Call Girls Hosur Just Call 9630942363 Top Class Call Girl Service Available
primary immuno deficiency.pptx
1. Common Terminology
òAgglutination: clumping effect occurring when an
antibody acts as a cross-link between two antigens.
òApoptosis: programmed cell death that results from
the digestion of DNA by endonucleases.
òComplement: series of enzymatic proteins in the serum
that, when activated, destroy bacteria and other cells.
2. òCytotoxic T cells: lymphocytes that lyse cells infected with
virus; also play a role in graft rejection.
òAtaxia: loss of muscle coordination.
òTelangiectasia: vascular lesions caused by dilated blood
vessels.
òEpitope: any component of an antigen molecule that
functions as an anti-genetic determinant by permitting the
attachment of certain antibodies.
11. General Functions of the Immune System
1. Protection from pathogens
• May include microorganisms
• Bacteria, viruses, Fungi and Protozoans
• May also include macroorganisms
• Parasites such as Hookworms and
Tapeworms.
12. 2. Clean up!
• Removal of dead and damaged cells and components.
3. Recognition and removal of abnormal cells
• Failure to do this can result in
• Cancers
• Autoimmune disorders
13. The human body’s defense mechanisms
The body has many defense mechanisms to
prevent infection, such as intact skin and mucous
membranes, and a functioning immune system
14. Three biological defense mechanisms that protect the body:
First line of defense (anatomic/biochemical barrier)
Skin and mucous membranes, cilia and stomach acid
Second line of defense (mechanical clearance).
White blood cells, lysozymes , t-cells and interferon
Third line of defense (immune response)
Long-lasting and sometimes permanent protection
15. The First Line of Defense~Skin and Mucous Membranes~
Oral mucous membranes have many
layers, making it difficult for
organisms to enter the body.
The skin has acidic (pH <7.0)
properties that render some
organisms unable to produce disease.
E.g. many bacteria prefer an alkaline
(pH >7.0)environment for
reproduction.
16. Cilia are hairlike structures lining
upper respiratory tract mucous
membranes that protect the lungs.
Cilia trap mucus, pus, dust, and
foreign particles to prevent them
from entering the lungs.
The First Line of Defense ~Cilia~
17. Gastric juices inside the stomach
are very acidic (pH 1.0 to 5.0).
This acidic environment destroys
most organisms that enter the
stomach.
The First Line of Defense ~Stomach Acid~
18. The Second Line of Defense~White Blood Cells~
WBCs, or leukocytes, participate in both the natural and the
acquired immune responses.
Granular leukocytes, or granulocytes (so called because of
granules in their cytoplasm), fight invasion by foreign bodies
or toxins by releasing cell mediators, such as histamine,
bradykinin, and prostaglandins, and engulfing the foreign
bodies or toxins.
19. There are five types of leukocytes:
• Neutrophils:are the first cells to arrive at the site where
inflammation occurs..
• Monocytes also function as phagocytic cells, engulfing,
ingesting, and destroying greater numbers and quantities of
foreign bodies or toxins than granulocytes do.
• Lymphocytes whose functions include antigen
recognition and antibody production.
20. • Basophils which respond to inflammation from
injury.
• Eosinophils which destroy parasites and respond in
allergic reactions.
21. Are bactericidal enzymes
present in white blood cells and
most body fluids, such as tears,
saliva, and sweat.
These enzymes dissolve the
walls of bacteria, destroying
them
White Blood Cells~Lysozymes
Where could invaders hide from
phagocytes?
22. The Second Line of Defense~Interferon~
If an invading organism is a virus, white blood cells
release interferon (a group of antiviral proteins).
Interferon – chemical that interferes with the ability to
viruses to attack other body cells.
Interferon aids in the destruction of infected cells and
inhibits production of the virus within infected cells.
Tumor cell growth may also be inhibited by interferon
23.
24. White Blood Cells ~T-Cells~
T-Cells, often called “natural
killer” cells, recognize infected
human cells and cancer cells
T-cells will attack these infected
cells, quickly kill them, and then
continue to search for more
cells to kill
25. The Third Line of Defense ~Antibodies~
Most infections never make it
past the first and second levels
of defense
Those that do trigger the
production and release of
antibodies
26. Antibodies
Proteins that latch onto, damage, clump, and slow
foreign particles.
Each antibody binds only to one specific binding site,
known as an antigen.
27. The Inflammatory Response
The inflammatory response occurs as a
result of any bodily injury.
This response can be caused by
pathogens, trauma, or other events
causing injury to tissues.
Injured body cells release chemicals
called histamines, which begin
inflammatory response
28. Vascular Response
The first step of the inflammatory process is local vasodilation,
which increases blood flow to the injured area. Increased
blood flow creates redness and heat at the injury.
Inflammatory Exudate
Increased permeability of the blood vessels allows plasma to
move out of the capillaries and into the tissues.
Swelling occurs resulting in pain from pressure on nearby
nerve endings.
29. Phagocytosis and Purulent Exudate
The final step of the inflammatory process is the
destruction of pathogenic organisms and their
toxins by leukocytes.
During this process, a purulent exudate (pus) may
form that contains protein, cellular debris, and dead
leukocyte.
30. Factors that affect immune system
Internal factors
• Modifiable
• Nutritional status
• Existence of underlying disease
• Nonmodifiable
• Age, gender, and inherited genes
32. Three Characteristics of the Immune System
Self regulation
• The immune system differentiates between normal and
abnormal constituents.
• Antigens (Ag)
• Nonself substances that stimulate an immune response
• Located on surfaces of living cells or environmental
substances.
33. Specificity
òThe immune response reacts to only one antigen
òDifferent immune response for each different
antigen
òAntigen-specific antibody production
34. Memory
Immune response develops long-lasting protection
Residual set of cells that are specific to an antigen
remain in the body
35. Immunity
Definition
It is a homeostatic condition in which the body maintains
protection against infection and tumor growth.
It is a series of delicately balanced complex, multi-cellular
and physiological mechanisms that allow an individual to
distinguish foreign material from ‘self ’ and to neutralize and
/or eliminate the foreign matter ‘non-self ’.
36. òThere are two general types of immunity:
òNatural (innate) immunity and
òAcquired (adaptive) immunity.
37. Natural (innate) immunity
GNatural immunity, which is non-specific, provides a
broad spectrum of defense against and resistance to
infection.
G It is considered the first line of host defense
following antigen exposure, because it protects the
host without “remembering” prior contact with an
infectious agent.
38. The cells involved in this response are monocytes,
macrophages, dendritic cells, natural killer (NK)
cells,basophils, eosinophils, and granulocytes.
Natural immune mechanisms can be divided into two
stages:
Immediate (occurrs within 4 hours) and
Delayed (occurring between 4 and 96 hours
after exposure)
39. Acquired (adaptive) immunity
GAcquired (adaptive) immunity-immunologic responses
acquired during life but not present at birth-usually develops
as a result of prior exposure to an antigen through
immunization (vaccination) or by contracting a disease.
GWeeks or months after exposure to the disease or vaccine,
the body produces an immune response that is sufficient to
defend against the disease upon re-exposure to it.
40. The two types of acquired immunity are known as
active and passive immunity.
In active acquired immunity, the immunologic
defenses are developed by the person’s own body.
This immunity generally lasts many years or even a
lifetime.
41. Passive acquired immunity is temporary immunity
transmitted from another source that has developed
immunity through previous disease or immunization.
For example, Gamma globulin may be administered to
those exposed to hepatitis.
42. Immunity resulting from the transfer of antibodies
from the mother to an infant in utero or through
breastfeeding is another example of passive
immunity.
Active and passive acquired immunity involve
humoral and cellular (cell-mediated) immunologic
responses.
43.
44. Cellular Components of Immune Response
Granulocytes
• Ingest and digest debris (remains) and foreign material
throughout the body
• Release chemicals that assist in the inflammatory process
Macrophage (mature monocytes)
• Role in inflammatory response
45. Cellular Components of Immune Response
•Lymphocytes
• Primary cells concerned with the development of immunity
• Have the ability of self-recognition, specificity, and memory
• Two types
• B lymphocytes (B cells) (humoral immune response)
• T lymphocytes (T cells) (cell-mediated immune response)
46. The humoral immune response
• Initiated when an antigen binds with the antibody
receptors on the surface of the mature B cell
• Triggers a sequence of events that results in
production of plasma cells that secrete antibodies
(immunoglobulin molecules).
47.
48. • Five classes of immunoglobins (MADGE is
acronym to aid memory).
IgM(pentamer)
First Ig class produced after initial exposure to
antigen; then its concentration in the blood
declines.
Promotes neutralization and agglutination of
antigens; very effective in complement activation.
49. IgG(monomer)
Most abundant Ig class in blood; also
present in tissue fluids.
Only Ig class that crosses placenta.
Promotes opsonization, neutralization,
and agglutination of antigens; less
effective in complement activation than
IgM.
50. IgA(dimer)
• Present in secretions such as tears,
saliva, mucus, and breast milk.
• Provides localized defense of mucous
membranes by agglutination and
neutralization of antigens.
• Presence in breast milk confers passive
immunity on nursing infant.
51. IgE(Monomer)
• Triggers release from mast cells and
basophils of histamine and other
chemicals that cause allergic
reactions.
52. IgD
• Present primarily on surface of naive B
cells that have not been exposed to
antigens.
• Acts as antigen receptor in antigen-
stimulated proliferation and
differentiation of B cells.
53. Cell-Mediated Immunity
• It does not involve the production of antibodies, but
it is effective against intracellular pathogens (such as
viruses), fungi, malignant cells, and grafts of foreign
tissue.
• The first step is the recognition of the foreign antigen
by helper T cells, assisted by macrophages.
54. • The activated T cells divide many times and become
specialized in one of several ways.
• Cytotoxic, or killer, T cells (CD8) are able to lyse cells
such as cancer cells or those infected by viruses or
other intracellular parasites.
• They also release chemicals that activate
phagocytes such as macrophages and neutrophils.
57. Assessment
An assessment of immune function begins with a
health history and physical examination.
The history should note the patient’s age along with
information about past and present conditions and
events that may provide clues to the status of the
patient’s immune system.
58. Assessment
• Areas to be addressed include nutritional status;
• Infections and immunizations;
• Allergies;
• Disorder and disease states, such as autoimmune disorders,
• Cancer, and chronic illnesses;
• Surgery;
• Medications; and
• Blood transfusions.
59. Assessment
• Physical assessment includes palpation of the lymph
nodes and examination of:
• The skin,
• Mucous membranes, and
• Respiratory,
• Gastrointestinal, Genitourinary,
• Cardiovascular, and
• Neurosensory systems.
60. Selected Tests for Evaluating Immunologic Status
• Leukocytes and Lymphocyte Tests
• White blood cell count and differential
• Bone marrow biopsy.
• Hypersensitivity Tests
• Scratch test
• Patch test
• Intradermal test, Radioallergosorbent test (RAST)
65. Regardless of the underlying cause, the cardinal
symptoms of immunodeficiency include:
• chronic or recurrent severe infections,
• infections caused by unusual organisms or organisms that
are normal body flora,
• poor response to treatment of infections, and
• chronic diarrhea.
66. Immunodeficiencies may be classified as either primary
or secondary and by the components of the immune
system that are affected.
Primary immunodeficiency diseases are genetic in origin
and are caused by intrinsic defects in the cells of the
immune system.
Secondary immunodeficiencies such as AIDS, caused by
infection with human immunodeficiency virus (HIV).
67. Primary Immunodeficiencies
• Primary immunodeficiencies, rare disorders with
genetic origins, are seen primarily in infants and young
children.
• To date, more than 95 immunodeficiencies of genetic
origin have been identified.
• Without treatment, infants and children with these
disorders seldom survive to adulthood.
72. • Ataxia-telangiectasia
• Immune component involved: B and T lymphocytes
• Major Symptoms: Ataxia with progressive neurologic
deterioration; telangiectasia (vascular lesions); recurrent
infections; malignancies
• Treatment: Antimicrobial therapy; fetal thymus transplant, IV
immunoglobulin.
73.
74.
75. • Severe combined immunodeficiency disease (SCID)
• Major symptoms: Overwhelming severe fatal infections
soon after birth (also includes opportunistic infections)
• Treatment: Antimicrobial therapy; IV immunoglobulin and
bone marrow transplantation
76.
77. • Wiskott-Aldrich syndrome
• Major symptoms: Thrombocytopenia, resulting in bleeding;
infections; malignancies.
• Treatment: Antimicrobial therapy; splenectomy with
continuous antibiotic prophylaxis; IV immunoglobulin and
bone marrow transplantation.
78.
79.
80.
81.
82. Selected Primary Immunodeficiency Disorders
• Hyperimmunoglobulinemia E (HIE) syndrome
• Immune component involved: Phagocytic cells
• Major symptoms: Bacterial, fungal, and viral infections; deep-
seated cold abscesses.
• Treatment: Antibiotic therapy and treatment for viral and
fungal infections
• Granulocyte-macrophage colony stimulating factor (GM-CSF);
granulocyte colony-stimulating factor (G-CSF)
