immunology Flashcards
treatment for scid
what is transient
stem cell transplant
most common type of acid
x linked scid
chronic granulomatous disease
usually presents in infancy, defect in phagocytosis, boys more common and staph
IG deficiency symptoms
often asymptomatic but may manifest with sinusitis, chronic diarrhoea, respiratory infections . often associated with other automiinue disorders like celiac, iBS, immune thrombocytopenia
di George symptoms
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)
tx for primary immune deficiency
T cell - cotrimox to protect pCP
B - AZITHROMCYIN
WHOs at risk for encapsulated
lack of B cells and a splenism
medication given to baby born to hiv mother
zidovudine
tx for hiv mother
HAART as soon as finds out, and if viral load >50 CS
Baby be established to have HIV
> 18
LACK OF PHARGOCYTOSIS put you at risk for
STAPH AND STREP
EOSINOPHILLIC GRANULOMATOUSS
strongly with asthma and allergy will have eosinophilia vasculitis granulomas and PANNCA
weighers granuloamtos s/granulomatosis with polyangitits
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
what’s more dangerous T cell or B cell dfieicecy
T cell as they transform into B cell
Omenn syndrom
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
most common humreal defect
brutons
When does Bruton present
around six months due to mothers antibodies
chronic pneumonia is more common in
cell mediated
signs of acid
absent/reudced lymphoid tissues
presents early infancy
pneumocystis carnini
what pathology is di George of
T cell or T and bell cell
di George features
heart problems small jaw hypoplasia of thymus improves with age seizures due to hypocalcemica
syndromes
- ataxia teleangiactasia
- WAS
DI GEORGE
WISCOTT ALDRICH
dermatitis
microthrombocytopenia
x linked
which is more common humeral and cell mediated
humeral 50_75%
signs of burtons and inherience
x linked recessive
frequent pnueumonias
skin rashes
tonsils (atrophic)
unpalpalble ln
what is affected in burtons
the b cells! the precursors fail to mature so t cells are normally
complications of CVID
WHAT IS IT
no immune memory so keep getting the sme disease again, no point of vaccinatating them
freuqunet pneumonias - bronchiactatsis
cancers
autoimmune disorders
where is IGA found
mucous membranes
tears, saliva
**
tx digeorge and whats the actual problem with di george
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
what is hyper iG M and what kind of immunity is affected
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
diagnosis of WAS
thrombocytopenia and ALSO the small size of plt, always susepct in a young child male with thrombocytopneia
LABS FOR WAS
thrombocytopenia + size
high levels of iG A and E
IG G /M can be normal or low
another name for job syndrome
kostman syndrome
HYPER E immunglobulin syndrome
congential neutrophil disorder
job syndform cause and features
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
neutophil disorders featurs
FUNGAL INFECTIONS
NBT test (wbc should turn blue)
MPO
glucose 6 phosphate
