ch 36 immunodefieciency D/O

Hyperimmunohlobulinemia E syndrome (formerly known as Job syndrome)

rare phagocytic disorder, white blood cells cannot initiate an inflammatory response to infectious organisms. This results in recurrent bacterial infections of the skin and lung; abnormalities of the connective tissue, skeleton, vascular system, and dentition; and extremely elevated levels of IgE

Primary immunodeficiencies predispose people to these three conditions

severe infections, autoimmunity, cancer

DiGeorge syndrome

thymic hypoplasia: T-cell deficiency that occurs when the thymus gland fails to develop normally during embryogenesis;

What are immune disroders caused by

Immunodeficiency may be caused by a defect or deficiency of phagocytic cells, B Lymphocytes, T Lymphocyes, or the complement system

Brutons disease (Bruton agammaglobulinemia)

This syndrome is called X-linked agammaglobulinemia (Bruton's disease), because all antibodies disappear from the patient's plasma. B cells in the peripheral blood and IgG, IgM, IgA, IgD, and IgE are low or absent. Infants born with this disorder suffer from severe infections starting soon after birth.

Angioneurotic edema

results from the deficiency of C1 esterase inhibitor, which opposes the release of inflammatory mediators. Sx includes recurrent attacks of edema formation in the subcutaneous tissue, gastrointestinal tract, and upper airway ( autosomal dominant disorder) complement system

Two types of inherited B cell deficiencies result from a lack of differentiation of B-cells into ____________ and ____________

Mature B-cells , plasma cells

List some of the common different primary immunodeficiencies

Disorders of humoral immunity (affecting B-cell differentiation or antibody production) T-cell defects, combined B and T-cell defects, phagocytic d/o and complement deficiencies.

Five disorders of common primary immunodeficiencies are

Humoral immunity, T-cell defects, combined B and T-cell defects, phagocytic disorder, complement production

Patients that have neutropenia are at risk for what problem

Patients with neutropenia are at risl for developing severe infections

More than 50% of patients with common variable immunodeficiency (CVID) develop the disorder

Pernicious anemia

The most prevelant cause of immunodefiency worldwode is

Severe malnutritiion

The nurse is administering an infusion of gamma-globulin to a patient in the hospital. When should the nurse discontinue the infusion

When the patient complains of flank pain • When the patient begins to have shaking chills • When the patient complains of tightness in the chest • When the patient complains of nausea

The majority of patient with primary immunodeficiency are in which age group

Younger than 20 About 80% of the patients with primary immunodeficiency are younger than age 20 years.

Cardinal symptoms of immune deficiency

chronic or recurrent and severe infections, infections caused by unusual organisms or by organisms in the normal flora, poor response to standard treatment and chronic diarrhea. Pt is also suseptible to secondary disorders including autoimmune disease & Lymphoreticular malignancies

Describe the symptoms that infants with X-linked agammaglobulinemia present with

infants become symptomatic after the natural loss of maternally transmitted immunoglobulins (5-6 mo). Sx of recurrent pyogenic infections usually occur by that time