Exam 2 Chapter 4 - Immune Deficiency Disorders

Question 1

What type of disorder causes vulnerability to infection or disease?

Answer 1

immune deficiency disorder

Question 2

Immune deficiency disorders are either _______ or ________

Answer 2

primary (congenital) or secondary (acquired)

Question 3

Which type of Immune deficiency disorder is rare, most commonly genetic, and has early onset (6-24 months)?

Answer 3

primary

Question 4

X-Linked agammaglobulinemia typically occurs in _ month old _____

Answer 4

6, males

Question 5

______ B cell maturation is a characteristic of X-Linked agammaglobulinemia

Answer 5

failed

Question 6

Bacterial or viral infection such as otitis media, pharyngitis, and sinusitis are characteristic of which primary IDD?

Answer 6

X-Linked agammaglobulinemia

Question 7

What is the treatment for X-Linked agammaglobulinemia?

Answer 7

IgG infusions (good prognosis)

Question 8

Which primary IDD (Immune deficiency disorder) has an decreased antibody response to infection?

Answer 8

Common variable immunodeficiency

Question 9

Common variable immunodeficiency has normal _ cells, but no ______ cells, and increased GI infections?

Answer 9

B, plasma

Question 10

Which primary IDD affects males and females (10-30 yrs, 1:50,000)?

Answer 10

Common variable immunodeficiency

Question 11

What is the most common primary IDD (Immune deficiency disorder)?

Answer 11

Isolated IgA deficiency

Question 12

Isolated IgA deficiency has ________ IgA production and is ___________ or very mild?

Answer 12

decreased, asymptomatic

Question 13

Isolated IgA deficiency can cause what 2 issues?

Answer 13

recurrent diarrhea or URTIs

Question 14

How common is Isolated IgA deficiency?

Answer 14

1 in 700 caucasians

Question 15

Hyper-IgM Syndrome is characterized by _______ IgM levels, and _______ IgG, IgA, IgE

Answer 15

increased, decreased

Question 16

Hyper-IgM Syndrome is also characterized by _______ ______ infection?

Answer 16

recurrent bacterial

Question 17

Which primary IDD is 70% X-linked mutations and occurs in 1:100,000 people?

Answer 17

Hyper-IgM Syndrome

Question 18

Which primary IDD is also known as “bubble boy”? (vulernable to ALL microbial infections)

Answer 18

Severe combined immunodeficiency (SCID)

Question 19

Severe combined immunodeficiency (SCID) is charactized by _______ and _______ atrophy

Answer 19

lymphopenia and lymphoid

Question 20

David Vetter is associated with which primary IDD?

Answer 20

Severe combined immunodeficiency (SCID)

Question 21

X-Linked agammaglobulinemia, Common variable immunodeficiency, Isolated IgA deficiency, Hyper-IgM Syndrome, Severe combined immunodeficiency (SCID) are the 5 ________ IDDs?

Answer 21

primary

Question 22

Are primary or secondary IDDs more common?

Answer 22

secondary (acquired)

Question 23

Infections, malnutrition, aging, cancer, and

Therapy-induced immunosuppression are all which type of IDD?

Answer 23

secondary

Question 24

What is the most common type of secondary IDD?

Answer 24

Therapy-induced immunosuppression

Question 25

Secondary IDDs suppress ______ and cause ________ dysfunction

Answer 25

marrow, lymphocyte

Question 26

Blood, semen, vaginal fluid, and breast milk are all ways to spread ____?

Answer 26

HIV (Human immunodeficiency virus)

Question 27

What condition destroys CD4+, T cells, and macrophages via apoptosis or cytotoxicity?

Answer 27

HIV

Question 28

Opportunistic infections, tumors, & CNS defects often affect people with what condition?

Answer 28

HIV

Question 29

AIDS is most commonly spread ____-____ sex in the US and ____-_____ sex in Africa/Asia

Answer 29

male-male, male-female

Question 30

What percentage of AIDs is sexually transmitted?

Answer 30

> 75%

Question 31

Having other STDs ________ the spread of AIDS

Answer 31

increases

Question 32

I.V drug abusers, needlestick injury, or transfusion recipients could acquire AIDS through _________ transmission

Answer 32

parenteral

Question 33

What are the 3 types of AIDS?

Answer 33

acute, chronic, crisis

Question 34

Which type of AIDS is characterized by pyrexia, pharyngitis, myalgia, or viremia?

Answer 34

acute

Question 35

Which type of AIDS is onset 2-10 years after infection?

Answer 35

chronic

Question 36

Generalized lymphadenopathy is the most common feature of what type of AIDS

Answer 36

chronic

Question 37

Chronic AIDS is characterized by a steady decrease of ___ and _ cells and ______ viremia

Answer 37

CD+ and T cells, increase

Question 38

What type of AIDS is characterized with ≤200 cells/µL, ↑ viremia, cachexia, fatigue, diarrhea, weight loss, and CNS defects?

Answer 38

crisis

Question 39

Opportunistic infections account for what percentage of AIDS related deaths?

Answer 39

80%

Question 40

Pneumocystosis and candidiasis are what type of virus/infection?

Answer 40

fungal

Question 41

KSHV, EBV and HPV are what type of virus/infection?

Answer 41

ontogenic

Question 42

What is common treatment for AIDS?

Answer 42

anti-retroviral meds

Question 43

Misfolded, mutated or unstable proteins can cause many inflammatory/inherited conditions known as what?

Answer 43

Amyloidosis

Question 44

In failed __________ Amyloid proteins accumulate, damaging tissues and disrupting function

Answer 44

phagocytosis

Question 45

Congo red dye tests for what? What is the positive indication?

Answer 45

Amyloid, apple-green color

Question 46

Electrophoresis tests what 2 substances?

Answer 46

Serum and urine

Question 47

What are the 3 types of Amyloid proteins?

Answer 47

1) amyloid light (AL) 2) amyloid associated (AA) 3) β-Amyloid (Aβ)

Question 48

B cell proliferation (plasma cells), immunoglobulin light chains, Bence-Jones proteins are characteristics of which type of amyloid protein?

Answer 48

amyloid light (AL)

Question 49

Liver: serum-associated amyloid (SAA) and chronic inflammation: TB, RA, AS, IBD, osteomyelitis are characteristics of which type of amyloid protein?

Answer 49

amyloid associated (AA)

Question 50

``` Amyloid precursor protein: cerebral plaques Alzheimer disease (APP, Down syndrome) are characteristics of which type of amyloid protein? ```

Answer 50

β-Amyloid (Aβ)

Question 51

What are the 3 types of Amyloidosis?

Answer 51

systemic, localized, hereditary

Question 52

Malignant plasma cells are characteristic primary or secondary amyloidosis?

Answer 52

primary

Question 53

Which type of amyloidosis is rare, familial, and geographic?

Answer 53

hereditary

Question 54

What is the most common organ affected by amyloidosis (localized)?

Answer 54

kidney

Question 55

Weakness, fatigue, cachexia are the most common features/side-effects of _______?

Answer 55

Amyloidosis

Question 56

Amyloidosis is most likely to be lethal if it involves what 2 organs?

Answer 56

kidney and heart

Question 57

Which type of amyloidosis has a poor prognosis?

Answer 57

systemic