A 1 1/2 -year-old girl is sent to a children's - zarah

[ArchivalUser]

A 1 1/2 -year-old girl is sent to a children's hospital for evaluation following a nosebleed which was so severe as to require nasal packing and transfusion of platelet concentrates. When a blood sample had been drawn in the emergency room for serum chemistry studies, the local hospital laboratory had noted that the clot that formed was unusual in that it failed to retract. Peripheral blood smear obtained by finger puncture showed an appropriate number of normal-sized platelets, all of which were individual, without clumping. At the children's hospital, it was noted that the child's parents were cousins. Special platelet studies showed that the child's platelet's failed to aggregate with any physiologic aggregating agent, including a high concentration of exogenous ADP. Which of the following is the most likely diagnosis?

A. Bernard-Soulier syndrome


B. Chediak-Higashi syndrome


C. May-Hegglin anomaly


D. Thrombasthenia


E. Von Willebrand disease

[ArchivalUser]

A.....unlike E which is failure to adhere, this is failure to aggregate.

[ArchivalUser]

AAA

[ArchivalUser]

i dont think it s bernard soulier which shows thrombocytopenia with large platelets.
i think it s d
glanzmann thrambasthenia

[ArchivalUser]

If it is failure to aggregate.....parents =cousins....

could be GT.(rare autosomal recessive disorder whereby the production and assembly of the platelet membrane glycoprotein (GP) IIb-IIIa is altered, preventing the aggregation of platelets and subsequent clot formation. )

bcz

Bernard-Soulier syndrome: there is decreased expression of the glycoprotein Ib/IX/V complex on the surface of the platelets. This complex 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.

[ArchivalUser]

d...

[ArchivalUser]

D is correct...and the explanations by amatista & oblongata are right