Conditions

Question 1

Heriditory angiodema

Answer 1

• C1 esterase inhibitor reduction/defect leading to C1activation
• can have abdominal symptoms
• non pitting, non urticarial
• does not respond to anaphylaxis treatment
• Rx with plasma derived or recombinant C1 or kalkekrien inhibitor

Question 2

Hyper IgE syndrome

Answer 2

very rare
staphyloccocal infections
eczema like skin reactions
penumonia

Question 3

Wisket Aldrich Syndrome

Answer 3

primary immunodeficiency disorder
Thrombocytopaenia
chronic infections
eczema
Low IgG/IgM
can be pale,

Question 4

Chediak hidashi

Answer 4

pale, red hair
AR
immunodeficiency syndrome - abnormal intracellular protein transport
fatal in childhood - infection/accelerated lymphoma
progressive neurological dysfunction - ataxia, neuropathy
photosensitivity
bleeding diathesis
hepatosplenomegaly