Exam 3: Section 4 Continued

Question 1

Primary immune deficiency disorders

Answer 1

Genetic
Early onset (6-24 months)
Recurrent infections

Question 2

Bruton Disease aka

Answer 2

X-linked agammaglobulinemia

Question 3

Bruton Disease affects

Answer 3

Males, 6 months old

Question 4

Bruton disease

B cells can’t mature, which means

Answer 4

No antibodies

Question 5

Bruton disease can become more apparent after recurrent ___ and ___

Answer 5

Bacterial and viral infections

Question 6

Treatment for Bruton Disease

Answer 6

IgG infusions

Good prognosis

Question 7

Severe Combined Immunodeficiency (SCID) symptoms

Answer 7

Lymphopenia and lymphoid atrophy

Question 8

SCID occurs in

Answer 8

1 in 65,000 (native Americans)

1 in 100,000 (other groups)

Question 9

____ immune deficiency disorders more common than ___

Answer 9

Secondary

Primary

Question 10

Secondary immune deficiency disorders can occur from

Answer 10

Infections
Malnutrition
Cancer
Therapy- induced immunosuppressive (MC)

Question 11

Mechanisms of secondary immune deficiency disorders

Answer 11

1. Bone marrow dysfunction

2. Lymphocyte dysfunction

Question 12

Example of lymphocyte dysfunction

Answer 12

Acquired immunodeficiency syndrome (AIDS)

Question 13

AIDS spread in

Answer 13

Blood
Semen
Vaginal fluid
Breast milk

Question 14

AIDS destroys

Answer 14

CD4 T cells
Macrophages
Dendritic cells

Question 15

Most common AIDS transmission in US

Answer 15

Male -male sex

48%

Declining

Question 16

Most common AIDS transmission in Africa and Asia

Answer 16

Male-female

34%

Increasing

Question 17

17% of AIDS spread via

Answer 17

IV drug abusers

Question 18

<1% of AIDS spread via

Answer 18

Blood recipients

Question 19

<1% AIDS spread via

Answer 19

Transplacental

Childbirth

Milk

Question 20

> 80% of cases of AIDS are ____ transmitted

Answer 20

Sexually

Question 21

HIV Diagnosis

Answer 21

1. ELISA test

2. Western blot

Question 22

3 Phases of HIV/AIDS

Answer 22

1. Acute
2. Chronic
3. Crisis

Question 23

Acute Phase of HIV

Answer 23

3-6 weeks post infection experience “flu-like” symptoms
High viremia
Death of many CD4 T cells

Question 24

Viremia of AIDS decreases around

Answer 24

12 weeks

Question 25

Chronic phase of HIV

Answer 25

2-10 years Generalized lymphadenopathy, fatigue, fever Steady decrease of CD4 T cells Minor infections

Question 26

Minor infections that can occur in chronic stage of HIV

Answer 26

Candidiasis HSV

Question 27

Crisis Phase of HIV

Answer 27

<200 cells/microliter Viremia spikes Cachexia Fatigue Fever >1 month

Question 28

HIV binds to ___ on CD4 T cells

Answer 28

Gp120

Question 29

HIV undergoes conformational changes due to interactions with

Answer 29

CCR5 protein

Question 30

HIV crosses BBB by infecting

Answer 30

Monocytes - macrophages

Question 31

Opportunistic infections

Answer 31

1. Pneumocystis Pneumonia 2. Candidiasis 3. Cytomegalovirus 4. TB

Question 32

Tumors common in HIV

Answer 32

1. Kaposi Sarcoma | 2. Lymphoma

Question 33

Amyloidosis occurs when extracellular fibrillar proteins are deposited and cause

Answer 33

Tissue damage and dysfunction

Question 34

Amyloid deposits are composed of

Answer 34

Non branching fibrils

Question 35

Amyloid deposited are unable to be removed by

Answer 35

Phagocytosis

Question 36

Forms of amyloid proteins

Answer 36

1. AL 2. AA 3. A beta 4. TTR

Question 37

AL proteins are associated with

Answer 37

Multiple myeloma

Question 38

AA proteins are deposited in patients with

Answer 38

Chronic inflammatory conditions

Question 39

Beta amyloid deposited in

Answer 39

Alzheimer’s disease

Question 40

Transthyretin (TTR) is a normal protein that accumulates in excessive amounts as a form of

Answer 40

Amyloidosis

Question 41

Older patients are most likely to have TTR accumulate following

Answer 41

Mutations in protein misfolding

Question 42

When TTR accumulates in the walls of the heart, it is called

Answer 42

Senile cardiac amyloidosis

Question 43

When TTR accumulates in multiple organ systems, it is called

Answer 43

Senile systemic amyloidosis

Question 44

Patterns of amyloid depositions

Answer 44

1. Systemic | 2. Localized

Question 45

MC cause of systemic amyloid distribution

Answer 45

AL proteins accumulated after multiple myeloma

Question 46

Systemic amyloid distribution has a ___ prognosis

Answer 46

Poorer

Question 47

Localized amyloid distribution causes changes in ___ and ___ in a singular region of the body

Answer 47

Tissue structure and function

Question 48

Common areas for localized amyloid distribution

Answer 48

``` Heat Lungs Brain Skin Liver Ligaments of wrist Tongue Larynx Kidney Spleen ```

Question 49

Diagnosis of amyloidosis requires ___ evaluation

Answer 49

Histological

Question 50

Congo red dye combined with polarized light produces

Answer 50

Apple-green birefringence | Characteristic of amyloidosis

Question 51

____ and ___ are MC cause of death related to amyloidosis

Answer 51

Renal and heart failure

Question 52

SCID Involved impaired development of

Answer 52

Lymphocytes (T and B cells)

Question 53

Common symptoms of SCID within first year

Answer 53

1. Oral candidiasis 2. Severe diaper rash 3. Cytomegaloviral infections 4. Fungal infections of lungs 5. Failure to thrive 6. Varicella zoster viral infection