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peds > immunology > Flashcards

immunology Flashcards

1
Q

treatment for scid

what is transient

A

stem cell transplant

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2
Q

most common type of acid

A

x linked scid

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3
Q

chronic granulomatous disease

A

usually presents in infancy, defect in phagocytosis, boys more common and staph

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4
Q

IG deficiency symptoms

A

often asymptomatic but may manifest with sinusitis, chronic diarrhoea, respiratory infections . often associated with other automiinue disorders like celiac, iBS, immune thrombocytopenia

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5
Q

di George symptoms

A

heart defects- TOF
hypocalcemia- seizures (because of the parthyroid gland)
dysmorophic face, hypertelerosim and microagnathia
absent thymus
cleft palatte
freuwnet infections (viral and fungal, pneumocytsisi)

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6
Q

tx for primary immune deficiency

A

T cell - cotrimox to protect pCP

B - AZITHROMCYIN

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7
Q

WHOs at risk for encapsulated

A

lack of B cells and a splenism

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8
Q

medication given to baby born to hiv mother

A

zidovudine

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9
Q

tx for hiv mother

A

HAART as soon as finds out, and if viral load >50 CS

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10
Q

Baby be established to have HIV

A

> 18

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11
Q

LACK OF PHARGOCYTOSIS put you at risk for

A

STAPH AND STREP

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12
Q

EOSINOPHILLIC GRANULOMATOUSS

A

strongly with asthma and allergy will have eosinophilia vasculitis granulomas and PANNCA

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13
Q

weighers granuloamtos s/granulomatosis with polyangitits

A

abs affecting neutrophils commonly presents with recurring ENT IS A VASUCLITS,, biopsy of organs will show abscesses . most commonly affects lungs and kidneys

you get granulomas that fomr

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14
Q

what’s more dangerous T cell or B cell dfieicecy

A

T cell as they transform into B cell

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15
Q

Omenn syndrom

A

Opportunistic infections red skin hepatosplenomegaly alopecia enlarged lymph nodes

EOSINIOHILA
IG E - even though everthing selse is down

scid but problem seems more t cell

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16
Q

most common humreal defect

A

brutons

17
Q

When does Bruton present

A

around six months due to mothers antibodies

18
Q

chronic pneumonia is more common in

A

cell mediated

19
Q

signs of acid

A

absent/reudced lymphoid tissues
presents early infancy
pneumocystis carnini

20
Q

what pathology is di George of

A

T cell or T and bell cell

21
Q

di George features

A 
heart problems 
small jaw 
hypoplasia of thymus 
improves with age 
seizures due to hypocalcemica
22
Q

syndromes

A
  1. ataxia teleangiactasia
  2. WAS
    DI GEORGE
23
Q

WISCOTT ALDRICH

A

dermatitis
microthrombocytopenia
x linked

24
Q

which is more common humeral and cell mediated

A

humeral 50_75%

25
Q

signs of burtons and inherience

A

x linked recessive

frequent pnueumonias
skin rashes
tonsils (atrophic)
unpalpalble ln

26
Q

what is affected in burtons

A

the b cells! the precursors fail to mature so t cells are normally

27
Q

complications of CVID
WHAT IS IT

A

no immune memory so keep getting the sme disease again, no point of vaccinatating them

freuqunet pneumonias - bronchiactatsis

cancers

autoimmune disorders

28
Q

where is IGA found

A

mucous membranes
tears, saliva

29
Q

**

tx digeorge and whats the actual problem with di george

A

bone marrow transplant - not curative?
thymus transplantation -?
lifelone vitamin d and calcium

absecnec of t cells, 22,q 11 deleteion defect in the phaygeal piuch system

30
Q

what is hyper iG M and what kind of immunity is affected

A

high levels of iG m but low levels of the others there is a defect in the class switching

lack of CD40 ligand on T CELLS

x linked recessive like burtons

the t cells are the problem it seems as they insruct the switching from iG M to others

31
Q

diagnosis of WAS

A

thrombocytopenia and ALSO the small size of plt, always susepct in a young child male with thrombocytopneia

32
Q

LABS FOR WAS

A

thrombocytopenia + size
high levels of iG A and E
IG G /M can be normal or low

33
Q

another name for job syndrome

kostman syndrome

A

HYPER E immunglobulin syndrome

congential neutrophil disorder

34
Q

job syndform cause and features

A

AD condition
combined immunodeficiency (T +B cells)
deficiency in stat 3 (intracellular communication)

triad of staph infections(skin), pulmonary infections and atopi dermatitis

can also have a lot candiiada infections

35
Q

neutophil disorders featurs

A

FUNGAL INFECTIONS

NBT test (wbc should turn blue)

MPO

glucose 6 phosphate

peds (26 decks)

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