Hemophilia A is characterized by the deficiency of:

Appeared in: BHU NO-2019

Explanation

Hemophilia A is a genetic bleeding disorder resulting from a deficiency of coagulation Factor VIII.
Factor VIII is a crucial protein for the intrinsic pathway of the blood coagulation cascade.
Its deficiency leads to impaired blood clotting and an increased risk of prolonged bleeding.
The condition is inherited in an X-linked recessive pattern, primarily affecting males.

Option A: A deficiency in von Willebrand factor (vWF) causes von Willebrand disease, not Hemophilia A. Although vWF stabilizes Factor VIII in circulation, its primary deficiency is a distinct disorder.
Option C: A deficiency in Factor IX is characteristic of Hemophilia B, also known as Christmas disease.
Option D: A deficiency or defect in the glycoprotein IIb/IIIa receptor on platelets leads to Glanzmann thrombasthenia, a disorder of platelet aggregation.

Clinical Relevance

Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Pathophysiology of Hemophilia A as background academic context rather than a clinical decision trigger.
Nurses must implement bleeding precautions for patients with Hemophilia A, such as using soft-bristled toothbrushes, avoiding intramuscular injections, and applying prolonged pressure to puncture sites.
Patient education is critical, focusing on recognizing signs of internal bleeding (e.g., joint pain, abdominal swelling) and the importance of avoiding contact sports and activities with a high risk of trauma.
The primary treatment is replacement therapy with Factor VIII concentrates. Nurses are responsible for administering these infusions, often teaching patients and families how to do so at home for prophylactic or on-demand treatment.

How to Approach the Question

First, identify the key terms in the question: 'Hemophilia A' and 'deficiency'. This indicates the question is asking for the specific cause of a known disease.
Recall the different types of hemophilia and their associated factor deficiencies. Hemophilia A is linked to Factor VIII, and Hemophilia B is linked to Factor IX.
Evaluate the given options based on this knowledge.
Eliminate options related to other bleeding disorders: von Willebrand factor (von Willebrand disease), and gpIIb/IIIa (platelet disorders like Glanzmann thrombasthenia).
Select the option that correctly links Hemophilia A with its specific factor deficiency.

Concept Tested & Keywords

Q7zoBLbeau7L5sjJsiYcI6

E6 PATHOLOGY QUICK REVIEWBased on Harsh Mohan Textbook of PATHOLOGY p. 134-136

E6 Medicine Davidson Principles Practice 24e p. 991-993

E6 Pathology- ROBBINS & COTRAN PATHOLOGIC BASIS OF DISEASE 10TH Ed p. 670-672

Practise the full BHU NO-2019

Attempt every question from this paper in a timed mock, then review the full solution for each one.