3_Immunology-and-Inflammation-Alterations-in-the-Immune-Response.pdf

NCM 212 LECTURE: IMMUNOLOGY AND INFLAMMATION CONCEPT PRELIMS – LECTURE 3 MR. EDRIC JOY A. EBERO, RM, RN, MAN – NURSING & RT DEPARTMENT 1ST SEMESTER | A.Y. 2022 – 2023 IMMUNOLOGY AND INFLAMMATION: A. PR...

NCM 212 LECTURE: IMMUNOLOGY AND INFLAMMATION CONCEPT PRELIMS – LECTURE 3 MR. EDRIC JOY A. EBERO, RM, RN, MAN – NURSING & RT DEPARTMENT 1ST SEMESTER | A.Y. 2022 – 2023 IMMUNOLOGY AND INFLAMMATION: A. PRIMARY IMMUNE DEFICIENCY ALTERATIONS IN THE IMMUNE RESPONSES 10 Warning Signs of PID: OUTLINE 1. 8 or more ear infections within 1 year I. Immune Deficiency 2. 2 or more sinus infections within 1 year II. B Cell Deficiencies 3. 2 or more sinus infections within 1 year III. T Cell Immunodeficiency 4. 2 or more pneumonias in a year IV. Combined B and T cell Immunodeficiency 5. Poor growth V. Phagocytic Cell Disorders VI. Acquired Immunodeficiency Syndrome (AIDS) 6. Recurrent deep = skin/ organ abscesses VII. Autoimmune Disorder 7. Persistent thrush in mouth/skin in a year VIII. Lupus Erythematosus 8. Need for intravenous antibiotics to clear IX. Kawasaki Disease infection X. Rheumatic Disease 9. 2 or more deep-seated infections (meningitis, cellulitis or sepsis) 10. A history of primary immune deficiency IMMUNOLOGIC AND INFLAMMATORY DISORDERS Other Symptoms: Immune Deficiency Frequent or unusual infections o Abnormality in one or more branches of Prolonged diarrhea the immune system that renders a Poor childhood growth person susceptible to diseases Characteristics of Primary Immune Deficiency: normally prevented by an intact Rare disorders with genetic origins. immune system. Seen primarily in infants and young children. o May be caused by a defect or Symptoms usually develop early in life. deficiency in phagocytic cells, B Seldom survive to adulthood. lymphocytes, T lymphocytes, or the May involve one or more components of the complement system. immune system. Autoimmune Disorder o Formation of antibodies against self- II. B CELL DEFICIENCY cells. Types of Inherited B Cell Deficiencies: o Factors: Genetics 1st type: Environment Results from lack of differentiation of B-cell Sex precursors into mature B cells. o Autoantibodies – Antibodies produced o Precursor- predecessor or a cell or against one’s own cells. substance or cellular component from Allergy and Hypersensitivity which another substance, cell or o An exaggerated or inappropriate cellular component is formed immune response. Plasma cell is lacking – leads to complete lack o Divided into 5 categories based on the of antibody production. immunologic mechanism involved in 2nd type: the reaction. Results from a lack of differentiation of B cells Gammopathy into plasma cells. o Hypergammaglobuline Diminished antibody production called hypogammaglobulinemia, is a frequently I. IMMUNE DEFICIENCY occurring immunodeficiency. Abnormality in one or more branches of the immune system that renders a person ANTIBODY / B CELLS IMMUNODEFICIENCY susceptible to diseases normally prevented by an intact immune system. A. X — linked Agammaglobulinemia/ Bruton Type May be caused by a defect or deficiency in Agammaglobulinemia/ X-linked Infantile/ phagocytic cells, B lymphocytes, T Congenital Agammaglobulinemia lymphocytes, or the complement system. B. Hypogammaglobulinemia ‘Common Variable 2 Classification of Immunodeficiency: Immunodeficiency (CVID) A. Primary immune deficiency: Congenital or inherited C. Selective: IgA, IgM, IgG deficiency B. Secondary immune deficiency: Acquired CASTILLON, SUAREZ BSN 3D 18  A. X – LINKED AGAMMAGLOBULINEMIA Diagnostic Test: a.k.a Bruton Type Agammaglobulinemia/ X- Low, IgG: 200 mg/ di, linked Infantile/ Congenital White blood cell counts: are normal, Agammaglobulinemia IgA, IgM, IgD, and IgE: low or, absent. First immunodeficiency to be identified. B-lymphocytes: absent Caused by lack of cell differentiation to mature T-cell responses: Normal B cells Normal Values: Unable to produce antibodies Most common in males A.1. BRUTON’S DISEASE OR CONGENITAL AGAMMAGLOBULINEMIA Defect on maturation of B cells X-Linked inheritance — in about 6 mos. Of age of a child (Ig G is depleted) First reported in 1952, where a man named Burton inherited ID disease caused by mutation in the gene (X chromosome 21.3 to 22) coding for Bruton tyrosine kinase (BTK) {critical to the maturation of pre-B-cell to differentiating mature B cell.} noted a t- Figure 1.Normal Values of Ig lynphocyte elevated. Treatment: Clinical Features: There is no cure for X — linked spleen, lymph nodes, tonsils, adenoids, Agammaglobulinemia Peyer’s patches decrease in size or absent in individuals Goal: Maintain lg G at 500mg/dl IgG depleted serious enteroviral infections Gammaglobulin Replacement therapy: chronic pulmonary disease IV infusion –Ig dose of 300 mg/ kg skin disease every three weeks, or more monthly. inflammatory bowel disease (UC and Crohn’s Lifelong maintenance disease) Prognosis: “Without gammaglobulin treatment, these CNS complication patients may die from infections at an early age.” Prognosis: Good – IVgammaglobulin therapy is given C. SELECTIVE: IGA, IGM, IGG DEFICIENCY before the age of 5 y.o. lack of both serum and secretory IgA Fatal – If Chronic Pulmonary Disease (CPD) it is the total absence or severe deficiency of develops. IgA. Blood serum levels for IgA deficient persons B. HYPOGAMMAGLOBULINEMIA are usually found to be 7 mg/ di a term that encompasses a variety of defects Usually asymptomatic ranging from immunoglobulin A (IgA) deficiency, in which only the plasma cells that Diagnostic Tests: produce IgA are lacking, to the other extreme, Palliative: immunosuppressive therapy, in which there is severe panhypoglobulinemia Antibiotics (general lack of immunoglobulins in the blood). Ig A: 7 mg/dl or less Treatment: Clinical Features: No Cure growth retardation Ig A Replacement abnormalities in lymphoid tissues and organs Prognosis: skin and mucus membrane abnormalities Many persons with selective IgA deficiency live ear, nose, throat abnormalities their full life span without any problems. pulmonary/ CV/ neurologic abnormalities Idiopathic-unknown cause Males develop serious Pyogenic Infection-pus is produced. III. T CELL IMMUNODEFICIENCY Other Manifestations: A. DI GEORGE’S SYNDROME / THYMIC Common sites are: HYPOPLASIA Inner ear: Otitis media (>3y.o: up and back; congenital failure of the thymus gland