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Discussion Starter · #1 ·
What about the defect ..i.e pathogenesis in Chediak Higashi Syndrome

plz anyone can explain....??
 

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It is a disease with impaired bacteriolysis due to failure of phagolysosome formation. As a result of disordered intracellular trafficking there is impaired lysosome degranulation with phagosomes, so phagocytosed bacteria are not destroyed by the lysosome's enzymes.

In addition, secretion of lytic secretory granules by cytotoxic T cells is also affected.

The disease is characterised by large lysosome vesicles in phagocytes (neutrophils), which thus have poor bactericidal function, leading to susceptibility to infections, abnormalities in nuclear structure of leukocytes, anemia, and hepatomegaly.

Also have a look here
http://www.usmle-forums.com/usmle-step-1-mnemonics/5537-chediak-higashi-mnemonic.html
 

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Discussion Starter · #3 ·
It is a disease with impaired bacteriolysis due to failure of phagolysosome formation. As a result of disordered intracellular trafficking there is impaired lysosome degranulation with phagosomes, so phagocytosed bacteria are not destroyed by the lysosome's enzymes.

In addition, secretion of lytic secretory granules by cytotoxic T cells is also affected.

The disease is characterised by large lysosome vesicles in phagocytes (neutrophils), which thus have poor bactericidal function, leading to susceptibility to infections, abnormalities in nuclear structure of leukocytes, anemia, and hepatomegaly.

Also have a look here
http://www.usmle-forums.com/usmle-step-1-mnemonics/5537-chediak-higashi-mnemonic.html
V.THANKFUL
THAT WAS greatly helpful....:)
 
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