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High Yield discussion for vaccination guidelines among patients with primary immunodeficiencies;
We discuss the following disorders of adaptive immune system resulting in primary immunodeficiency:
T-cell Disorders: DiGeroge, IL-12 receptor pathway disorders (Mendelian Susceptibility to Mycobacterial Infection), Chronic Mucocutaneous Candidiasis
Combined B & T cell disorders: Severe combined immunodeficiency (SCID), Autosomal- Dominant Hyper-IgE Syndrome, Wiskott-Aldrich, Ataxia-Telangiectasia, Hyper-IgM syndrome
These (HLH & ALPS) are the major syndromes of innate and adaptive immune dysregulation respectively resulting in primary immunodeficiency. For the discussion of complement system dysregulation refer to episode on innate immune deficiencies of innate immune system. Also compare HLH with MAS (macrophage activation syndrome) discussed in pharmacotherapy of Juvenile Rheumatoid Arthritis
Basic science foundational knowledge required for understanding the pathogenesis of different primary immunodeficiency syndromes as well as closely-related mechanisms interrupted by immunosuppressive agents;
Question-based review on Classification & Pathogenesis of Drug and Hypersensitivity Reactions
High-yield question-based discussion on Risk Factors, Pathogenesis, Epidemiology, Diagnostic Workup and Management options of osteoporosis (relevant for USMLE steps 1, 2, 3)
A question-based review of Paget's disease, Osteopetrosis and Avascular Necrosis relevant for USMLE step 1 & 2
Discussion of overlap syndrome named MCTD followed by primary versus secondary forms of Raynaud's phenomenon / disease are discussed
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