VI - Immunodeficiency Disorders Flashcards Preview

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Flashcards in VI - Immunodeficiency Disorders Deck (36):
1

State in which the immune system's ability to fight infectious disease is compromised, can occur if any of the four major components of the immune system are compromised

Immunodeficiency

2

Major Components of the Immune System

B-cells (antibodies), T-cells, phagocytes, complement

3

Recurrent infections with pyogenic bacteria indicate

B-cell Deficiency

4

Recurrent infections with fungi, viruses or protozoa indicate

T-cell Deficiency

5

Immunodeficiency: Genetic susceptibility to infections since childhood

Primary

6

Immunodeficiency: Acquired susceptibility to infection as a result of external processes or disease

Secondary

7

Very low levels of all immunoglobulins, virtual abscence of B-cells due to tyrosine kinase mutation, cell-mediated inmmunity is normal

X-Linked/Bruton's Agammaglobulinemia

8

B-Cell Disorders: Male infants, 6 mos. old, recurrent pyogenic bacterial, enteroviral infections and giardiasis

X-Linked/Bruton's Agammaglobulinemia

9

X-Linked/Bruton's Agammaglobulinemia: Treatment

pooled gamma globulin

10

Failure of isotype switching, recurrent bacterial sinus and lung infections

Selective IgA Deficiency

11

Why shouldn't you treat Selective IgA Deficiency with gamma globulin preparations?

formation of antibodies against foreign IgA → cross-reaction depletes their already low IgA or may cause anaphylaxis

12

Defect in B-cell maturation to plasma cells, diagnosis of exclusion, recurrent pyogenic bacterial infections, most common form of severe antibody deficiency affecting both children and adults

Common Variable Immunodeficiency

13

Common Variable Immunodeficiency: Treatment

pooled gamma globulin

14

Profound deficit of T-cells from failure of development of thymus and parathyroids due to a defect in the 3rd and 4th pharyngeal pouches, humoral immunity is normal

Di George Syndrome

15

T-Cell Disorders: Tetany due to hypocalcemia, severe viral, fungal or protozoal infections during childhood

Di George Syndrome

16

Di George Syndrome: Treatment

transplant of fetal thymus (< 14 wks old)

17

Specific T-cell deficiency for Candida albicans, other T-cell and B-cell functions are normal, recurrent candidiasis (skin, mucous membranes), in children

Chronic Mucocutaneous Candidiasis

18

Chronic Mucocutaneous Candidiasis: Treatment

azole antifungal drugs

19

X-Linked Severe Combined Immunodeficiency (SCID)

defect in IL-2 receptors in T-cells

20

Autosomal Severe Combined Immunodeficiency (SCID)

ADA deficiency

21

Recurrent bacterial, viral, fungal and protozoal infections at 3 mos., enclosure in plastic bubble, bone marrow transplant

Severe Combined Immunodeficiency (SCID)

22

X-linked, male infants, inability to mount IgM response, mutation in WASP gene for actin filament assembly recurrent pyogenic infections, eczema and bleeding due to thrombocytopenia, bone marrow transplant

Wiskott-Aldrich Syndrome

23

Autosomal recessive, mutations in DNA repair enzymes, IgA deficiency, ataxia, telangiectasia, recurrent infections by 2 years of age, supportive management

Ataxia-Telangiectasia

24

Lack of NADPH oxidase activity, failure of oxidative burst, normal B- and T-cell activity, recurrent infections with catalase (+) bacteria and fungi (A. fumigatus), widespread granulomas of unknown etiology, antibiotic chemoprophylaxis

Chronic Granuloma Disease

25

Chronic Granuloma Disease: Diagnosis

Nitroblue Tetrazolium Test (NBT)

26

Autosomal recessive, failure of phagolysosomal fusion, faulty microtubules impair neutrophil chemotaxis, recurrent pyogenic infections caused by staphylococci, antibiotics

Chediak-Higashi Syndrome

27

Autosomal recessive, mutation in integrins, defective adhesion (LFA-1) proteins on the surface of phagocytes, severe pyogenic infections in infancy, delayed separation of umbilical cord, antibiotics, bone marrow transplant

Leukocyte Adhesion Deficiency (LAD)

28

Early Complement Deficiency

C2 & C3 Deficiency

29

Terminal Complement Deficiency

C5b-C9 Deficiency

30

Most common complement defect, usually asymptomatic but may develop septicemia of SLE

C2 Deficiency

31

Complement deficiency with recurrent pyogenic infections due to Staphylococcus aureus

C3 Deficiency

32

Inability to form membrane attack complexes, bacteremia with Neisseria meningitidis and gonorrhoeae, vaccination

C5b-C9 Deficiency

33

Secondary Immunodeficiency: Decreased supply of AA and synthesis of IgG and complement, malnourished child with recurrent pyogenic infections, antibiotics, nutritional support

Malnutrition

34

HIV infects and kills

CD4+ helper T-cells

35

Secondary Immunodeficiency: Loss of cell-mediated immunity, opportunistic infections caused by bacteria, viruses, fungi and protozoa

AIDS

36

AIDS: Treatment

Highly-Active Antiretroviral Therapy (HAART)