NUR 205 Ch 51 Immunodeficiency

Immunodeficiency disorders are caused by defects or deficiencies in?

a. the complement system

b. B and T lyjmphocytes

c. phagocytic cells

d. all of the above.

d

The cardinal symptoms of immunodeficiency are?

a. Chronic diarrhea

b. chronic or recurrent severe infections.

c. poor response to treatment of infections

d. inclusive of all of the above.

d

The nitrobule tetrazolium reductase (NTR) test is used to diagnose immunodeficiency disorders related to?

a. complement

b. B lymphocytes

c. T lymphocytes

d. phagocytic cells

d

More than 50% of individuals with which disease develop pernicious anemia?

a. Bruton disease

b. Common variable immunodeficiency (CVID)

c. DiGeorge syndrome

d. Nezelaf syndrome

b

The most frequent presenting sign in patients with DiGeorge syndrome is?

a. chronic diarrhea

b. Hypocalcemia

c. neurtropenia

d. pernicious anemia

b

The primary cause of death for individuals with ataxia telangiectasia is?

a. acute renal failure

b. chronic lung disease

c. neurologic dysfunction

d. overwhelming infection.

d

The most common secondary immunodeficiency disorder is?

a. AIDS

b. DAF

c. CVID

d. SCID

a

The recommended dose of intravenous gramma globulin for a 60kg man given monthly is?

a. 15g

b. 30g

c. 45g

d. 60g

b

When gamma globulin is infused intravenously the rate should not exceed?

a. 1.5mL/min

b. 3 mL/min

c. 6 mL/min

d. 10 mL/min

b

The nurse knows to stop an infusion of gamma globulin if the patient experiences?

a. flank pain

b. shaking chills

c. tightness in the chest

d. all of the above.

d

Primary immunodeficiencies predispose people to three conditions what are they?

1. Severe infections
2. autoimmunity
3. cancer

List five disorders of common, primary immunodeficiencies.

1. Humoral immunity
2. T cell defects
3. combined t and b cell defects
4. phagocytic disorders
5. complement disorders

The two types of inherited B cell deficiencies result from lack of differentiation of B cells into?

mature B cells and Plasma cells

More than 50% of patients with CVID develop which disorder?

Pernicious anemia

DiGeorge syndrome is an example of which immunodeficiency?

Primary T cell

The most prevalent cause of immunodeficiency worldwide is?

severe malnutrition

Name this disorder?

There is a disappearance of germinal centers from lymphatic tissue. There is a complete lack of antibody production. It is associated with the most common immunodeficiency seen in childhood. Disease onset occurs most often in the second decade of life.

Phagocytic Dysfunction

Name this disorder?

Lymphopenia is usually present. Thymus gland fails to develop. Chronic mucocutaneous candidiasis is an associated disorder.

B cell deficiency (CVID)

Name that disorder?

IgAdeficiency is present in 40% of individuals. T cell deficiencies become more severe with age Neuroloic symptoms ususally occur before 5 years of age.

T cell deficiency

Name that disorder?

It usually occurs as a result of underlying disease processes. It frequently is caused by certain autoimmune disorders. it may be caused by certain viruses.

Secondary Immunodeficiency

Disorder marked by an almost complete lack of immunoglobulins or antibodies.

Agammaglobulinemia

Condition marked by development of urticaria and an edematous area of skin, mucous membranes, or viscera

angioneurotic edema

Loss of muscle coordination

ataxia

Autosomal recessive disorder affecting T and B cell immunity primarily seen in children and resulting in a degenerative brain disease.

ataxia telangiectasia

lack of one or more of the five immunoglobulins caused by B cell deficiency

hypogammaglobulinemia

Person with a secondary immunodeficiency and associated immunosuppression .

immunocompromised host

general lack of immunoglobulins in the blood

panhypoglobulinemia

disorder involving a complete absence of humoral and cellular immunity resulting from an x linked or autosomal genetic abnormality.

severe combined immunodefeciency disease

vascular lesions caused by dilated blood vessels

telangiectasia

T cell deficiency that occurs when the thymus gland fails to develop normally during embryo-genesis; also known as DiGeorge syndrome

thymic hypoplasia

immunodeficency characterized by thrombocytopenia and the absence of T and B cells

Wiskott Aldrich syndrome

This primary immunodeficiency disorder presents as a bacterial, fungal, and viral infection; deep seated cold abscesses. Treatment is antibiotic therapy and treatment for viral and fungal infections. Granulocyte macrophage colony (GM - CSF); granulocyte colony stimulating factor (G - CSF). What is the disorder and its Immune component?

Hyperimmunoglobulinemia E (HIE) syndrome; Phagocytic cells

This primary immunodeficiency disorder presents as a severe pyogenic infections soon after birth. Treatment for this is passive pooled plasma or grammaglobulin. What is the disorder and it immune component?

Burtons disease aka sex linked agammaglobulinemia; B lymphocytes.

This primary immunodeficiency disorder presents as bacterial infections such as giardia lamlia. Treatment is IV immunoglobulin (IVIG), and flagyl. What is the disorder and its immune component?

Common variable Immunodeficiency (CVID); B lymphocytes

This primary immunodeficiency disorder presents as pernicious anemia. The treatment is vitamin B12. What is missing immune component?

B lymphocytes

This primary immunodeficiency disorder presents as chronic respiratory infections. The treatment is antimicrobial therapy. What is the missing immune component?

B lymphocytes

This primary immunodeficiency disorder presents as a predisposition to recurrent infections adverse reactions to blood transfusions or immunoglobulin, autoimmune diseases and hypothyroidism. There is not current treatment. What is the disorder and what is the missing immune component?

Immunoglobulin A (IgA) deficiency; B lymphocytes

This primary immunodeficiency disorder presents as a hightened incidence of infectious diseases. Treatment is pooled immunoglobulin. What is the disorder and what is the missing immune component?

IgC2 deficiency; B lymphocytes

This primary immunodeficiency disorder presents as recurrent infections hypoparathyroidism, hypocalcemia, tetany, convulsions, congenital heart disease, possible renal and facies abnormalities. Treatment is Thymus graft. What is the disorder and what is the missing immune component?

Thymic hypoplasia (diGeorge syndrome; T lymphocytes

This primary immunodeficiency disorder presents as a candida albicans infection of the mucous membrane, skin, and nails; endocrine abnormalties (hypoparathyroidism, Addisons disease). Treatment includes antiungal agents: Topical; miconazole. Oral; clotrimazole, ketoconazole. IV; Amphotericin B. What is the disorder and the missing immune component?

Chronic mucocutaneous candidiasis; T lymphocytes

This primary immunodeficiency disorder presents as ataxia with progressive neurologic deterioration, telangeictasia (vascular lesions), recurrent infections; malignancies. Treatment is antimicrobial therapy; management of presenting symptoms; fetal thymus transplant, IVIG. what is the disorder and the missing immune component?

Ataxia telangiectasia; B and T lymphocytes.

This primary immunodeficiency disorder presents as severe infections and malignancies. Treatment is antimicrobial therapy, IVIG, bone marrow transplantation; thymus transplantation; Thymus factors. What is the disorder and the missing immune component?

Nezelof's syndrome; B and T lymphocytes.

This primary immunodeficiency disorder presents as thrombocytopenia resulting in bleeding, infections; malignancies. Treatment includes antimicrobial therapy; splenectomy with continuous antibiotic prophylaxis; IVIG and bone marrow transplantation. What is the disorder and the missing immune component?

Wiskott Aldrich syndrome; B and T lymphocytes

This primary immunodeficiency disorder presents as overwhelming severe fatal infections soon after birth (also includes opportunistic infections). Treatment includes; antimicrobial therapy; IVIG and bone marrow transplantation. What is the disorder and the missing immune component?

Severe combined immunodeficiency disease (SCID); B and T lymphocytes

This primary immunodeficiency disorder presents as episodes of edema in various parts of the body including respiratroy tract and bowels. Treatment includes: Pooled plasma, and androgen therapy. What is the disorder and the missing immune component?

Angioneurotic edema; Complement system

This primary immunodeficiency disorder presents as lysis of erythrocytes due to lack of decay accelerating factor (DAF) on erythrocytes. Treatment; none. What is the disorder and the missing immune component?

Proxysmal nocturnal hemoglobinuria (PNH); Complement system.