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DOWNLOAD PDFLeukocyte Adhesion Deficiency is a rare disease inherited in an autosomal-recessive pattern.
The molecular defect in patients with LAD is a deficiency of the beta 2 integrin subunit called CD18. This subunit is responsible for making the protein LFA1 integrin, which allows neutrophils to make their way out of the bloodstream.
The molecular defect in patients with LAD is a deficiency of the beta 2 integrin subunit called CD18. This subunit is responsible for making the protein LFA1 integrin, which allows neutrophils to make their way out of the bloodstream.
The LFA1 integrin allows neutrophils to make their way out of the bloodstream by binding to the Ig family receptor ICAM on the surface of endothelial cells in the infected areas of the body.
The defect in neutrophil adhesion results in poor chemotaxis and phagocytosis, as well as elevated levels of neutrophils in the blood due to the inability to extravasate into tissue.
The defect in neutrophil adhesion results in poor chemotaxis and phagocytosis, causing individuals with this deficiency to suffer from recurrent bacterial infections that are often life-threatening.
It is important to note that the infections are non-pyogenic because neutrophils cannot migrate to the site of infection.
Delayed umbilical cord separation beyond the normal range of 3-45 days can raise suspicion of Leukocyte adhesion deficiency because this is common in infants with LAD.
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