Chapter 23: Hemophilia Flashcards
Which of the following statements accurately describes the genetic pattern of hemophilia?
A. Hemophilia A is an autosomal dominant disorder.
B. Hemophilia B is more common than hemophilia A.
C. Hemophilia is inherited in an X-linked recessive pattern.
D. Hemophilia is an autosomal recessive disorder.
C. Hemophilia is inherited in an X-linked recessive pattern.
Rationale: Hemophilia A and B are inherited in an X-linked recessive pattern, which means the disorder typically affects males, with females being carriers.
Which of the following is the most common type of hemophilia?
A. Hemophilia A.
B. Hemophilia B.
C. Hemophilia C.
D. Hemophilia D.
A. Hemophilia A.
Rationale: Hemophilia A (caused by a deficiency of factor VIII) is the most common type, accounting for 85% of hemophilia cases.
Which of the following are characteristics of hemophilia A? (SATA)
A. Caused by a deficiency of factor VIII.
B. Accounts for 85% of hemophilia cases.
C. More common in females than in males.
D. Associated with a deficiency of factor IX.
E. Occurs in 1 in 5000 male births.
A. Caused by a deficiency of factor VIII.
B. Accounts for 85% of hemophilia cases.
E. Occurs in 1 in 5000 male births.
Rationale: Hemophilia A is caused by a deficiency of factor VIII, occurs in 1 in 5000 male births, and accounts for 85% of hemophilia cases. It is more common in males and is not associated with factor IX deficiency.
Which of the following describes hemophilia B (Christmas disease)?
A. Caused by a deficiency in factor VIII.
B. Occurs in 1 in 5000 male births.
C. More common than hemophilia A.
D. Caused by a deficiency in factor IX.
D. Caused by a deficiency in factor IX.
Rationale: Hemophilia B is caused by a deficiency in factor IX and occurs in 1 in 25,000–30,000 male births. It is less common than hemophilia A.
Which of the following statements about the inheritance of hemophilia is correct?
A. Hemophilia A and B are inherited in an autosomal dominant pattern.
B. A male with hemophilia A will pass the disorder to all his sons.
C. Hemophilia is an X-linked recessive disorder.
D. Carrier females with hemophilia A can have hemophilia in their daughters.
C. Hemophilia is an X-linked recessive disorder.
Rationale: Hemophilia A and B are X-linked recessive disorders, meaning they primarily affect males. Carrier females typically do not show symptoms but can pass the gene to their children.
A 4-year-old boy is brought to the clinic for a check-up. His father has hemophilia A, and the mother has no family history of bleeding disorders. The child has not had any major bleeding episodes but has had easy bruising in the past few months. The nurse is discussing the potential risks of bleeding with the parents. What is the most likely explanation for this child’s condition?
A. The child inherited the disorder from both parents.
B. The child may have inherited the disorder from his father as an X-linked recessive trait.
C. The child inherited the condition from his mother, who is a carrier.
D. The child’s hemophilia is due to a new mutation, as the mother has no family history.
B. The child may have inherited the disorder from his father as an X-linked recessive trait.
Rationale: The child likely inherited the disorder from his father, as hemophilia is X-linked recessive. Since males inherit the X chromosome from their mother, the child’s hemophilia likely comes from his father, who carries the defective gene on his X chromosome.
In patients with hemophilia, the severity of bleeding is primarily related to:
A. The amount of clotting factor present.
B. The number of platelets in circulation.
C. The presence of an underlying viral infection.
D. The age of the patient.
A. The amount of clotting factor present.
Rationale: The severity of bleeding in hemophilia is primarily related to the amount of the deficient clotting factor (VIII or IX) present in the blood.
Which of the following are true regarding the inheritance of hemophilia? (SATA)
A. A female carrier of hemophilia can pass the gene to her sons.
B. Hemophilia affects only males.
C. Hemophilia is transmitted through an autosomal dominant inheritance pattern.
D. A father with hemophilia will pass the gene to all his sons.
E. Some children with hemophilia may have no family history of the disorder.
A. A female carrier of hemophilia can pass the gene to her sons.
B. Hemophilia affects only males.
E. Some children with hemophilia may have no family history of the disorder.
Rationale: A female carrier of hemophilia can pass the gene to her sons, and hemophilia almost exclusively affects males. Some children may inherit hemophilia due to a new mutation, even without a family history of the disorder.
A 7-year-old boy with hemophilia B is brought to the emergency department after falling off his bicycle. He is experiencing significant bleeding from a cut on his arm. His mother reports no family history of hemophilia, and the father is healthy. What is the most likely explanation for the child’s hemophilia?
A. The child inherited hemophilia B from his mother, who is a carrier.
B. The child’s hemophilia is due to a new mutation, as the father is healthy.
C. The child inherited the disorder from his father, who is a carrier of hemophilia B.
D. The child has an autoimmune disorder causing bleeding tendencies.
B. The child’s hemophilia is due to a new mutation, as the father is healthy.
Rationale: Since the father is healthy, the child most likely has hemophilia due to a new mutation. Hemophilia B is X-linked recessive, and the absence of a family history on both sides suggests a spontaneous mutation in the child’s X chromosome.
At what age do children with hemophilia typically begin to show symptoms, such as bleeding tendencies?
A. At birth
B. After 6 months of age
C. After 1 year of age
D. After 3 years of age
B. After 6 months of age
Rationale: Children with hemophilia often do not demonstrate symptoms until after 6 months of age, as they become more mobile and prone to injuries and falls.
A 2-year-old child with hemophilia presents to the clinic with swelling and pain in the knee joint. The mother reports that the child fell while walking and has been unable to move the leg without pain. What is the most likely cause of the child’s symptoms?
A. Bone fracture
B. Hemarthrosis
C. Osteoarthritis
D. Soft tissue injury
B. Hemarthrosis
Rationale: Hemarthrosis (bleeding into the joint space) is a common manifestation in children with hemophilia, especially in weight-bearing joints like the knees, ankles, and elbows. It results in pain, swelling, and limited motion.
Which of the following is a common site for bleeding in children with hemophilia?
A. Hip joint
B. Wrist joint
C. Knee joint
D. Shoulder joint
C. Knee joint
Rationale: Bleeding into the joint spaces of the knees, ankles, and elbows is common in children with hemophilia and can lead to limited motion and deformities over time.
Which of the following are common clinical manifestations of hemophilia? (SATA)
A. Easy bruising (ecchymosis)
B. Hemarthrosis (bleeding into joint spaces)
C. Spontaneous bleeding
D. Hematuria
E. Severe headache
A. Easy bruising (ecchymosis)
B. Hemarthrosis (bleeding into joint spaces)
C. Spontaneous bleeding
D. Hematuria
Rationale: Common manifestations of hemophilia include easy bruising, hemarthrosis, spontaneous bleeding, and hematuria. Severe headache is not a common manifestation unless intracranial bleeding occurs.
A 5-year-old boy with severe hemophilia is brought to the emergency department after a fall. His mother reports that he has had difficulty moving his right elbow and is in severe pain. On examination, the right elbow is swollen. Which of the following is the most likely complication?
A. Hemarthrosis
B. Fracture of the elbow
C. Dislocated elbow
D. Tendon rupture
A. Hemarthrosis
Rationale: Hemarthrosis, or bleeding into the joint space, is a common complication in children with hemophilia after trauma. The child’s difficulty moving the elbow and the swelling are consistent with this condition.
Which of the following complications is most commonly associated with severe hemophilia?
A. Intracranial bleeding
B. Subcutaneous hemorrhage
C. Bone fractures
D. Chronic diarrhea
A. Intracranial bleeding
Rationale: Intracranial bleeding occurs in 10% of patients with severe hemophilia and carries a high mortality rate. It is a serious complication that requires immediate attention.
Which of the following is a major concern in children with hemophilia who experience intracranial bleeding?
A. Increased risk of osteoarthritis
B. Mortality rate of 30%
C. Risk of hip dislocation
D. Development of blood clots
B. Mortality rate of 30%
Rationale: Intracranial bleeding occurs in 10% of patients with severe hemophilia and has a mortality rate of 30%. This makes it a critical complication that requires prompt medical intervention.
A 3-year-old boy with hemophilia is being prepared for dental surgery. The surgeon is concerned about bleeding risks. Which intervention is most appropriate to prevent excessive bleeding during the procedure?
A. Administering a blood transfusion before surgery
B. Using desmopressin (DDAVP) to release von Willebrand factor
C. Administering a clotting factor replacement therapy
D. Avoiding the use of local anesthetics
C. Administering a clotting factor replacement therapy
Rationale: Children with hemophilia are at risk for excessive bleeding during surgical procedures. Administering clotting factor replacement therapy before surgery is a standard practice to reduce this risk.
Which of the following diagnostic methods can be used to identify hemophilia before birth?
A. Genetic testing of family members
B. Prothrombin time (PT) test
C. Activated partial thromboplastin time (aPTT)
D. Chorionic villus sampling or amniocentesis
D. Chorionic villus sampling or amniocentesis
Rationale: Chorionic villus sampling or amniocentesis are prenatal diagnostic methods that can be used to detect hemophilia before birth.
Which laboratory finding is typically seen in children with hemophilia?
A. Elevated fibrinogen levels
B. Normal prothrombin time (PT)
C. Low levels of factor VIII or IX
D. High platelet count
C. Low levels of factor VIII or IX
Rationale: Laboratory tests in children with hemophilia typically show low levels of factor VIII or IX, as well as prolonged activated partial thromboplastin time (aPTT). Prothrombin time, thrombin time, fibrinogen, and platelet count are usually normal.
What is the primary goal of medical management for children with hemophilia?
A. To control bleeding by replacing the missing clotting factor
B. To prevent the need for blood transfusions
C. To reduce platelet count
D. To enhance the production of von Willebrand factor
A. To control bleeding by replacing the missing clotting factor
Rationale: The primary goal of medical management in hemophilia is to control bleeding by replacing the missing clotting factor, which helps prevent bleeding episodes.
A 4-year-old child with hemophilia A is diagnosed with a bleeding episode after a fall. The physician orders desmopressin (DDAVP) for treatment. Which of the following is the most likely reason for the physician’s choice of therapy?
A. To increase the production of factor IX
B. To increase the percentage of available factor VIII
C. To stimulate platelet aggregation
D. To prevent the release of thrombin
B. To increase the percentage of available factor VIII
Rationale: Desmopressin (DDAVP) stimulates the release of stored factor VIII from blood vessels, thereby increasing the percentage of available factor VIII, which is beneficial for patients with mild to moderate hemophilia A.
Which type of hemophilia is most likely to benefit from desmopressin (DDAVP) therapy?
A. Hemophilia A with severe bleeding episodes
B. Hemophilia B with normal factor IX levels
C. Hemophilia A with mild to moderate bleeding episodes
D. Hemophilia B with prolonged aPTT
C. Hemophilia A with mild to moderate bleeding episodes
Rationale: Desmopressin (DDAVP) is effective in some patients with mild to moderate hemophilia A, as it stimulates the release of stored factor VIII.
Which of the following is typically required for children with severe hemophilia to prevent serious bleeding episodes?
A. Immediate blood transfusions
B. Gene therapy
C. Prophylactic replacement therapy
D. Anticoagulant therapy
C. Prophylactic replacement therapy
Rationale: Children with severe hemophilia may be on a prophylactic regimen of replacement therapy, which helps prevent serious bleeding episodes and joint damage due to repeated hemarthrosis.
Which of the following are potential treatments for hemophilia? (SATA)
A. Recombinant factor VIII concentrates
B. Prophylactic therapy with clotting factor replacements
C. Platelet transfusions
D. Desmopressin (DDAVP) for hemophilia A
E. Blood thinners for clot management
A. Recombinant factor VIII concentrates
B. Prophylactic therapy with clotting factor replacements
D. Desmopressin (DDAVP) for hemophilia A
Rationale: Treatment options for hemophilia include recombinant factor VIII concentrates, prophylactic therapy with clotting factor replacements, and desmopressin (DDAVP) for hemophilia A. Platelet transfusions and blood thinners are not typically used for hemophilia management.
What is the current potential future treatment being explored for hemophilia?
A. Gene therapy for a possible cure
B. Increased use of blood thinners
C. Bone marrow transplants for factor production
D. Long-term use of desmopressin (DDAVP)
A. Gene therapy for a possible cure
Rationale: Gene therapy is being researched as a potential treatment for hemophilia, with the goal of eventually providing a cure.
A 6-year-old boy with hemophilia A is undergoing regular prophylactic therapy with factor VIII replacement. He experiences a minor injury at school and is brought to the clinic. What is the next appropriate step in managing the bleeding episode?
A. Administer additional factor VIII replacement therapy
B. Observe the injury without further intervention
C. Administer desmopressin (DDAVP)
D. Perform a platelet transfusion
A. Administer additional factor VIII replacement therapy
Rationale: For a child with hemophilia A, the next step in managing a bleeding episode is to administer additional factor VIII replacement therapy to control the bleeding.
Which of the following is the most appropriate course of treatment for a child with hemophilia B?
A. Factor IX concentrate replacement
B. Desmopressin (DDAVP) therapy
C. Prophylactic factor VIII infusion
D. Platelet inhibitors
A. Factor IX concentrate replacement
Rationale: Hemophilia B is caused by a deficiency of factor IX, and the most appropriate treatment is factor IX concentrate replacement. Desmopressin (DDAVP) is used for hemophilia A, not B.
Which of the following is the most important nursing diagnosis for a child with hemophilia?
A. Pain related to bleeding episodes
B. Mobility: Physical, Impaired, related to joint stiffness or contractures
C. Injury, Risk for, related to bleeding disorder
D. Family Processes, Interrupted, related to family role shift
C. Injury, Risk for, related to bleeding disorder
Rationale: The most important nursing diagnosis for a child with hemophilia is “Injury, Risk for,” related to the bleeding disorder, as this is the primary concern in hemophilia management.
What is a key aspect of the physiologic assessment for a child with hemophilia?
A. Focus only on the child’s nutritional intake
B. Assess only for signs of bleeding in the mouth
C. Limit assessment to neurologic examination
D. Observe for joint pain, swelling, or permanent deformity
D. Observe for joint pain, swelling, or permanent deformity
Rationale: Key aspects of the physiologic assessment include observing for joint pain, swelling, or permanent deformity, especially around the knees, elbows, ankles, and shoulders, as well as noting signs of bleeding or oozing blood.
A 5-year-old child with hemophilia is being assessed by the nurse. During the assessment, the nurse notes swelling and joint pain in the child’s right knee. The child has had a history of bleeding episodes, and the parents express concern about the child’s ability to walk. Which of the following should the nurse prioritize?
A. Administer pain medication and monitor vital signs
B. Assess for any other signs of bleeding, particularly in the abdominal region
C. Initiate factor replacement therapy and immobilize the joint
D. Offer emotional support to the parents and provide pain management
C. Initiate factor replacement therapy and immobilize the joint
Rationale: In a child with hemophilia, the priority is to initiate factor replacement therapy to control the bleeding, followed by immobilizing the joint to prevent further injury. Monitoring pain and providing emotional support are also important, but addressing the bleeding is the priority.
