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Hemophilia

A 7-year-old child with hemophilia A is admitted to the pediatric unit after a fall that resulted in a suspected intracranial hemorrhage. Which nursing action should be the immediate priority?

The nurse is caring for a child with hemophilia A who sustained a head injury from a playground fall.
해설
In hemophilia A, factor VIII deficiency leads to impaired blood clotting. With suspected intracranial hemorrhage, immediate factor replacement is critical to prevent life-threatening bleeding progression.

This question assesses critical thinking about emergency management of hemophilia complications. Hemophilia A is an X-linked recessive bleeding disorder caused by factor VIII deficiency, occurring in approximately 1 in 5,000 male births. When a child with hemophilia sustains head trauma and intracranial hemorrhage is suspected, this is a life-threatening emergency requiring immediate intervention.

Pathophysiologically, factor VIII deficiency results in inadequate coagulation cascade function. Normal hemostasis requires both primary hemostasis (platelet plug formation) and secondary hemostasis (fibrin clot formation via the coagulation cascade). In hemophilia A, the intrinsic pathway is impaired, leading to prolonged bleeding time and failure to form adequate clots.

Intracranial hemorrhage in hemophilia patients can progress rapidly because stable clot formation cannot occur. The enclosed space of the skull means even small amounts of bleeding can cause dangerous increases in intracranial pressure, which can lead to brain herniation and death. Immediate factor VIII replacement is essential to restore coagulation function and prevent bleeding progression.

Factor VIII concentrate administration is the definitive treatment addressing the underlying pathophysiology. The goal is to achieve factor VIII levels of 80-100% of normal for life-threatening bleeding episodes. This typically requires an initial loading dose followed by maintenance doses to maintain adequate factor levels during the critical period.

Neurological assessment, ice application, and positioning are important supportive measures but do not address the fundamental problem of impaired hemostasis. Without factor replacement, bleeding will continue regardless of other interventions, making factor VIII administration the absolute priority in this emergency.
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