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Peds Clerkship > Peds Immunology > Flashcards

Peds Immunology Flashcards

1
Q

X-linked agammaglobulinemia

A

dec B-cells with
dec immunoglobulins

DDx CVID which is normal B-cells

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2
Q

Common variable immunodeficiency

A

normal B-cells with
dec immunoglobulins

ddx. X-linked agammaglobulinemia which is dec B-cells

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3
Q

IgA deficiency

A

normal B cells

dec IgA

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4
Q

Hyper IgM

A

Normal B-cells
dec IgA
dec IgG
Inc IgM

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5
Q

IgG def

A

normal B cells

dec IgG

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6
Q

Hyper IgM defect

A

defect in CD40 ligand

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7
Q

Hyper IgM inheritance pattern

A

X-linked

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8
Q

Doll-like face (fat cheeks)
thin extremities
protuberant abdomen (from enlarged liver)

lactic acidosis and low sugar

A

Von-Gierkes (Glucose 6 Phosphate deficiency)

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9
Q

Acid Maltase Deficiency

A

Pompes Disease

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10
Q

Pompes

A

Acid maltase Glucose 1,4 glucosidase

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11
Q

Pompes presentation

A

macroglossia and floppy baby and heart failure

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12
Q

Type 3 glycogen storage Cori’s

A

just like type 1 Von-Geirkes but labs have elevated LFTs and ketoacidosis

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13
Q

Type 4 McArdles?

A

Progressive cirrhosis of the liver

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14
Q

recurrent sinus infx from H Infl and Strep Pneumo

Giardia infx from IgA deficiency

A

B-Lymphocyte maturation problem

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15
Q

B-Lymphocyte problem

A

Loss of IgA

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16
Q

problem in Chronic Granulomatous Disease

A

impaired oxidative metabolism

defect in NADPH-oxidase

susceptible to catalase organisms (Aspergillus, Staph, Kleb, Seratia, Burkholderia)

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17
Q

complement deficiency infx

A

Gonococcal and meningococcal

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18
Q

T-cell deficiency infx

A

viral and fungal infx

(DiGeorge) thymic aplasia

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19
Q

SCID deficiency

A

Adenosine deaminase resulting in B and T cell deficiencies

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20
Q

serum sickness is what hypersensitivity response

A

Type 3

21
Q

what may indicate serum sickness

A

fever,
urticaria (hives)
polyarthralgia

22
Q

which hypersensitivity appears immediately after exposure

A

Type 1

23
Q

Non-Hodgkin Lymphoma

A

unexplained fever
weight loss
intestinal obstruction

24
Q

unexplained fever
weight loss
intestinal obstruction

A

Non-Hodgkin Lymphoma

25
Q

chronic granulomatous disease cause

A

dysfunction in NADPH oxidase. Susceptible to catalase positive organisms

lymphadenopathy
hypergammaglobulinemia
hepatomegally & spleenomegaly
AOCD
chronic diarrhea and dermatitis
26
Q

How to diagnose CGD

A

Nitroblue Tetrazolium slide test
Flow cytometry
Cytochrome C reduction

27
Q

Treatment for CGD

A

TMP-SMX
Gamma-Interferon

All of this three times a week

28
Q

Chediak-Higashi

A

recurrent infections from decreased degranulation and chemotaxis

Neutropenia and Giant Lysosomes
Oculocutaneous Albinism

29
Q

Chediak-Higashi Tx

A

TMP-SMX

daily ascorbic acid

30
Q

Leukocyte Adhesion Defect

Why and How does it present

A

Neutrophilia without polymorphs in the pus (failure to localize from defective tethering and adhesion)

Delayed separation of umbilical cord, necrotic skin lesions, severe gingivitis

31
Q

Hyper-IgE

A

recurrent staph infections in skin and lungs
chronic pruritic dermatitis
(elevated IgE too)

32
Q

Kawasaki complication

A

aseptic meningitis

33
Q

Kawasaki prodrome?

tx?

A 
conjunctivitis
lympadenopathy
desquamation of hands
strawberry tongue
coronary artery anyeurism

Aspirin and IVIG

34
Q

what is Reyes syndrome

A

?

35
Q

HSP

A

2/4

36
Q

What is HSP complication

A

intussuception

37
Q

sail sign on x-ray

A

thymus… if missing… think DiGeorge

38
Q

anterior mediastinal mass

A

thymoma and lymphoma

39
Q

residual thymus tissue associated with what

A

may convert to thymoma in myasthenia gravis

40
Q

Burkitt Lymphoma

A

Rapidly growing B-Cell tumor, may present as intussuception

41
Q

most common SCID infections

A

Pneumocystis Jiroveci,
Candida,
parainfluenza,
HSV

42
Q

compliment deficiency results in what infx

A

encapsulated bacteria

43
Q

DiGeorge presentation and embryology

A

failure of 3 & 4th pouches = aplastic thymus and no parathyroids = hypocalcemic seizures

44
Q

IgA deficiency and transfusions

A

may be predisposed to transfusion reactions due to IgA antibodies

45
Q

X-linked agammaglobulinemia

A

Bruton’s

lacks B-cells and therefore all classes of Ig

46
Q

Bruton’s infections

A

Sinopulminary and Pseudomonas are common

47
Q

Hyper IgM infections

A

Giardia and recurrent sinopulminary infections

IgA def. may also have Giardia infx

48
Q

agammaglobulinemia diagnostic test

A

flow cytometry

Peds Clerkship (17 decks)

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