Innate errors of immunity Flashcards
What does LAD-1 mean?
Leukocyte adhesion deficiency
What are the 2 major families of proteins involved in LAD?
Selectins, integrins
What are the 3 types of selectins?
E-selectin, L-selectin, P-selectin
What are the four steps of extravasation?
Rolling, adhesion, diapedesis, chemotaxis
At what stage of extravasation does the endothelium express selectins?
A) Rolling
B) Adhesion
C) Diapedesis
D) Chemotaxis
In LAD, there is a mutation in which gene?
A) CD21
B) CD29
C) CD18
D) CD14
CD18
What happens if there is a failure in the expression of the CD18 gene?
Adhesion cant happen
Clinical manifestations
-Delayed umbilical cord separation
-Omphalitis
-Leukocytosis >15,000
-Recurrent infections
-Gingivitis and periodontitis
Diagnosis
-Clinical manifestations
-Cytometric analysis of PMN with low CD18
-Blood count → elevated neutrophils
Treatment
Hematopoietic stem cell transplantation
What do phagocytes do to kill a pathogen?
Engulf pathogens and wrap them inside them forming a phagosome → lysosome fuses with fagosome forms phagolysosome → digestive enzymes destroys pathogens
NADPH undergoes which process to lose 2 of its electrons?
Oxidation
Which 2 forms of mutations are seen in CGD?
X linked, autosomal recessive
Which pathogens are difficult to destroy? And why?
Catalase positive pathogens, catalase breaks down hydrogen peroxide into oxigen and water before it damages the pathogen
In respiratory burst, which ROE destroys pathogens?
Hydrogen Peroxide (H2O2)
