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Author4 Posts
  #1

Differentiate chediak higashi and bernard soulier ?

  #2

chediak higashi---autosomal recessive disorder
Mutations in the CHS1 gene. The primary defect is in intracellular granules, that results in chronic infection, decreased pigmentation in skin and eyes, neurological disease, and early death.

bernard soulier----In it there is the absent or decreased expression of the glycoprotein Ib/IX/V complex on the surface of the platelets. it is the receptor for von Willebrand factor (vWF), and the result of decreased expression is deficient binding of vWF to the platelet membrane at sites of vascular injury, resulting in defective platelet adhesion

___________________
hi how r u

  #3

Chediak higashi- remember 5 'N's

Neutrohil chemotaxis defective
No pigmentation in eye and skin(oculocutaneous albinism)
Neuropathy
Neutropenia
Nystagmus
Neutrophils has large cytoplasmic granules
Notorious Bugs- staph & strepto-recurrent infections

Bernard soulier- defective platlet adhesion- Bad

dmnz :wink:

  #4

you r perfect dmnz smiling face







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