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REVIEW OF BASIC FACTS

Plasma Protein Albumin Largest and numerous plasma CHON Maintains osmotic pressure preventing edema Globulins Alpha Globulins = transport steroids, bilirubin and hormones Beta Globulins = copper and iron Gamma Globulins = a. Ab & Ig b. PT & fibrogen clotting factor Immunoglobulins Ig G = only antibody that pass placental circulation (Passive Immunity), Bloodborne / tissue infections, Most abundant Ig Ig A = bodily secretions (tears, saliva), passes breast milk for protection Secretory Ig Ig M = acute in inflammation Ig E = allergic reaction Ig D = chronic inflammation Formed Elements in Blood RBC (Erythrocytes) Normal life span = 80-120 days & killed in red pulp of SPLEEN WBC (Leukocytes) Granulocytes Neutrophils (acute inflammation) Basophils (parasite infections) Eosinophils (allergic reaction) Non Granulocytes Monocytes (macrophages)= chronic inflammation Lymphocytes B cell (Bone marrow) = for immunity T cell (Thymus ) = for immunity

NK cells = anti viral and tumor property

Immune System Disorders (HIGA) H = Hypersensitivity G = Gammopathies I = Immunodefiencies A = Autoimmunity

Hypersensitivity Exaggerated Responses to Specific Antigen Gammopathies Overproduced Immunoglobulins Immunodefiencies Deficiency of Phagocytic Cells, B And T Lymphocytes And Complement System Autoimmunity Production of antibodies against or attacks the tissues of your own body

IMMUNODEFIENCY DISORDER
Primary or PID (Congenital/Inherited) Improper development of immune cells / tissues Secondary or SID (Acquired) Interference with an already developed immune system

B & T lymphocytes Ataxia-Telangiectasia Severe Combined Immunodeficiency Disease (SCID) Wiskott-Aldrich Syndrome (WAS) Complement System Hereditary Angioedema Paroxysmal Nocturnal Hemoglobinuria (PNH) Hyperimmunoglobulinemia E Syndrome (HIE) or Job Syndrome

The 10 Warning Signs of PID 1. Eight or more new ear infections w/in a yr 2. Two or more serious sinus infections w/in a yr 3. Two or more months on antibiotics with little effect 4. Two or more PN w/in a yr 5. Failure of an infant to gain weight or grow normally 6. Recurrent, deep skin or organ abscesses 7. Persistent thrush in mouth or elsewhere on skin after age 8. Need for IV antibiotics to clear infections 9. Two or more deep seated infections (meningitis, osteomyelitis, cellulitis, sepsis) 10. A family history of PID Primary Immunodeficiency Disorders Phagocytic cells Hyperimmunoglobulinemia E Syndrome (HIE) or Job Syndrome B lymphocytes Sex-linked Agammaglobulinemia (Brutons Disease) Common Variable Immunodeficiency (CVID) or Hypogammaglobulinemia T lymphocytes Thymic Hypoplasia (DiGeorge Syndrome) Chronic Mucocutaneous Candidiasis

HIE Syndrome or Job Syndrome S/Sx Bacterial, fungal, & viral infections, deep-seated cold abscesses Tx Antibiotic Granulocyte-Macrophage ColonyStimulating Factor (GM-CSF) Granulocyte Colony-Stimulating Factor (G-CSF) Sex-linked Agammaglobulinemia (Brutons Disease) S/Sx Severe infections soon after birth Tx Passive pooled plasma or gammaglobulin

Common Variable Immunodeficiency (CVID) or Hypogammaglobulinemia S/Sx Bacterial infections, infection with Giardia lambia Autoimmune & neoplasmic d/o

Tx IV Ig Metronidazole (Flagyl) Quinacrine HCl (Atabrine)

Severe Combined Immunodeficiency Disease (SCID) S/Sx

Thymic Hypoplasia (DiGeorge Syndrome) Tx S/Sx Recurrent infections, Hypoparathyroidism, hypocalcemia, tetany, convulsions, Congenital Heart Dse, possible renal abnormalities, abnormal facies Tx Thymus graft Chronic Mucocutaneous Candidiasis S/Sx Candida albicans infection of mucous membrane, skin, & nails; Hypoparathyroidism, Addisons disease Tx Antifungal agents (Miconazole=topical, Clotrimazole, Ketoconazole = oral, Amphotericin B = IV) Ataxia-Telangiectasia S/Sx Ataxia with progressive neurologic deterioration, telangiectasia (vascular lesions), recurrent infections; malignancies Tx Antimicrobial therapy Fetal thymus transplant IV Ig

Overwhelming severe fatal infections soon after birth (also includes opportunistic infections) Antimicrobial therapy Bone Marrow transplantation IV Ig

Wiskott-Aldrich Syndrome (WAS) S/Sx Tx Antimicrobial therapy Splenectomy with continuous antibiotic prophylaxis IV Ig and Bone marrow transplantation

Thrombocytopenia resulting in bleeding, infections; malignancies

Hereditary Angioedema (HAE) S/Sx

Episodes of edema (Respiratory tract and bowel)

Tx Pooled plasma Androgen therapy

Paroxysmal Nocturnal Hemoglobinuria (PNH)) S/Sx

Lysis of RBC (erytrocytes) due to lack of decay-accelerating factor (DAF) on erythrocytes None

Tx

Secondary Immunodeficiency D/O (SID) More common Result of underlying disease processes/ treatment of disease AIDS = most common d/o Immunocompromised host

Causes: Malnutrition Chronic Stress Burns Uremia DM Autoimmune D/O Viruses Immunotoxic and chemicals exposure Drug and Alcohol Self-administration

Management: Assessment of patients immune status = ESSENTIAL Health teaching (Avoidance of contributing factors) Infection Control - Strict Aseptic Technique = ESSENTIAL (venipunctures, changes of dressing,bladder catheterization) Monitors lab values (wound drainage, culture and sensitivity, urine, blood, sputum, lesion, stool) Encouraging the patient and family active partners in management = KEY successful outcomes

Prepared by: Teacher Zussette O. Villar, RN,MAN