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1.5 CE Test Hours

Understanding the Nurse's Role in Managing Gaucher Disease

Contrada, Emily

AJN The American Journal of Nursing: June 2018 - Volume 118 - Issue 6 - p 43,44
doi: 10.1097/01.NAJ.0000534824.49739.45
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Figure.

Figure.

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Understanding the Nurse's Role in Managing Gaucher Disease

GENERAL PURPOSE:

To provide information about the epidemiology and pathophysiology of Gaucher disease and the latest advances in diagnosis and treatment.

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LEARNING OBJECTIVES/OUTCOMES:

After completing this educational activity, you should be able to

  • outline the epidemiology and pathophysiology of Gaucher disease.
  • identify recent advances in Gaucher disease screening and diagnosis.
  • delineate various treatment options for Gaucher disease.
  1. The incidence of lysosomal storage disorders ranges from one in
    1. 5,000 to one in 7,000 live births.
    2. 2,000 to one in 4,000 live births.
    3. 400 to one in 1,000 live births.
  2. For the patient in the case study, a definitive diagnosis of Gaucher disease was made following
    1. ultrasound of the liver.
    2. liver biopsy and blood enzyme assay.
    3. abdominal computed tomography scan with contrast.
  3. the Ashkenazi Jewish population, the incidence of Gaucher disease ranges from one in
    1. 5,000 to one in 7,000 live births.
    2. 2,000 to one in 4,000 live births.
    3. 400 to one in 1,000 live births.
  4. People who have Gaucher disease inherited nonworking copies of the GBA gene from
    1. their mother.
    2. their father.
    3. both parents.
  5. The catalyst for the multisystemic symptoms of Gaucher disease are lipid-laden cells found in several organs and tissues, including the
    1. pancreas.
    2. bone marrow.
    3. small intestine.
  6. Typical symptoms reported by patients with Gaucher disease include
    1. bloating.
    2. headache.
    3. palpitations.
  7. About 30% of patients with Gaucher disease have a splenic volume greater than how many multiples of normal?
    1. 5
    2. 10
    3. 15
  8. Thrombocytopenia can present in early childhood as
    1. fatigue.
    2. epistaxis.
    3. frequent infections.
  9. One of the 3 bones most often injured in patients with Gaucher disease is the
    1. femur.
    2. tibia.
    3. radius.
  10. With Gaucher disease, osteopenia is the result of osteoblastic dysfunction due to the accumulation of
    1. galsulfase.
    2. alglucerase.
    3. glucocerebroside.
  11. Risk factors for major bone complications in patients with Gaucher disease include
    1. anemia.
    2. hypothyroidism.
    3. diabetes mellitus.
  12. About 94% of Gaucher disease diagnoses in the Western hemisphere are of which type?
    1. type 1
    2. type 2
    3. type 3
  13. Type 3 Gaucher disease is characterized by
    1. microcephaly.
    2. slow neurologic progression.
    3. rapid brain stem deterioration.
  14. Enzyme testing for carrier detection of Gaucher disease
    1. is not recommended.
    2. correlates with disease severity.
    3. reliably distinguishes between disease types.
  15. In patients with Gaucher disease, GCase enzyme activity is typically what percentage of normal?
    1. 0% to 15%
    2. 15% to 30%
    3. 30% to 45%
  16. Which of the following is true about treatment of Gaucher disease?
    1. Early treatment slows nonneuronopathic disease progression.
    2. Experts agree on standardized treatment guidelines.
    3. Substrate reduction therapy breaks down glucocerebroside.
  17. Enzyme replacement therapy for patients with Gaucher disease reduces the incidence of
    1. neurotoxicity.
    2. cardiomyopathy.
    3. hepatosplenomegaly.
  18. Enzyme replacement therapy for the treatment of Gaucher disease carries the risk of antibody production, which, if high, can cause
    1. anaphylaxis.
    2. hemorrhage.
    3. septicemia.
  19. Enzyme replacement therapy infusions for patients with Gaucher disease are given
    1. over 30 minutes to an hour.
    2. every other month.
    3. for life.
  20. The most common adverse effects of substrate reduction therapy include
    1. constipation.
    2. headache.
    3. insomnia.
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