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Infectious Disease
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
Susanna J. Dunachie, Hanif Esmail, Ruth Corrigan, Maria Dudareva
The killed rabies vaccine is effective and safe. It can be given before exposure to those at particular risk or as PEP after a possibly infected bite. Additionally, immunoglobulin may also be given following high-risk exposure. Present regimens prevent the onset of rabies in the great majority of cases. However, it is essential that prophylaxis is started as soon as possible after any skin abrasion caused by any mammal in an endemic area.
Transforming Growth Factor-β: A Cytokine Paradigm
Published in Thomas F. Kresina, Immune Modulating Agents, 2020
Michelle R. Frazier-Jessen, Nancy McCartney-Francis, Sharon M. Wahl
While tolerance can be induced by several mechanisms, early studies found that by feeding a protein and then subsequently challenging with that protein, a state of systemic hyporesponsiveness to the protein is created [46]. In an attempt to provide a more focused, and ideally a less toxic, treatment than is presently offered by current immunosuppressive drugs, tolerance induction via oral ingestion has been extensively exploited in the last decade. While the actual mechanism(s) behind oral tolerance induction is not completely understood, TGF-β is considered to play a key role [46]. After ingestion, antigen is taken up and delivered to the gut-associated lymphoid tissue (GALT), which includes Peyer’s patches and intraepithelial lymphocytes, for antigen processing. Antigenic stimulation leads to a change in the cytokine secretion profile by GALT lymphocyte populations. Production of Th1 cytokines, such as IL-2 and IFN-γ, is down-regulated, whereas Th2 cytokines, such as IL-4 and IL-10 as well as TGF-β, are up-regulated (Figure 1) [47]. Release of TGF-β1 by T cells is dependent upon antigen-specific triggering by the oral toleragen. As previously mentioned, local secretion of TGF-β1 within the GALT aids in IgA class switching by resident B cells. Immunoglobulin A is essential for the clearance of orally ingested antigens (food), preventing the development of deleterious inflammatory responses within the gut.
The Small IntestineSecretions, Digestion and Motility
Published in Peter Kam, Ian Power, Michael J. Cousins, Philip J. Siddal, Principles of Physiology for the Anaesthetist, 2020
Peter Kam, Ian Power, Michael J. Cousins, Philip J. Siddal
The small gut is an immunologically active organ. T cells are present in the peripheral zone of Peyer's patches and B cells in the germinal centres of Peyer's patches. Large antigens may penetrate the intestinal lining and promote the production of immunoglobulins. The plasma cells in the lamina propria of the small intestine contain immunoglobulins IgA, IgM and IgG. IgA is an important secretory immunoglobulin that has a protective function.
How do nuclear factor kappa B (NF-κB)1 and NF-κB2 defects lead to the incidence of clinical and immunological manifestations of inborn errors of immunity?
Published in Expert Review of Clinical Immunology, 2023
Nazanin Fathi, Hanieh Mojtahedi, Marzieh Nasiri, Hassan Abolhassani, Mahsa Yousefpour Marzbali, Marzie Esmaeili, Fereshte Salami, Furozan Biglari, Nima Rezaei
The severity of the effects of mutations in these components depends on the variety of inheritance patterns and the significance of their expression in the function and development of various cells. Given disease heterogeneity, it is impossible for therapeutic implications to be uniform and must be individualized based on the patient’s particular presentation. Immunoglobulin replacement therapy for individuals with antibody deficiency, immunosuppression, steroids, and rituximab for patients with autoimmunity, and antibiotic, and antiviral medication is considered in the case depending on the clinical condition. However, the advantages must be considered against the immunosuppression’s risk of infection. Although hematopoietic stem cell transplantation could be a therapy option for NF-κB-deficient patients with a CID phenotype, to the best of our knowledge, there has been no relevant experience. This might be a consequence of NF-κB2 expression not simply being present in hematopoietic cells.
Autoimmune disorders associated with common variable immunodeficiency: prediction, diagnosis, and treatment
Published in Expert Review of Clinical Immunology, 2022
Niloufar Yazdanpanah, Nima Rezaei
The fundamental of the treatment of pulmonary complications is administrating sufficient immunoglobulin replacement therapy. High immunoglobulin trough levels of 800–100 mg/dl have been reported to decrease infections in the lungs [225]. In addition, prophylactic antibiotics (e.g. azithromycin) are suggested to prevent respiratory exacerbation of the disease and restrain pulmonary complications following chronic infections [226]. On the other side, to prevent permanent sequelae and progression to ILD, cyclophosphamide, cyclosporine A, or azathioprine in combination with rituximab could be beneficial according to the observations concerning the presence of B cells, T cells, and macrophages in lung biopsies of CVID patients [227,228]. Azathioprine and rituximab have shown promising results in CVID patients complicated with GLIDL [228,229]. Some patients with CVID-associated lung granulomas reported resolution in their symptoms with infliximab [230].
Long Term Follow-Up of the Patients with Severe Combined Immunodeficiency After Hematopoietic Stem Cell Transplantation: A Single-Center Study
Published in Immunological Investigations, 2022
Duygu Demirtas, Deniz Cagdas, Tuba Turul Ozgur, Baris Kuskonmaz, Duygu Uckan Cetinkaya, Ozden Sanal, Ilhan Tezcan
CD19 counts were normal in 45.4% (20/44) of the patients at the last follow-up [8.7 years (2–21 years)]. B+ SCID (14/18, 77.8%) patients had better CD19 counts than B- SCID (6/26, 23.1%) after HSCT (p < .001). Although CD19 counts were normal in 45.4% (n = 20), monthly IVIG therapy could be ceased in 72.7% of the patients (n = 32). Additionally, IVIG therapy was stopped in 79.4% and 40% of the patients who had HLA identical and haploidentical HSCT, respectively. Immunoglobulin A levels were normal in 59.1% (26/44) of the patients at the last follow-up. When only patients transplanted from HLA identical donors were evaluated, IgA levels were normal in 70.6% (24/34). Although B+ SCID patients had better CD19 counts than B- SCID; IVIG cessation ratios and Ig levels did not vary between B- and B+ immunophenotypes.