Innate Immune Defects Flashcards Preview

Ch 20: Allergy and Immunology > Innate Immune Defects > Flashcards

Flashcards in Innate Immune Defects Deck (9)
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1
Q

What is Chronic Granulomatous Disease?

A

Disorder of phagocytic function. Phagocytes cannot undergo the respiratory burst to kill bacteria and fungi.

2/3s are X-linked

2
Q

How do patients with CGD present and what bugs cause it?

A

Present within first 5 years of life

Affects organs that are barriers:
Skin
Lungs
Liver
Lymph Nodes
Spleen
*Can have granulomas of the GU tract that leaves to urinary retention and bowel obstruction

You get deep abscesses, pneumonia, lymphadenitis, osteomyelitis, and systemic infections

*S. aureus, B. cepacia, Serratia, fungi

3
Q

How do you diagnose and treat CGD?

A

Diagnosis: nitroblue tetrazolium (NBT) test-assays phagocytic oxidase activity

Ppx: TMP/SMX and Itraconazole

*CAN receive LIVE viral vaccines, NO live bacterial vaccines (oral typhoid or BCG)

4
Q

What is Leukocyte Adhesion Deficiency (LAD)?

A

Defect in chemotaxis-WBCs can’t go where they need to

5
Q

How do pts present with LAD?

A
High WBC (20,000)
Perirectal Abscess
Indolent skin infection
Omphalitis
Delayed wound healing
Delayed umbilical separation 
Recurrent infections in the skin, mucosa, and respiratory tract
6
Q

How do you diagnosis and treat pts with LAD?

A

Diagnosis: Flow cytometry

Treat: Bone marrow and hematopoietic stem cell transplant (CURATIVE)

7
Q

What is Chediak-Higashi Syndrome, how is it inherited?

A

Autosomal Recessive

WBCs have lysosomal granules and abnormal chemotaxis

8
Q

How do pts with Chediak-Higashi Syndrome present?

A

Frequent infections-lung and skin
Easy bruisability
Oculocutaneous albinism

  • VERY fair-skinned, blonde, blue-eyed kid with frequent bad skin infections
  • S. aureus, Step pyogenes, and pneumococcus
9
Q

How do you diagnosis and treat pts with Chediak-Higashi Syndrome?

A

Diagnose-
Blood smear with GIANT granules in the neutrophils

Treat-
BMW transplant or die by 10 yo