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Gen Path > Exam 3: Section 4 Continued > Flashcards

Exam 3: Section 4 Continued Flashcards

1
Q

Primary immune deficiency disorders

A

Genetic
Early onset (6-24 months)
Recurrent infections

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2
Q

Bruton Disease aka

A

X-linked agammaglobulinemia

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3
Q

Bruton Disease affects

A

Males, 6 months old

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4
Q

Bruton disease

B cells can’t mature, which means

A

No antibodies

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5
Q

Bruton disease can become more apparent after recurrent ___ and ___

A

Bacterial and viral infections

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6
Q

Treatment for Bruton Disease

A

IgG infusions

Good prognosis

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7
Q

Severe Combined Immunodeficiency (SCID) symptoms

A

Lymphopenia and lymphoid atrophy

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8
Q

SCID occurs in

A

1 in 65,000 (native Americans)

1 in 100,000 (other groups)

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9
Q

____ immune deficiency disorders more common than ___

A

Secondary

Primary

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10
Q

Secondary immune deficiency disorders can occur from

A

Infections
Malnutrition
Cancer
Therapy- induced immunosuppressive (MC)

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11
Q

Mechanisms of secondary immune deficiency disorders

A
  1. Bone marrow dysfunction

2. Lymphocyte dysfunction

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12
Q

Example of lymphocyte dysfunction

A

Acquired immunodeficiency syndrome (AIDS)

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13
Q

AIDS spread in

A

Blood
Semen
Vaginal fluid
Breast milk

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14
Q

AIDS destroys

A

CD4 T cells
Macrophages
Dendritic cells

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15
Q

Most common AIDS transmission in US

A

Male -male sex

48%

Declining

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16
Q

Most common AIDS transmission in Africa and Asia

A

Male-female

34%

Increasing

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17
Q

17% of AIDS spread via

A

IV drug abusers

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18
Q

<1% of AIDS spread via

A

Blood recipients

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19
Q

<1% AIDS spread via

A

Transplacental

Childbirth

Milk

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20
Q

> 80% of cases of AIDS are ____ transmitted

A

Sexually

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21
Q

HIV Diagnosis

A
  1. ELISA test

2. Western blot

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22
Q

3 Phases of HIV/AIDS

A
  1. Acute
  2. Chronic
  3. Crisis
23
Q

Acute Phase of HIV

A

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

24
Q

Viremia of AIDS decreases around

A

12 weeks

25
Q

Chronic phase of HIV

A

2-10 years

Generalized lymphadenopathy, fatigue, fever

Steady decrease of CD4 T cells

Minor infections

26
Q

Minor infections that can occur in chronic stage of HIV

A

Candidiasis

HSV

27
Q

Crisis Phase of HIV

A

<200 cells/microliter

Viremia spikes

Cachexia

Fatigue

Fever >1 month

28
Q

HIV binds to ___ on CD4 T cells

A

Gp120

29
Q

HIV undergoes conformational changes due to interactions with

A

CCR5 protein

30
Q

HIV crosses BBB by infecting

A

Monocytes - macrophages

31
Q

Opportunistic infections

A
  1. Pneumocystis Pneumonia
  2. Candidiasis
  3. Cytomegalovirus
  4. TB
32
Q

Tumors common in HIV

A
  1. Kaposi Sarcoma

2. Lymphoma

33
Q

Amyloidosis occurs when extracellular fibrillar proteins are deposited and cause

A

Tissue damage and dysfunction

34
Q

Amyloid deposits are composed of

A

Non branching fibrils

35
Q

Amyloid deposited are unable to be removed by

A

Phagocytosis

36
Q

Forms of amyloid proteins

A
  1. AL
  2. AA
  3. A beta
  4. TTR
37
Q

AL proteins are associated with

A

Multiple myeloma

38
Q

AA proteins are deposited in patients with

A

Chronic inflammatory conditions

39
Q

Beta amyloid deposited in

A

Alzheimer’s disease

40
Q

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

A

Amyloidosis

41
Q

Older patients are most likely to have TTR accumulate following

A

Mutations in protein misfolding

42
Q

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

A

Senile cardiac amyloidosis

43
Q

When TTR accumulates in multiple organ systems, it is called

A

Senile systemic amyloidosis

44
Q

Patterns of amyloid depositions

A
  1. Systemic

2. Localized

45
Q

MC cause of systemic amyloid distribution

A

AL proteins accumulated after multiple myeloma

46
Q

Systemic amyloid distribution has a ___ prognosis

A

Poorer

47
Q

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

A

Tissue structure and function

48
Q

Common areas for localized amyloid distribution

A 
Heat 
Lungs 
Brain
Skin 
Liver 
Ligaments of wrist 
Tongue 
Larynx 
Kidney 
Spleen
49
Q

Diagnosis of amyloidosis requires ___ evaluation

A

Histological

50
Q

Congo red dye combined with polarized light produces

A

Apple-green birefringence

Characteristic of amyloidosis

51
Q

____ and ___ are MC cause of death related to amyloidosis

A

Renal and heart failure

52
Q

SCID Involved impaired development of

A

Lymphocytes (T and B cells)

53
Q

Common symptoms of SCID within first year

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

Gen Path (10 decks)

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