24), low novelty-seeking (7/24), high persistence (2/23), low persistence (2/23) and high reward dependence (2/24) were related to psychopathology.ConclusionsAll traits provided unique psychobiological tools for differential diagnosis of mental disorders. However, high harm avoidance and low self-directedness played a canonical role in psychopathology. Despite the study limitations, additional studies are warranted to evaluate the differential diagnoses suggested by the present model.     

Enhancement of third order nonlinear optical responses via alteration of the density of states of electrons: VS2–NiS2 hybrid nanostructure

We successfully synthesized VS₂–NiS₂ hybrid nanostructures via a one-pot hydrothermal technique. Microstructural characterizations were carried out by X-ray diffraction (XRD), field emission scanning electron microscopy (FESEM), UV-visible spectroscopy, and Fourier transform infrared spectroscopy (FTIR). The optical indexes such as n, k, and ε were assessed based on the reflectance spectra data and Kramers–Kronig method. By adding varying amounts of the NiS₂ phase in the hybrid nanostructure, the morphology of the hybrid nanostructures is altered to produce microflowers, nanoflakes, and nanoflowers. These changes affect the nonlinear optical properties. The third order nonlinear optical parameters (n₂, β) were analyzed via a simple and accurate Z-scan technique using different laser powers. The two-photon absorption phenomenon has a significant enhancement effect on the nonlinear absorption process, but the nonlinear refractive behaviour of the different hybrid nanostructures changes from self-defocusing to self-focusing due to the alteration of the electronic state alignment and morphology. The magnitudes of n₂ and β are in the order of 10⁻⁹ cm² W⁻¹ and 10⁻³ cm W⁻¹, respectively. The increasing of the density of states of electrons leads to the improvement of the nonlinear optical responses of the VS₂–NiS₂ hybrid nanostructures in comparison with the pure VS₂ structure. This study demonstrates the great potential of this hybrid nanostructure for optical limiters and modulators as well as photonic devices.

Increasing of the density of states of electrons is an important criterion in nonlinear optical phenomena. The hybridization of VS₂ with NiS₂ leads to the improvement of nonlinear optical properties using in the optical and photonic applications.     

Study of programmed cell death 1 (PDCD1) gene polymorphims in Iranian patients with ankylosing spondylitis

Ankylosing spondylitis (AS) is a chronic inflammatory disease, characterized by axial arthritis in which the genetic-environmental factors seem to be involved in the pathogenesis of the disease. This study was performed to investigate the role of polymorphisms of the programmed cell death 1 (PDCD1) gene on susceptibility to AS. In this study, 161 Iranian patients with AS and 208 normal controls were enrolled; two single-nucleotide polymorphisms (SNPs) of the PDCD1 gene PD-1.3 (G, A) in nucleotide position +7146 of intron 4 and PD-1.9 (C, T) in nucleotide +7625 of exon 5 were studied. Analysis of PD-1.3 revealed that 82% of patients and 79% of controls had GG genotype, while GA and AA genotypes were detected in 17% and 0.6% of patients, respectively, and 20% and 1.4% of controls, respectively. Moreover, the genotype CC (PD-1.9) was present in 92% of patients and 97% of controls. Although these differences were not statistically significant between patients and controls, comparisons of genotypes frequencies in the AS patients, based on human leukocyte antigen (HLA)-B27, revealed that all patients who had CT genotype (PD-1.9) were HLA-B27 positive, whereas 30% of patients with CC genotype were HLA-B27 negative. There was no evidence of association for PDCD1 SNPs with AS in our study, but CT genotype (PD-1.9) seems to be associated with HLA-B27 positivity in the patients with AS.