A pediatric nurse health educator provides a teaching session to the nursing staff regarding hemophilia. Which of the following information regarding this disorder would the nurse plan to include in the discussion?
A. Hemophilia is a Y linked hereditary disorder
B. Males inherit hemophilia from their fathers
C. Females inherit hemophilia from their mothers
D. Hemophilia A results from a deficiency of factor VIII
Correct Answer: D. Hemophilia A results from a deficiency of factor VIII
Hemophilia A results from a deficiency of factor VIII. Hemophilia B (Christmas disease) is a deficiency of factor IX. Both hemophilia A and B result from factor VIII and factor IX protein deficiency or dysfunction, respectively, and is characterized by prolonged and excessive bleeding after minor trauma or sometimes even spontaneously.
Option A: Hemophilia is inherited in a recessive manner via a genetic defect on the X-chromosome. Hemophilia is usually an inherited condition and is caused by the deficiency of clotting factors in the blood. It is almost always due to a defect or mutation in the gene for the clotting factor.
Option B: Males inherit hemophilia from their mothers. Female carrier mothers have a 50% chance of having affected males and a 50% chance of having carrier females. Females could also be affected if there is a complete inactivation of chromosome X through lionization, partial or complete absence of chromosome X such as in Turner Syndrome or if both parents carry the abnormal gene.
Option C: Females inherit the carrier status from their fathers. Both hemophilia A and B are inherited via an X-linked recessive pattern where 100% of females born from affected fathers will be carriers, and none of the males born will be affected.
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