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Peds Shelf > Immunologic Disease > Flashcards

Immunologic Disease Flashcards

1
Q

What mediates anaphylaxis?

A

IgE

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2
Q

How do you dx anaphylaxis?

A

Hx of exposure
Rapid onset
Serum tryptase @ 1, 4, 8 hours

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3
Q

How do you treat anaphylaxis?

A 
ABC
100% O2
Epi (1:1000 - 0.01 ml/kg) 0.1 mL - 0.3 mL
Diphenhydramine
Cimetidine
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4
Q

What are risk factors for severe anaphylactic reaction

A

Asthma
Beta-blockade
Adrenal Insufficiency

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5
Q

What is the most common cause of urticarial in children?

A

Viral infections

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6
Q

What causes hives? (6)

A 
Viruses & Bacteria
Medications
Foods
Insect stings
Autoimmune diseases
Malignancies
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7
Q

What type of hypersensitivity reaction is hives?

A

Type 1

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8
Q

What type of hypersensitivity reaction is serum sickness?

A

Type 3

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9
Q

What can cause serum sickness

A 
Anti-gout: allopurinol
Antimicrobials: PCN, ceph
Antiarrhythmic: quinidine, procainamide
Antihypertensive: captopril, hydralazine
Thyroid Meds
Serum blood products/vaccines
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10
Q

What are s/sx of serum sickness

A

6-21 days post exposure
Fever, joint pain, LAD, edema
Red, purpuric rash at junction of palmar or plantar skin
+/- renal/CV/pulm/neuro compx

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11
Q

How do you treat serum sickness

A

Symptoms usually spontaneously resolve

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12
Q

What type hypersensitivity is a drug reaction

A

All Types!

`

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13
Q

Type 1 Hypersensitivity is mediated by what?

A

IgE!!!

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14
Q

What is type 3 hypersensitivity mediated by?

A

Immune complex = Serum Sickness

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15
Q

What types of hypersensitivity reactions can penicillin cause?

A

Types 1-3

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16
Q

Ampicillin rash

A

Not urticarial!

Seen with Mono and Hyperuricemia

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17
Q

True or False: Most adverse food reactions have an immunologic basis?

A

False - most are intolerances i.e. N/V/D

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18
Q

The following is a classic triad of what disease:

  • Conjunctivitis
  • Oral ulceration
  • Urethritis
A

Stevens-Johnson Syndrome

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19
Q

What causes SJS?

A

Drugs: Sulfonamides and anticonvulsants

Mycoplasma pneumoniae

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20
Q

What is the prodromal phase of SJS?

A

1-14 days: Fever, headache, and malaise

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21
Q

What does the rash of SJS look like?

A

Red blistering target lesions

Inflamed bullous lesions

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22
Q

What is the clinical criteria for SJS

A

Cutaneous lesion + 2 mucosal surfaces

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23
Q

How do you diagnose SJS? What do you expect to find?

A

Skin biopsy

Perivascular mononuclear cell infiltrate

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24
Q

How do you treat SJS?

A

Hospital
IVF
Hyperalimentation i.e. TPN
Skin Care

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25
Q

Hyperalimentation

A

TPN

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26
Q

Left shift

A

Neutrophilic predominance

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27
Q

A 10 month old male infant presents with oral candidiasis. What should you consider?

A

Immune deficiency

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28
Q

What is the defect in SCID?

A

KO Humoral & Cellular immunity

Dec. adenosine deaminase –> defect in stem cell maturation

29
Q

How do you dx SCID? What else might you see?

A

Lymphopenia + dec. serum IgG, IgA, IgM

  • Absent thymus
  • *Frequent and severe infections, FTT
30
Q

What are 2 T-cell deficiencies

A

DiGeorge Syndrome

Chronic Mucocutaneous Candidiasis

31
Q

What are 3 humoral deficiencies?

A

IgA Deficinecy
Brutons agammaglobulinemia
CVID

32
Q

What are 3 diseases with deficient phagocytosis?

A

Chronic granulomatous disease
Chediak-Higashi Syndrome
Job Syndrome

33
Q

What do you get with a 22, q 11 deletion

A

DiGeorge Syndrome

34
Q

What two diseases have no thymic shadow?

A

SCID, DiGeorge Syndrome

35
Q

What structures are affected in DiGeorge?

A 
3rd and 4th brachial pouches
Thymus - absent T cells
Parathyroid - hypoCa tetany
Esophageal atresia
R. sided aortic arch, ASD/VSD
36
Q

How do you treat DiGeorge?

A

Thymic tissue transplant
BMT - at risk for GVDH
Irradiated blood products only

37
Q

Recurrent/persistent candidal infections of skin, nails and mucous membranes

A

Chronic Mucocutaneous Candidiasis

-Hypo PTH, hyper thyroid

38
Q

Selective IgA Deficiency S/Sx (4)

A

RTI, UTI, GI infections - giardia

AI diseases

39
Q

What 2 things are contraindicated in someone with Selective IgA deficiency?

A

Blood products and IVIg

40
Q

How do you dx IgA deficiency

A

IgA less than 5 mg/dL

41
Q

What is defective in Brutons Agammaglobulinemia?

A

Protein tyrosine kinase on B cell (Xq22)

X-linked

42
Q

What types of infections are those with Brutons agammaglobulinemia more likely to get?

A

Strep
H flu
Echovirus meningoencephalitis
Small tonsils, adenoids and LN

43
Q

CVID

A

Hypogammaglobulinemia, less severe than SCID
thymoma
alopecia areata
pernicious anemia
non-caseating sarcoid-like granulomas of lungs, spleen, skin and liver

44
Q

How do you diagnose chronic granulomatous disease?

A 
Leukocytosis
Inc. ESR
Abnormal CXR
Hypergammaglobulinemia
Leukocytes unable to reduce nitroblue tetrazolium
45
Q

What are the s/sx of chronic granulomatous disease?

A

Severe recurrent skin and LN infections
Pneumonitis -> pneumatocele
Osteomyelitis
HSmegaly

46
Q

What are the symptoms of Chediak Higashi Syndrome (4)

A

Skin and respiratory infection
Albinism
Pancytopenia
Progressive peripheral neuropathy

47
Q

How do you diagnose Chediak Higashi?

A

Large inclusions in all nucleated cells

48
Q

How do you treat Chediak Higashi? (3)

A

High-dose ascorbic acid
BMT - doesn’t treat/prevent neuropathy
Abx

49
Q

What is the defect in Job Syndrome? How do you diagnose it

A

Neutrophil chemotactic defect

IgE > 10,000 and eosinophilia

50
Q

Recurrent Staph infections, resistant to therapy
Pruritic eczematoid dermatitis
Coarse facial features

A

Job Syndrome (Hyper IgE)

51
Q

How do you treat Job Syndrome

A

Penicillinase-resistant abx

IVIG

52
Q

Ataxia telangiectasia: What are the 3 main symptoms

A

Telangiectasia
Ataxia
Variable Immunodeficiency

53
Q

When does Ataxia Telangiectasia present?

A

First 6 years
Wheelchair by 10-12 years
*Inc. risk of malignancy

54
Q

What lab abnormalities are seen in Ataxia Telangiectasia?

A

Inc. serum AFP

Inc. CEA

55
Q

How do you treat Ataxia Telangiectasia?

A

Supportive - pulmonary fx and immunologic status

56
Q

What disease has the triad of (male)
Thrombocytopenia
Eczema
Recurrent bacterial infections

A

Wiskott-Aldrich syndrome

57
Q

What is wrong in Wiskott-Aldrich? What labs do you see?

A

Can’t form antibodies to bacterial capsular polysaccharide antigens
Low IgM
High IgA

58
Q

Excessive bleeding from circumcision site can be an indicator for what disease?

A

Wiskott-Aldrich - thrombocytopenia

59
Q

In whom do you see hypocomplimentemia?

A

Lupus nephritis
Post strep GN
not HSP or minimal change

60
Q

C1q is deficient in ____.

A

SLE

61
Q

C1r, C1r/C1s, C4, C2 and C3 deficiency

A

Vasculitis - SLE

62
Q

C5-C8 deficiency

A

Neisserial infection

63
Q

C3 deficiency

A

Pneumococci

64
Q

C1 esterase inhibitor deficiency

A

Hereditary angioedema

65
Q

What bugs can pts with chronic granulomatous disease not kill?

A 
Catalase positive (SPACE)
Staph, Pseudomonas, Aspergilus, Candida, Enterobacteriaciae (klebsiella, seratia )
66
Q

What diseases can cause hyposplenia?

A

SLE, RA, IBD, GVDH, nephrotic syndrome

Sickle cell or hemoglobin-opathy

67
Q

What are asplenic individuals more susceptible to?

A 
Encapsulated organisms
S neuro
H flu
Neisseria
E coli
Salmonella
Klebsiella
68
Q

What s/sx occur with acute GVHD? (4)

A 
5-40 days
Erythroderma
Cholestatic hepatitis - abnormal LFTs
Diarrhea
Inc. susceptibility to infections
69
Q

What s/sx occur with chronic GVHD? (5)

A 
Sjogrens
SLE
Scleroderma
Lichen planus
Primary biliary cirrhosis
Infections

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