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- Asgardoon, MH, et al.
(författare)
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Monogenic Primary Immunodeficiency Disorder Associated with Common Variable Immunodeficiency and Autoimmunity
- 2020
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Ingår i: International archives of allergy and immunology. - : S. Karger AG. - 1423-0097 .- 1018-2438. ; 181:9, s. 706-714
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Tidskriftsartikel (refereegranskat)abstract
- <b><i>Background:</i></b> Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency disorder mainly characterized by recurrent bacterial infections besides other immunological defects including loss of or dysfunction of B cells and decreased immunoglobulin levels. In this study, our aim is to evaluate clinical, immunological, and molecular data of patients with a primary clinical diagnosis of CVID and autoimmune phenotype with a confirmed genetic diagnosis. <b><i>Methods:</i></b> Among 297 patients with CVID, who were registered in the Iranian Primary Immunodeficiency Registry at Children’s Medical Center Hospital in Iran, 83 patients have been genetically examined and 27 patients with autoimmunity and confirmed genetic mutations were selected for analysis. Whole-exome sequencing and confirmatory Sanger sequencing methods were used for the study population. A questionnaire was retrospectively filled for all patients to evaluate demographic, laboratory, clinical, and genetic data. <b><i>Results:</i></b> In the 27 studied patients, 11 different genetic defects were identified, and the most common mutated gene was <i>LRBA</i>, reported in 17 (63.0%) patients. Two patients (7.7%) showed autoimmune complications as the first presentation of immunodeficiency. Eleven patients (40.7%) developed one type of autoimmunity, and 16 patients (59.3%) progressed to poly-autoimmunity. Most of the patients with mono-autoimmunity (<i>n</i> = 9, 90.0%) primarily developed infectious complications, while in patients with poly-autoimmunity, the most common first presentation was enteropathy (<i>n</i> = 6, 37.6%). In 13 patients (61.9%), the diagnosis of autoimmune disorders preceded the diagnosis of primary immunodeficiency. The most frequent autoimmune manifestations were hematologic (40.7%), gastrointestinal (48.1%), rheumatologic (25.9%), and dermatologic (22.2%) disorders. Patients with poly-autoimmunity had lower regulatory T cells than patients with mono-autoimmunity. <b><i>Conclusion:</i></b> In our cohort, the diagnosis of autoimmune disorders preceded the diagnosis of primary immunodeficiency in most patients. This association highlights the fact that patients referring with autoimmune manifestations should be evaluated for humoral immunity.
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| 3. |
- Choopani, S., et al.
(författare)
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Ferromagnetic resonance and magnetic anisotropy in biocompatible Y3Fe5O12@Na0.5K0.5NbO3 core-shell nanofibers
- 2019
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Ingår i: Ceramics International. - : Elsevier Ltd. - 0272-8842 .- 1873-3956. ; 46:2, s. 2072-2078
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Tidskriftsartikel (refereegranskat)abstract
- Y3Fe5O12@Na0.5K0.5NbO3 (YIG@NKN) core-shell nanofibers were synthesized by the coaxial electrospinning technique. For comparison, samples of YIG and NKN nanofibers were prepared. Scanning Electron Microscopy (SEM) and 3D laser-scanning confocal microscopy (TDLM) of YIG@NKN nanofibers revealed long uniform size distributed fibers with the average diameter of 100–150 nm. X-Ray diffraction (XRD) examination shows the existence of the distinct peaks of orthorhombic NKN and cubic YIG. Magnetic force microscopy (MFM) of individual YIG@NKN nanofiber demonstrates a magnetic core that is extended in one half of the diameter of the fiber. These nanofibers show obvious Ferromagnetic resonance (FMR) with resonance near 2 KOe similar to YIG fibers but in such a way that it starts to increase linearly with applying magnetic field from zero up to near resonance field. Also they show a soft magnetic behavior with saturation magnetization of 10 emu/gr. Furthermore, we propose a model to explain line shape of randomly oriented fibers and extract all the magnetic anisotropy parameters from FMR data. The results rely the shape anisotropy as dominant effect, however the dipolar field among fibers should be considered. The highest degree of asymmetry observed in the case of core-shell fibers in hard direction that it can be originated from magneto electric effects. By taking into account the observed FMR, the ability of adequate control of microwave absorption by applying magnetic field and biocompatibility, the synthesized core-shell nanofibers are the most promising candidate for clinical application such as microwave cancer thermotherapy and adjustable microwave absorbers.
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