LAD 1 Flashcards
1
Q
What are the 3 types of of selectins
A
E-selectin, L-selectin, P-selectin
2
Q
What do integrins do?
A
Firm adhesion to the endothelium
3
Q
For what is β chain (CD18) required for?
A
Surface expression of the CD11 chains. Leukocyte adhesion to endothelium
4
Q
In what step of extravasation is selectins involved?
A
Rolling
5
Q
What it the pathophysiology of LAD?
A
Mutation in a gene that codifies the molecule that interact with phagocytes and endothelial cells
Gene CD18 in chromosome 21q22
6
Q
Most common clinical manifestations
A
-Delayed umbilical cord separation
-Omphalitis
-Leukocytosis
-Gingivitis
-Periodontitis
7
Q
Treatment
A
Hematopoietic stem cell transplantation
