Immune Deficiencies


Bruton's

- no receptors on B cells, can't make antibodies

- encapsulated bacteria
- enterovirus enter through GI track

IgA Deficiency


CVID

- rare disorders in one disorders with variable degrees of deficiency

- immune dysregulation: autoimmune

DiGeorge



- similar to end stage HIV
- improve with age, may mature in other locations
- cleft palate, mandible: 3rd/4th pharyngeal pouches

Hyper-IgE



CMCC

- autoimmune T cell attack endocrine glands



SCID

- loss of entire adaptive immune system

- live in a bubble
- low CD3, low Ig levels

- can't do T cell count
- do TRECs

ADA

Ataxia




Hyper-IgM



Wiskott

- immunologic synapse: way T react to APC
- low or normal IgG/IgM. Elevated IgE/A

- bleeding/petechiae unique
Innate
LAD


- neutrophils can't get to stump
Chediak Higashi

- unique: oculocutaneous albinism
- melanocyte granules can't be transferred
CGD

- catalase breaks down H2O2
- catalase - can't break down H2O2
- Burkhoderia capacia




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