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Pediatrics General bead378e

Characteristic lab findings of hemophilia A are –a) ↑ PTb) ↑ aPTTc) X–linked recessived) Presence of 30% of factor level express the diseasee) Increased bleeding time

A
a
B
c
C
ac
D
bc
High-Yield Explanation
Hemophilia A                                                                                                                      Hemophilia A is due to deficiency offactor VIII. Hemophilia 'A' is inherited as an X-linked recessive trait. Factor VIII is an intrinsic pathway component required for activation of factor X. Clinical manifestations are due to defect in coagulation system - Large post-traumatic ecchymoses or hematoma Prolonged bleeding after a laceration or any form of surgical procedure. Bleeding into weight bearing joints. Petechiae are characteristically absent (in contrast to platelet dysfunction where bleeding occurs from small vessels of the skin and mucous membrane, e.g. petechiae). Laboratory findings PIT   Normal PT  Normal BT      Normal platelet counts                 Remember Hemophilia B (Christmas disease) is due to deficiency of factor IX Hemophilia B has clinical features and laboratory findings similar to haemophilia A.

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