Clotting factor IX deficiency causes?

Appeared in: VSSC Nursing Officer - 2021

Explanation

A deficiency in clotting factor IX is the specific cause of Hemophilia B.
Christmas disease is the eponym (a name derived from a person) for Hemophilia B.
It is an X-linked recessive disorder, meaning it predominantly affects males, who inherit the faulty gene from their mothers.
Clinically, its symptoms are indistinguishable from Hemophilia A (Factor VIII deficiency), requiring specific laboratory assays for diagnosis.

Option B: This is a general term. While Factor IX deficiency is a type of hemophilia (Hemophilia B), 'Christmas disease' is the more specific and correct name for this particular condition.
Option C: Thalassemia is a genetic disorder related to the production of hemoglobin within red blood cells. It does not involve clotting factors.
Option D: Thrombocytopenia is a condition characterized by a low number of platelets (thrombocytes) in the blood. While it causes impaired clotting and bleeding, it is not a deficiency of a specific protein clotting factor like Factor IX.

Clinical Relevance

Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Hereditary Coagulation Disorders as background academic context rather than a clinical decision trigger.
Nurses must implement bleeding precautions for patients with hemophilia, such as using soft toothbrushes, avoiding razors with blades, and preventing falls.
Patient education is critical. The nurse must teach the patient and family to recognize signs of internal bleeding (e.g., joint pain/swelling, abdominal pain, headache) and to seek immediate medical attention.
Administration of replacement Factor IX concentrate is the primary treatment. The nurse must be proficient in IV administration and monitoring for adverse reactions.

How to Approach the Question

First, identify the key terms in the question: 'Clotting factor IX deficiency'. This points to a specific defect in the coagulation cascade.
Recall or look up the specific diseases associated with deficiencies of major clotting factors.
Evaluate the options. 'Christmas disease' is the specific eponym for Factor IX deficiency (Hemophilia B).
Analyze the distractors. 'Hemophilia' is a correct but general category. 'Thalassemia' and 'Thrombocytopenia' are different types of blood disorders (hemoglobinopathy and platelet deficiency, respectively).
Select the most specific and accurate answer. In medical multiple-choice questions, the most precise option is usually the correct one.

Concept Tested & Keywords

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E6 Pathology- ROBBINS & COTRAN PATHOLOGIC BASIS OF DISEASE 10TH Ed p. 671-673

E6 PATHOLOGY QUICK REVIEWBased on Harsh Mohan Textbook of PATHOLOGY p. 133-135

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