Immunoglobulin Therapy

Apr 15, 2019 by in PEDIATRICS Comments Off on Immunoglobulin Therapy

Key Points • Replacement immune globulin, administered by intravenous or subcutaneous route, is indicated as replacement therapy in patients with antibody immunodeficiency disorders. • Dosing intravenous immune globulin (IVIG) to…

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Congenital Immune Dysregulation Disorders

Apr 15, 2019 by in PEDIATRICS Comments Off on Congenital Immune Dysregulation Disorders

Key Points • Monogenic defects have been identified that interfere with self tolerance. • Human defects of self tolerance can affect both central and peripheral tolerance. • Linking specific genetic…

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Autoinflammatory Disorders

Apr 15, 2019 by in PEDIATRICS Comments Off on Autoinflammatory Disorders

Key Points • In the majority of the autoinflammatory diseases innate immune system dysregulation is the cause of the inflammation. • Most of these conditions have a monogenic cause. •…

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Rheumatic Diseases of Childhood: Therapeutic Principles

Apr 15, 2019 by in PEDIATRICS Comments Off on Rheumatic Diseases of Childhood: Therapeutic Principles

Key Points • Rheumatologic disorders are perpetuated by ongoing immune activation resulting from failure to clear triggering antigens combined with aberrant control mechanisms. • The resulting chronic inflammation leads to…

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Defects of Innate Immunity

Apr 15, 2019 by in PEDIATRICS Comments Off on Defects of Innate Immunity

Key Points • White blood cells (lymphoid or myeloid) can suffer from quantitative or functional congenital disorders. • The type of cells or mechanisms that are mainly affected will define…

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Inflammatory and Effector Cells/Cell Migration

Apr 15, 2019 by in PEDIATRICS Comments Off on Inflammatory and Effector Cells/Cell Migration

Key Points • A hallmark of allergic inflammation is the accumulation of a large number of leukocytes, including eosinophils, neutrophils, lymphocytes, basophils and macrophages, in the inflammatory tissues. • Leukocyte…

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Complement Deficiencies

Apr 15, 2019 by in PEDIATRICS Comments Off on Complement Deficiencies

Key Points • SLE and neisserial infections are the common complement deficiency phenotypes seen in Allergy Immunology practices. • CH50 and AH50 assays represent logical initial studies to diagnose many…

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T Cell Immunodeficiencies

Apr 15, 2019 by in PEDIATRICS Comments Off on T Cell Immunodeficiencies

Key Points • Defects of T cell development and/or function cause increased susceptibility to infections of bacterial, viral and fungal origin, and are often associated with autoimmune manifestations and malignancies….

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Antibody Deficiency

Apr 15, 2019 by in PEDIATRICS Comments Off on Antibody Deficiency

Key Points • A clinician must maintain an index of suspicion for immunodeficiency when confronted with patients with infections considered unusual with respect to frequency, severity, response to treatment, or…

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