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USMLE Step 1 Classic Clues Classic clues seen in clinical case scenarios in USMLE Step 1 questions.

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Old 10-16-2009
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Arrow Delayed Umbilical Sloughing

The title of this message could well constitute a buzzword. It's a very classical description of the autosomal recessive disorder Leukocyte Adhesion Deficiency.
Newborns presents with omphalitis and delayed sloughing with frequent bacterial infections.
However, abscesses do not occur as the neutrophils lack the ability to adhere to the endothelium because of a defective integrin molecule also called CD18.
More specifically the defective gene (on chromosome 21) encodes for beta2 integrin subunit that is found in all three integrin molecules namely LFA1, MAC1, and VLA4.
Once the diagnosis is suspected a series of immune tests carried until finally the CD18 is found missing.
One of the characteristics of the disease is the extreme leukocytosis seen in this patient as the neurtrophils are unable to leave the blood vessels.

It's important not confuse CD18 with CD34, CD34 is present on the endothelial surface of venules and it binds the L-selectin on T lymphocytes calling them to home in (circulating Naive lymphocytes either find their target antigen or go HOME via the process of homing into secondary lymphoid). organs).

Last edited by lee-usmle; 10-16-2009 at 08:36 PM.
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Old 10-16-2009
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Nice work Lee
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Old 10-25-2009
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Default thanks a lot

This is a very informative post and gives directions to those who are new to the whole USMLE thing
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