NVS Staff Nurse - 2019
Medical & Surgical Nursing
Easy
Hemophilia B is the deficiency of?
Appeared in: NVS Staff Nurse - 2019
Explanation
- Hemophilia B, also known as Christmas disease, is specifically caused by a deficiency or dysfunction of coagulation Factor IX.
- This deficiency disrupts the intrinsic pathway of the coagulation cascade, which is essential for normal blood clotting.
- It is an X-linked recessive disorder, which explains why it is almost exclusively seen in males.
- Clinically, it is indistinguishable from Hemophilia A (Factor VIII deficiency) without specific factor assays, but the treatment is different.
Why Other Options Were Wrong
- Option A: A deficiency of Factor XI causes Hemophilia C (Rosenthal syndrome), not Hemophilia B.
- Option C: Factor X is part of the common pathway of coagulation. Its deficiency is a separate, rare autosomal recessive disorder that would prolong both PT and aPTT.
- Option D: Factor VII is part of the extrinsic pathway. Its deficiency is a rare bleeding disorder that results in a prolonged Prothrombin Time (PT) but a normal activated Partial Thromboplastin Time (aPTT).
Related Visual
Clinical Relevance
- Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Identification of the specific clotting factor deficiency in Hemophilia B as background academic context rather than a clinical decision trigger.
- Nurses must be vigilant in monitoring patients with hemophilia for signs of bleeding, especially spontaneous joint bleeding (hemarthrosis) and muscle hematomas, which are hallmarks of the disease.
- Patient education is a critical nursing role, including teaching the patient and family how to administer factor concentrates, recognize bleeds early, and avoid medications like aspirin and NSAIDs that increase bleeding risk.
- Accurate diagnosis is vital because treatment is factor-specific. A patient with Hemophilia B needs Factor IX concentrate, and Factor VIII concentrate (for Hemophilia A) would be ineffective.
How to Approach the Question
- First, identify the key term in the question: 'Hemophilia B'. This is a specific medical diagnosis.
- The question asks for the 'deficiency' associated with this diagnosis. This is a direct recall question testing your knowledge of hematological disorders.
- Mentally review the common types of hemophilia: Hemophilia A (most common), Hemophilia B, and Hemophilia C.
- Recall the specific factor associated with each: A is Factor VIII, B is Factor IX, and C is Factor XI. A useful mnemonic is to remember them in numerical order (A=8, B=9).
- Scan the options to find the factor corresponding to Hemophilia B, which is Factor IX.
- Confirm your answer by considering why the other options are incorrect. Factor XI is Hemophilia C, while Factors X and VII are associated with other, rarer clotting disorders.
Concept Tested & Keywords
- Concept Tested: Identification of the specific clotting factor deficiency in Hemophilia B.
- Stem keywords: Hemophilia B, deficiency
- Lead-in keywords: is the
- Negative lead-in flag: false
Question ID
Qba2VBaSs-DufYh-4dk91w
Reference Book
E6 PATHOLOGY QUICK REVIEWBased on Harsh Mohan Textbook of PATHOLOGY p. 133-135
E6 Nelson Textbook of Pediatrics(2024) — Volume 2 p. 827-829
Practise the full NVS Staff Nurse - 2019
Attempt every question from this paper in a timed mock, then review the full solution for each one.
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