By Asghar Aghamohammadi M.D., Ph.D. (auth.), Asghar Aghamohammadi, Nima Rezaei (eds.)
Primary immunodeficiency ailments (PIDs) are a heterogeneous workforce of inherited issues characterised through diversified defects within the improvement and serve as of the immune approach. This ebook goals to extend the medical expertise and data of training clinicians concerning the prognosis and administration of PIDs. which will do so objective, approximately ninety instances drawn from actual existence are offered, besides nearly three hundred comparable questions. the chosen case reviews are the results of the useful cooperation of greater than forty scientists within the box of immunodeficiency. They concentration either at the offering gains of sufferers with PIDs and at the required extra research and administration. all the numbered instances is by means of the questions, their solutions, and extra dialogue. every one query specializes in a specific element of the PID into consideration, and the subjects coated comprise scientific prognosis, laboratory findings, molecular mechanisms, and therapy.
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Additional info for Clinical Cases in Primary Immunodeficiency Diseases: A Problem-Solving Approach
1 (continued) Normal Serum Ig Normal Associated features Inheritance Recurrent fever, AD serositis, rash, and ocular or joint inflammation, mutations in 55-kD TNF receptor leading to intracellular receptor retention or diminished soluble cytokine receptor available to bind TNF; PMNs, monocytes are affected, Early onset AR enterocolitis enteric fistulas; perianal abscesses; chronic folliculitis; mutation in IL-10 or IL-10 receptor leads to increase of TNFg and other proinflammatory cytokines; monocyte/ macrophage, activated T cells are affected Mutations in IL-10, IL10RA or IL10RB Genetic defect/presumed pathogenesis Mutations in TNFRSF1A 40 A.
Aghamohammadi et al. 1 (continued) Normal Normal Circulating T cell Normal Normal Normal Circulating B cell Normal Normal Normal Serum Ig Normal SLE-like syndrome, rheumatoid disease, infections, absent CH50 hemolytic activity, defective MAC faulty dissolution of immune complexes, faulty clearance of apoptotic cells AR Associated features Inheritance Chronic recurrent AR multifocal osteomyelitis, transfusiondependent anemia, cutaneous inflammatory disorders; neutrophils and bone marrow cells are affected PMNs and monocytes AR are affected, mutations in the IL1 receptor antagonist allows unopposed action of Interleukin 1, neonatal onset of sterile multifocal osteomyelitis, periostitis, and pustulosis Mutations in C1QA, C1QB, C1QC and loss of early complement activation Mutations in IL1RN Genetic defect/presumed pathogenesis Mutations in LPIN2 42 A.
Aghamohammadi et al. 1 (continued) Low IgG and IgM Low IgG, normal or elevated IgM and IgA Low IgG and IgA and/or IgM Low IgG, low or normal IgA and IgM Low IgG and IgA and/or IgM Low IgG and IgA and/or IgM Serum Ig Low IgG and IgA and/or IgM Variable clinical expression Variable clinical expression May have glomerulonephritis May have glomerulonephritis AR AD or AR or complex AR AR AR Associated features Inheritance Clinical phenotypes Variable vary: most have recurrent infections, some have polyclonal lymphoproliferation, autoimmune cytopenias, and/or granulomatous disease AR Mutations in TNFRSF13C (BAFF-R) Mutations in TNFRSF13B (TACI) Mutations in CD19; transmembrane protein that amplifies signal through BCR Mutations in CD81; transmembrane protein that amplifies signal through BCR Mutations in CD20 Mutations in ICOS Genetic defect/presumed pathogenesis Unknown 18 A.