This document discusses immunodeficiency diseases. It defines immunodeficiency as a state where the immune system's ability to fight infections is compromised or absent. There are two types: primary immunodeficiency which is inherited and diagnosed in childhood, and secondary immunodeficiency which is acquired from diseases or environmental factors like HIV. Primary immunodeficiencies are classified into humoral deficiencies, cellular deficiencies, combined deficiencies, complement disorders, and phagocytosis disorders. Examples like X-linked agammaglobulinemia and common variable immunodeficiency are described. Nursing management focuses on infection assessment, patient teaching, and supportive care.

1. IMMUNODEFICIENCY DISEASES
GROUP 6A

3. GROUP MEMBERS
1. Alexandria Mungai
2. Keith Nyambeki
3. Lucy Muthoni
4. Elizabeth Makena Netia
5. Johnbosco Muema
6. Yvonne Mwita
7. Chris Karanja

4. DEFINITION
Immunodeficiency or immune
deficiency is the state in which
immune system’s ability to fight
infectious disease is compromised
or entirely absent.

5. TYPES
There are two types of immunodeficiency disorders:
1 Primary immunodeficiency PID: Inherited immune disorders resulting
from genetic mutations usually present at birth and diagnosed in
childhood.
2 Secondary immunodeficiency (SID): acquired immunodeficiency from a
disease or environmental factors such as HIV, malnutrition or medical
treatment e.g. chemotherapy.

6. CLASSIFICATION OF PRIMARY
IMMUNODEFICIENCY DISEASES
1. Humoral Immunodeficiencies
2. Cellular Immunodeficiencies
3. Combined immunodeficiencies
4. Disorders of complement
5. Disorders of phagocytosis

7. B CELL DEFICIENCY
• IgA deficiency
• IgG subclass deficiency
• Immunodeficiency with increased Igm
• Common variable immunodeficiency
• Transient hypogammaglobulinemia of infancy
• X linked agammaglobulinemia

8. X LINKED AGAMMAGLOBULINEMA
• In X-LA early maturation of B cells fails.
• Affect mails
• Few or no B cells in blood
• Very small lymph nodes and tonsils
• No Ig.
• Small amount of IgG in early age.
• Recurrent pyogenic infection.

9. IgA AND IgG SUBCLASS DEFICIENCY
• IgA deficiency is most common
• Patients tend to develop immune complex disease
• About 20% lack IgG2 and IgG4
• Susceptible to pyogenic infection
• Results from failure in terminal differentiation of B cells

10. IMMUNODEFICIENCY WITH INCREASED IgM
(HIgM)
• Results on patients with IgA and IgG deficiency
• Production of large amount of IgM >200 mg/dl of polyclonal IgM
• Susceptible to pyogenic infection.
• Formation of IgM to neutrophils, platelets and other blood components.
• Due to inability of B cells to isotype switching.

11. HYPOGLOBULINAEMIA OF INFANCY
Due to delay in IgG synthesis approximately up to 36 months.
In normal infants synthesis begins at 3 months.
Normal B lymphocytes
Probably lack help of T lymphocytes

12. DISORDERS OF T CELL
DiGeorge’s syndrome:
• Also known as Congenic thymic aplasia/hypoplasia.
• Associated with hypoparathythoidism, congenital heart disease, fish shaped mouth.
• Defects results from abnormal development of fetus during 6th-10th week of gestation when
parathyroid, thymus, lips, ears, and aortic arch are being formed.

13. T CELL DEFICIENCIES WITH VARIABLE DEGREES
OF B CELL DEFICIENCY
1.Ataxia-telangiectasia:
• Associated with a lack of coordination of movement (ataxia) and dilation of small blood vessels of
the facial area (telangiectasias).
• T-cells and their functions are reduced to various degrees.
• B cell numbers and IgM concentrations are normal to low.

14. • IgG is often reduced
• IgA is considerably reduced (in 70% of the cases).
• There is a high incidence of malignancy particularly leukemia in these patients.
• The defects arise from a breakage in chromosome 14 at the site of TCR and Ig heavy chain
genes.

15. WISKOTT-ALDRICH SYNDROME
• Associated with normal t cell numbers with reduced functions which get progressively worse.
• IgM concentrations are reduced but IgG level are normal
• Both IgA and IgE levels are elevated.
• Boys with this syndrome develop severe eczema.
• They respond poorly to polysaccharide antigens and are prone to pyogenic infection.

16. BARE LEUKOCYTE SYNDROME
• Due to defect in the MHC class II trans activator (CIITA) protein gene, which results in a lack of
class-II MHC molecule on APC.
• Patients have fewer CD4 cells and are infection prone.
• There are also individuals who have a defect in their transport associated protein (TAP) gene and
hence express the class-I MHC molecules and constantly are deficient in CD8+ T cells.

17. DEFECTS OF THE PHAGOCYTIC SYSTEM
• Defects of phagocytic cells ( numbers and/or functions) can lead to increased susceptibility
to a variety of infections.
Cyclic neutropenia:
• Its marked by low numbers of circulating neutrophil approximately every three weeks. The
neutropenia lasts about a week during which the patients are susceptible to infection. The
defect appears to be due to poor regulation of neutrophil production.

18. Chronic granulomatous disease (CGD):
CGD is characterized by marked lymphadenopathy, hepato-splenomegaly and chronic draining
lymph nodes.
In majority of patients with CGD, the deficiency is due to a defect in NADPH oxidase that
participate in phagocytic respiratory burst.

19. Leukocyte Adhesion Deficiency
• Leukocytes lack the complement receptor CR3 due to a defect in CD11 or CD18 peptides and consequently
they cannot respond to C3b opsonin.
• Alternatively there may be a defect in integrin molecules, LFA-1 or mac-1 arising from defective CD11a or
CD11b peptides, respectively.
• These molecules are involved in diapedesis and hence defective neutrophils cannot respond effectively to
chemotactic signals.

20. Chediak-Higashi syndrome
• This syndrome is marked by reduced (slower rate) intracellular killing and chemotactic movement
accompanied by inability of phagosome and lysosome fusion and proteinase deficiency.
• Respiratory burst is normal.
• Associated with NK cell defect, platelet and neurological disorders.

21. DISORDERS OF COMPLEMENT SYSTEM
• Complement abnormalities also lead to increased susceptibility to infections.
• There are genetic deficiencies of various components system, which lead to increased infections.
• The most serious among these is the C3 deficiency which may arise from low C3 synthesis or
deficiency in factor I or factor H.

22. SEVERE COMBINED IMMUNODEFICIENCY
• In about 50% of SCID patients the immunodeficiency is x-linked whereas in the other half the
deficiency is autosomal.
• They both characterized by an absence of T cell and B cell immunity and absence (or very low
numbers) of circulating T and B lymphocytes.
• Patients with SCID are susceptible to a variety of bacterial, viral, myotic and protozoan infections.
• The x-linked SCID is due to a defect in gamma-chain of IL-2 also shared by IL-4, -7, -11 and 15, all
involved in lymphocyte proliferation and/or differentiation.
• The autosomal SCIDs arise primarily from defects in adenosine deaminase (ADA) or purine
nucleoside phosphorylase (PNP) which results is accumulation of dATP or dGTP, respectively, and
cause toxicity to lymphoid stem cells.

23. SECONDARY IMMUNODEFICIENCY
Secondary immunodeficiency or acquired immunodeficiency, is the loss of immune function and
results from exposure to various agents (not a genetic or developmental problem).
The most common secondary immunodeficiency is Acquired Immunodeficiency Syndrome (AIDS),
which results from infection with the human immunodeficiency virus1 (HIV-1).

24. FOUR STAGES OF INFECTION
• Incubation period- the initial incubation period infection is asymptomatic and usually lasts between two and four
weeks.
• Acute infection- lasts an average of 28 days and can include symptoms such as:
1. Fever
2. Lymphadenopathy(swollen lymph nodes)
3. Pharyngitis(sore throat)
4. Rash
5. Myalgia(muscle pain)
6. Malaise
7. Mouth and esophageal sores
• Latency stage- shows few or no symptoms and last anywhere from two to twenty years and beyond.

25. • AIDS- shows as symptoms of various opportunistic infections
0.5% of HIV-1 infected individuals retain high levels of CD4 T-cells and a low or clinically undetectable viral load
without anti-retroviral treatment. These individuals are classified as HIV controllers or long-term non progressors
(LTNP).
The two main types of T-cells are the “helper ”T-cell and the cytotoxic T-cell. The T-helper population is further
divided into those which help B-cells (Th2) and those which help cytotoxic T-cells (Th1).
Therefore, in order for a B-cell to do its job requires the biochemical help of Th2 helper T-cells; and, for a cytotoxic T-
cell to be able to eliminate a damaged cell (say, a virally-infected cell), requires the biochemical help of a Th1 helper
T-cell.
The effect of HIV on the immune system is the result of a gradual (usually) elimination of the Th1 and Th2 helper T-
cell sub-populations.

27. IMMUNODEFICIENCY CAUSED BY DRUGS
Corticosteroids
• Cause changes in circulating
leukocytes.
• Depletion of CD4 cells.
• Monocytopenia.
• Decreased in circulating eosinophils
and basophils.
• Inhibition of T cell activation and B cell
maturation.
• Inhibit cytokine synthesis.

28. METHOTREXATE
• Structural analogue of folic acid.
• Blocks folic acid dependent synthetic pathways essential for DNA
synthesis.
• Prolonged use for treatment reduces immunoglobulin synthesis.
• CYCLOSPORIN
• Have severe effects on T cell signaling and functions.
• It binds to immunophilins which are believed to have a critical
role in signal transduction.
• Also inhibit IL2 dependent signal transduction.

29. OTHER CAUSES:
• Malnutrition
• Minerals
• Obesity
• Vitamins

30. NURSING MANAGEMENT FOR PATIENTS WITH
IMMUNODEFICIENCY DISEASES
• Nursing management includes assessment, patient teaching, and supportive care.
• Assessment of the patient for infection and for response to treatment is important if it is to be effective.
• Nursing care of patients with primary and secondary immunodeficiencies depends on the underlying cause of the immunodeficiency, the type of
immunodeficiency, and its severity.
• Because immuno-deficiencies result in a compromised immune system and high risk for infection, careful assessment of the patient’s immune status
is essential.
• The assessment focuses on history of past infec-tions, particularly the type and frequency of infection; signs and symptoms of any current skin,
respiratory, gastrointestinal, or genitourinary infection; and measures that prevent infection.
• The nurse monitors the patient for signs and symptoms of infection: fever; chills; cough with or without sputum; shortness of breath; difficulty
breathing; difficulty swallowing; white patches in the oral cavity; swollen lymph nodes; nausea; vomiting; persistent diarrhea; frequency, urgency, or
pain on urination; redness, swelling, or drainage from skin wounds; lesions on the face, lips, or perianal area; persistent vaginal discharge with or
without perianal itching; and persistent abdominal pain.

31. NURSING MANAGEMENT CONTINUED
• Because the inflammatory response may be blunted, the patient is monitored for subtle and unusual signs and changes in physical status.
• Vital signs and the development of pain, neurologic signs, cough, and skin lesions are monitored and reported.
• Pulse rate and respiratory rate should be counted for a full minute, as even subtle changes can signal deterioration in the patient’s clin-ical status
• Thorough auscultation and assessment of the breath sounds are also key in detecting changes in respiratory status.
• Any unusual response to treatment and any significant change in the patient’s clinical condition are promptly reported to the physician.
• The nurse also monitors laboratory values (ie, white blood cell count and differential cell count) for changes indicating infection.
• Culture and sensitivity reports from wound drainage, lesions, spu-tum, stool, urine, and blood are monitored to identify pathogenic organisms and
appropriate antimicrobial therapy.
• Changes in laboratory results and subtle changes in clinical status must be reported to the physician because the immunocompromised patient may
not develop typical signs and symptoms of infection.

32. NURSING MANAGEMENT CONTINUED
• Assessment also focuses on nutritional status; stress level and coping skills; use of alcohol, drugs, or tobacco; and general hy-giene, all of which may
affect immune function.
• Strategies the patient has used to reduce risk for infection are identified.
• Other aspects of nursing care are directed toward reducing the patient’s risk for infection, assisting with medical measures aimed at improving immune
status and treating infection, improving the nutritional status, and maintaining bowel and bladder function.
• These include careful hand hygiene, encouraging the patient to cough and perform deep-breathing exercises at regular inter-vals, and protecting the
integrity of the skin and mucous mem-branes.
• All health care personnel must use strict aseptic technique when performing invasive procedures, such as dressing changes, venipunctures, and bladder
catheterizations.
• Other aspects of nursing care include assisting the patient in managing stress and in adopting a lifestyle that enhances immune system function.

33. THANK YOU
THE END