Immunodeficiency may be caused by a defect of deficiency of the following (4)
phagocytic cells (lymphocytes, monocytes)
b lymphocytes (humoral i.r)
t lymphocytes (cellular i.r)
complement system (from the liver)
What are the cardinal symptoms of immunodeficiency? (4)
✔️chronic or recurrent and severe infections
✔️infections caused by unusual organisms or by organisms that are normal body flora
✔️poor response to standard treatment for infections
✔️chronic diarrhea
2 types of immunodeficiency
primary
secondary
This type of immunodeficiency is from genetic and may affect phagocytic function,b cell and/or t cells or the compliment system.
primary / secondary
primary
This type of immunodeficiency is acquired, an example would be HIV/AIDS.
primary / secondary
secondary
Secondary immunodeficiency may be related to (4)
underlying disorders
diseases
toxic substances
medication
______ represent inborn errors of the immune system
primary / secondary
priamary
Primary immunodeficies may predispose poeple to (3).
📌frequent, sever infections
📌autoimmunity
📌cancer
What are the immunodeficiencies that are known to increase incidence of malignancy or cancer. (5)
(CVID) Common variable immunodeficiency
Immunoglobulin A deficiency
DNA repair disorders
Severe combined immunodeficiency disease
Wiskott- Aldrich syndrome
What is the function of the immunoglobulin A?
Protect mucosal surfaces in the body from infection.
IgA is the most abundant antibody in mucous membranes, which line the respiratory tract, gastrointestinal tract, and other body surfaces that are exposed to the external environment.
In Wiskott-Aldrich syndrome there is an absence of _____ and _____ that may also lead to cancer.
thrombocytopenia (platelets)
T and B cells
Primary immunodeficiencies include disorders of the (5)
1.Humoral immunity (affecting B-cell differentuation or antibody production)
2.T-cell defects
3.Combine B and T-cell defects
4.Phagocytic disorders
5.Complement deficiencies
The phagocytic dysfunction affects the ____ immune sytem.
natural / acquired
natural (non-specific) immune system
Phagocytic dysfunction usually affects the
A. neutrophil
Non-specific immune responce, involves ______ type of cells, quick response.
granulocyte / agranulocyte
granulocyte
Specific immune responce, involves ______ type of cells, quick response.
granulocyte / agranulocyte
agranulocyte (invoving the B and T cells)
Give atleast (5) clinical manifestations of phagocytic dysfunction.
👉🏻increase incidence of bacterial and fungal infections caused by organisms that are normally nonpathogenic (candida organisms, viral (herpes simplex or herpes zoster)
👉🏻recurrent cutaneous abscesses (puss)
👉🏻chronic eczema
👉🏻bronchitis
👉🏻pneumonia
👉🏻chronic otitis media
👉🏻sinusitis
Another disease caused by the dysfunction of the phagocytes. Where the white blood cells cannot initiate an inflammatory response to infectious organisms.
Hyperimmunoglobulinemia E syndrome (formerly known as Job Syndrome)
Although patients with phagocytic cell disorder may be asymptomatic they may present with ______.
neutropenia
Symptoms of neutropneia are the following:
deep and painful mouth ulcers
gingivitis
stomatitis
cellulitis
These signs and manifestations suggest a primary immunodeficiency is present with you or your children. Give at least 5.
Within a year
☛ 8+ new ear infections
☛ 2+ sessions of sinus infections
☛ 2+ months on antibiotic with little effect
☛ 2+ pneumonia
☛ Failure of an infant to gain weight or grow normally
☛ Recurrent deep skin or organ abscesses
☛ Persistent thrush I mouth or elsewhere on skin after age 1
☛ Need for Iv antibiotics to clean infections
☛ 2+ deep-seated infections (meningitis, osteomyelitis, cellulitis or spesis)
☛ Family history of primary immune deficiency
Primary immune deficiency can be serious and fatal.
True or False
False
Though primary immune deficiency disease can be serious, they are rarely fatal and can generally be controlled.
Diagnosis of primary immune deficiency is based on (3)
history
signs and symptoms
laboratory analysis
This laboratory analysis to diagnose phagocytic dysfunction- primary immunodeficiency (neutropenia) indicates cyclotidal activity of the phagocytic cells.
nitroblue tetrazolium reductase test
Treatment for a phagocytic dysfunction (4)
granulocyte transfusion
granulocyte macrophase colony-stimulating factor
granulocyte colony -stimulating factor
hematopoetic stem cell transplantation
Review. Supply the missing answers
The bone marrow produces this type of agranulocyte cell _____ that turns into a B-cells.
B cells mature in the ______. Until they further differentiate into memory B cells or _______ which will be the body's ______.
This process is termed as _______ immune response.
(1) lymphocyte
(2) bone marrow
(3) plasma b cells
(4) antibodies
(5) humoral immune response
(2) B-cell deficiencies
Burton's disease (x-linked agammaglobulinemia)
Hypogammaglobulinemia (CVID)
It is the lack of differentiation of the B cell into plasma cells.
Hypogammaglobulinemia
X linked agganaglobulinemia usually becomes symptomatic after _____
the natural loss of maternally transmitted immunoglobulins
What immunoglobulin is transmitted transplacentally from mother to fetus?
A. IgG
CVID stands for
common variable immunodeficiency
patients with CVID are at increase risk for having these diseases (3)
autoimmunse disease
granulomatous disease
malignancy
Patients with CVID are susceptible to infections with ______ bacteria.
encapsulated
Examples of encapsulated bacteria are (3)
Haemophilus influenza
streptococcus pneumonia
staphylococcus aures
Defects in T cells lead to ______ infections.
opportunistic (from the body's normal flora)
Examples of T-cell deficiencies:
Digeorge syndrome or thymic hypoplasia
What is a thymic hypoplasia?
rare, complex, multisystem genetic abnormality, which affects multiple organ systems
T-cell deficiency occurs when...
the thymus gland fails to develop normally during thrombogenesis
DiGeorge syndrome manifests in the neonatal period as _______.
cardiac anomaly
Conditions of infants with DiGeorge syndrome.
1. They have hypoparathyroidism with resultant hypocalcemia. (When there isn’t enough PTH, the body cannot effectively regulate calcium levels, leading to hypocalcemia (low blood calcium levels).)
2. These infants are susceptible to yeast, fungal, protozoan, and viral infections and are particularly susceptible to childhood diseases.
Infection with ______ is almost universal in patients with severe deficiencies in the t-cell-mediated immunity.
candida albicans
What are the Medical Management for T-cell deficiencies?
Patients with T-cell deficiency should receive prophylaxis for
1. Patients with T-cell deficiency should receive prophylaxis for PCP (pneumocystis pneumonia)
2. Hypocalcemia is controlled by oral calcium supplementation
with administration of vitamin D / parathyroid hormone.
3. Transplantation of fetal thymus, postnatal thymus, or human leukocyte antigen (HLA).
Diseases with combined b and t-cell immunodeficiencies
ataxia-telengiectasia
severe combined immunodeficiency disease
______ has been used for the permanent reconstitution of T-cell immunity.
Bone marrow (HLA)
Vascular lesions caused by dilated blood vessels refer to
telengiectasia
_____ means loss of muscle coordination
ataxia
An autosomal recessive neurodegenerative disorder characterized by cerebellar loss of muscle coordination and vascular lesions caused by dilated blood vessels and immune deficiency.
ataxia-telangiectasia
A disorder in which both B cells and T cells are missing.
severe combined immunodeficiency disease (SCID)
With SCID there is marked susceptibility to
serious fungal bacterial and viral infections
The onset of ataxia and telengiectasia occurs when?
in the first 4 years of life
As patients approach the second decade of life in patients with combine b and t-cell deficiencies they may experience these clinical manifestations. (4)
chronic lung disease
cognitive impairment
neurologic symptoms
physical disability becomes severe
The onset of SCID symtoms occurs within the _____ of life.
B. 3 months
Symptoms of SCID in combined b and t cell deficiencies are (5)
Respiratory infections
Pneumonia (often secondary to PCP infection)
Thrush
Diarrhea
Failure to thrive
In Severe combine immunodeficiency many of these infections are resistant to treatment.
True or False
True
What are the medical management of Ataxia-telangiectasia:
1.Early management of infections with antimicrobial therapy
2. management of chronic lung disease with postural drainage (needs doctor order) and physical therapy
3. management of other presenting symptom
Management for SCID includes
Stem cell and bone marrow transplantation through HSCT —the definitive therapy.
Nursing management (SCID) during bone marrow transplantation. (3)
1. Appropriate infection control precautions.
2. Thorough hand hygiene is essential.
3. Continual monitoring of the patient's condition is critical, so early signs of impending infection may be detected and treated before they seriously compromise the patient's status.
4.Standard precautions
In deficiencies of the complement system, the usual affected part is the
C2 and C3
This could increase the susceptibility to infectious diseases and immune-mediated disorders.
Results from the deficiency of C1 -esterase inhibitor — oppose the release of inflammatory mediators. (histamine, bradykinin)
angioneurotic edema
Diseases in Deficiencies of complement system (2)
Deficiency of C2 and C3
Angioneurotic edema
Deficiencies of complementary systems are usually manifested by
attacks of edema formation in the subcutaneous tissue, gastrointestinal tract, and upper airways.
Is an acquired clonal stem cell disorder resulting from a somatic mutation in the hematopoietic stem cells. (PNH is a condition where red blood cells break down too soon, often causing dark urine and other symptoms)
paroxysmal nocturnal hemoglobinuria
Symptoms of paroxysmal nocturnal hemoglobinuria include:(5)
1. Hemoglobinuria - hemoglobin protein, not the intact red blood cells, appears in the urine causing a dark urine. *which increases during sleep as well as intravascular hemolysis*
2. cytopenia
3. infections
4. bone marrow hyperplasia (a part of the body starts to gro more than normal)
5. High incidence of life-threatening venous thrombosis, which occurs most commonly in abdominal and cerebral veins.
Occurs as a result of underlying disease processes or the treatment of these disorders. Acquired.
Secondary immunodeficiencies
Give at least (5) common causes of secondary immunodeficiencies.
chronic stress
burns
uremia (High level of waste products in the blood because the kidneys aren’t working properly)
diabetes mellitus
certain autoimmune disorders
certain viruses
exposure to immunotoxic medications and chemicals
self administration of recreational drugs and alcohol
What are the nursing management for patients with immunodeficiencies?
1. Assessment of patient for infection and timely initiation of treatment are essential.
2. For patients who develop oral manifestations, provide education about promoting good dental hygiene to diminish oral discomfort and complications — as this may result in inadequate nutritional intake.
3. Because the inflammatory response may be blunted, the patient is observed for subtle and unusual signs and changes in physical status.
4. Pulse rate and respiratory rate should be counted for a full minute because subtle changes can signal deterioration in the patient's clinical status.
What to assess during infection. Give at least (5)
🦠fever with or without chills
🦠cough with or without sputum
🦠shortness of breath
🦠 difficulty breathing
🦠difficulty swallowing
🦠white patches in the oral cavity
🦠swollen lymph nodes
🦠nausea with or without vomiting
🦠persistent diarrhea
🦠frquency, urgency or pain on urination
🦠change in the character of the urine
🦠lesions on the face, lips, or perianal area
🦠redness, swelling or drainage from skin lesions
🦠 persistent vaginal discharge with or without perianal itching