5 research outputs found

    Study of nano-particle deposition in tubular pipes under turbulent condition

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    This paper aims to study the deposition and dispersion of nano-particles in turbulent tubular pipe flows. In this work, the Eulerian-Lagrangian particle tracking method is used to simulate the transport of nano-particles in turbulent flows under the conditions of one-way coupling. In order to simulate the turbulent flow in a pipe, the Reynolds stress transport model (RSTM) is used with the help of CFD method. RSTM is one of the most accurate Reynolds-averaged Navier-Stokes methods. In addition, near wall region, Two-layer zonal model is employed in the CFD model to simulate the viscous sublayer separated from the fully-turbulent layer in the pipe. The results show that RSTM with Two-layer zonal model can well predict the turbulent flow and particle deposition velocity in tubular pipes. The output of this study can provide a guideline for evaluating the nano-particle transport and deposition in turbulent pipe flows

    Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort

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    Background: Predominantly antibody deficiencies (PADs) are the most common primary immunodeficiencies, characterized by hypogammaglobulinemia and inability to generate effective antibody responses. Objective: We intended to report most common monogenic PADs and to investigate how patients with PAD who were primarily diagnosed as suffering from agammaglobulinemia, hyper-IgM (HIgM) syndrome, and common variable immunodeficiency (CVID) have different clinical and immunological findings. Methods: Stepwise next-generation sequencing and Sanger sequencing were performed for confirmation of the mutations in the patients clinically diagnosed as suffering from agammaglobulinemia, HIgM syndrome, and CVID. Results: Among 550 registered patients, the predominant genetic defects associated with agammaglobulinemia (48 Bruton's tyrosine kinase [BTK] and 6 μ heavy chain deficiencies), HIgM syndrome (21 CD40 ligand and 7 activation-induced cytidine deaminase deficiencies), and CVID (17 lipopolysaccharides-responsive beige-like anchor deficiency and 12 atypical Immunodeficiency, Centromeric instability, and Facial dysmorphism syndromes) were identified. Clinical disease severity was significantly higher in patients with μ heavy chain and CD40 ligand mutations compared with patients with BTK (P = .003) and activation-induced cytidine deaminase (P = .009) mutations. Paralysis following live polio vaccination was considerably higher in patients with μ heavy chain deficiency compared with BTK deficiency (P < .001). We found a genotype-phenotype correlation among patients with BTK mutations regarding clinical manifestation of meningitis and chronic diarrhea. Surprisingly, we noticed that first presentations in most patients with Immunodeficiency, Centromeric instability, and Facial dysmorphism were respiratory complications (P = .008), whereas first presentations in patients with lipopolysaccharides-responsive beige-like anchor deficiency were nonrespiratory complications (P = .008). Conclusions: This study highlights similarities and differences in the clinical and genetic spectrum of the most common PAD-associated gene defects. This comprehensive comparison will facilitate clinical decision making, and improve prognosis and targeted treatment

    Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort

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    Predominantly antibody deficiencies (PADs) are the most common primary immunodeficiencies, characterized by hypogammaglobulinemia and inability to generate effective antibody responses

    Fourth Update on the Iranian National Registry of Primary Immunodeficiencies: Integration of Molecular Diagnosis

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