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PPOM1 Block 4 > Lecture 153 > Flashcards

Lecture 153 Flashcards

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1
Q

What type of PID is common variable immunodeficiency?

A

B-cell

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2
Q

What type of PID is selective IgA deficiency?

A

B cell

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3
Q

What type of PID is IL-12 receptor deficiency?

A

T cell

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4
Q

What type of PID is chronic mucocutaneous candidiasis?

A

T cell

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5
Q

What type of PID is Chediak-Higashi syndrome?

A

Phagocytic

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6
Q

What type of PID is chronic granulomatous disease?

A

Phagocytic

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7
Q

When do B cell disorders typically present? Why?

A

3-4 months of age due to maternal IgG protection

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8
Q

What is the pathogenesis of CVID?

A

Normal B cells do not differentiate due to arrest in plasma cell differentiation

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9
Q

Hypogammaglobulinemia: low IgG, low IgA, +/- low IgM describs what PID?

A

CVID

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10
Q

When does CVID typically present?

A

2nd-3rd decade

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11
Q

What infectious components are common in CVID?

A

Recurrent sinopulmonary infections & chronic GI infections

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12
Q

What type of malignancy are patients with CIVD predisposed to?

A

Lymphoma (usually EBV-associated)

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13
Q

What are some non-infectious features of CVID?

A

Autoimmune disease, granulomatous disease, chronic lung disease, GI disorders

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14
Q

Lab results showing low serum IgG, IgA, and/or IgM and poor immunization response corresponds to what PID?

A

CVID

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15
Q

What is the treatment for CVID?

A

Antibiotics, IVIG

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16
Q

What features indicate a poor prognosis in CVID?

A

Auotimmune involvement or lymphoproliferation

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17
Q

Isolated absence of serum/secretory IgA describes the pathophysiology of what PID?

A

Selective IgA deficiency

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18
Q

What is the most common presentation of selective IgA deficciency?

A

Majority of patients are asymptomatic

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19
Q

What infectious features are seen in selective IgA deficiency?

A

Recurrent sinopulmonary infections and GI infections (especially giardia)

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20
Q

Anaphylactic reaction to blood products, foo/respiratory allergies, GI disorders, and autoimmine disorders are all clinical features of what PID?

A

Selective IgA deficiency

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21
Q

What is the earliest that selective IgA deficiency can be diagnosed?

A

At least 4 years old

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22
Q

Viral infections are more severe, persistent, and disseminated is what group of PIDs?

A

T cell disorders

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23
Q

What cytokine is secreted from macrophages, meutrophils, and dendritic cells in response to infection with mycobacteria?

24
Q

Where are IL-12 receptors located?

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A

NK cells and T cells

25
IL-12 binding to its receptor stimulates secretion of what cytokine?
IFN-gamma
26
What type of cells does IFN-gamma activate to secrete TNF-alpha?
Phagocytes
27
What is the inheritance pattern of IL-12 receptor deficiency?
Autosomal recessive
28
BCG disseminated infection after vaccination is common id what PID?
IL-12 receptor deficiency
29
What are the clinical features of IL-12 receptor deficiency?
Recurrent infections of salmonella, mycobacterium tuberculosis, and nontuberculosis mycobacterium
30
When does IL-12 receptor deficiency typically present?
Early childhood
31
How is IL-12 receptor deficiency treated?
Antibiotics to specific infection & IFN-gamma
32
WHat PID is characterized by an impaired immune response to Candida?
Chronic mucocutaneous candidiasis
33
Defective Th17 lymphocytes or defective synthesis/signaling of IL-17 are associated with what PID?
Chonic mucocutaneous candidiasis
34
What PID is AIRE gene defect associated with? What is the inheritance pattern?
Chronic mucocutaneous candidiasis, autosomal recessive
35
What PID is STAT1 gene defect associated with? What is the inheritance pattern?
Chronic mucocutaneous candidiasis, autosomal dominant
36
When does chronic mucocutaneous candidiasis typically present?
1st month of life
37
Recurrent Candida infections, autoimmune polyendocrinopathy, and skin dystrophy are features of what medical condition?
APECED aka APS-1
38
Autoimmune disease, bone marrow failure, and mouth/esophagus malignancy are clinical features associated with what PID?
Chronic mucocutaneous candidiasis
39
What is the treatment for chronic mucocutaneous candidiasis?
Systemic azoles
40
What PID is described as a disorder of intracellular granule formation?
Chediak-Higashi Syndrome
41
Autosomal recessive mutation of CHS1/LYST is associated with what PID?
Chediak-Higashi syndrome
42
What are the common infectious agents associated with Chediak-Higashi syndrome?
Gram + and Gram - bacteria, fungi, most commonly Staph. aureus
43
What are the three most common sites of infection in Chediak-Higashi syndrome?
Mucuous membranes, skin, and respiratory tract
44
Partial albinism, easy bruising, and peripheral neuropathy are clinical features of what PID?
Chediak-Higashi syndrome
45
Pancytopenia, fever, and massive lymphohistiocytic infiltration of liver, spleen, and lymph nodes is a complication of what PID?
Chediak-Higashi syndrome, accelerated phase
46
What is the primary test for Chediak-Higashi syndrome?
Genetic testing for CHS1/LYST gene
47
How is Chediak-Higashi syndrome treated?
Prophylactic TMP-SMX, INF-gamma, HSCT
48
What are the results of HSCT in Chediak-Higashi syndrome?
Prevents accelerated phase, does not prevent neurologic degenration or albinism
49
What PID is caused by defective phagocytes with inability to form respiratory burst?
Chronic granulomatous disease
50
What is the inheritance pattern of CGD?
X-linked (more common) or autosomal recessive
51
What is the most common microbial group to cause infections in CGD?
Catalase positive bacteria and fungi
52
When does CGD typically present?
In first 5 years of life
53
Pneumonia, lymphadenitis, osteomyelitis, and skin infections from catalase pozitive microorganisms are indicative of what PID?
CGD
54
Growht failure, anemia of chronic disease, and granulomas and non-infectious clinical features of what PID?
CGD
55
What is the preferred test for CGD?
DHR 123 test, can distinguish between x-linked and autosomal forms
56
Only X-linked carriers of CGD can be detected with this lab test
NBT test
57
How is CGD treated?
TMP-SMX, itraconozole, and IFN-gamma prophylaxis, HSCT is curative

PPOM1 Block 4 (44 decks)

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