Monitoring Adult Thalassemia Patients
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Questions and Answers

What hormone levels should be monitored in patients with hypogonadism?

  • Adrenaline, insulin, glucagon, and thyroxine
  • Testosterone, estrogen, insulin, and glucagon
  • FSH, LH, testosterone, and estrogen (correct)
  • PTH, calcium, phosphate, and magnesium

    • How often should patients on chelation therapy have their hormone levels monitored?

  • Every 12 months
  • Every 24 months
  • Every month (correct)
  • Every 2 weeks

    • What test is used to diagnose diabetes mellitus?

  • Electrolyte panel
  • Liver function test
  • Fasting glucose or oral glucose tolerance test (correct)
  • Complete blood count

    • What is the recommended frequency for performing liver MRI in patients with frequent transfusions or high serum ferritin levels?

    <p>At baseline only</p> Signup and view all the answers

    What is the recommended frequency for performing cardiac MRI in patients with iron overload?

    <p>Only if LIC is ≥10 mg/g dry weight</p> Signup and view all the answers

    What hormone levels should be monitored in patients with hypothyroidism?

    <p>Free thyroxine (FT4) and TSH</p> Signup and view all the answers

    What is the recommended frequency for performing hormone assessments in patients not receiving iron chelation therapy?

    <p>Every 24 months</p> Signup and view all the answers

    What is the recommended frequency for performing hormone assessments in patients receiving iron chelation therapy?

    <p>Every 12 months</p> Signup and view all the answers

    What is the recommended frequency for performing exams in patients with evidence of iron overload?

    <p>Annually</p> Signup and view all the answers

    What electrolytes should be monitored in patients with hypoparathyroidism?

    <p>Calcium, phosphate, and magnesium</p> Signup and view all the answers

    What is the primary purpose of monitoring adult patients with α- or β-thalassemia from the time of diagnosis?

    <p>To track disease progression over time</p> Signup and view all the answers

    What is recommended to be performed at every visit (Q3 months) for adult patients with α- or β-thalassemia?

    <p>Complete blood count, liver and kidney function test, and physical exam</p> Signup and view all the answers

    Which specialist should a patient with abnormal assessments be referred to?

    <p>Endocrinologist or bone health specialist</p> Signup and view all the answers

    What laboratory test is recommended to assess iron overload in adult patients with α- or β-thalassemia?

    <p>Liver MRI for LIC</p> Signup and view all the answers

    What does an elevated TRV indicate?

    <p>Pulmonary hypertension</p> Signup and view all the answers

    What is recommended to assess cardiac function in adult patients with α- or β-thalassemia?

    <p>ECHO, including TRV</p> Signup and view all the answers

    Which of the following is a symptom of hemolytic crisis?

    <p>All of the above</p> Signup and view all the answers

    What is recommended to assess osteoporosis and bone disease in adult patients with α- or β-thalassemia?

    <p>DEXA scan</p> Signup and view all the answers

    What is the frequency of routine assessments recommended for adult patients with α- or β-thalassemia?

    <p>Every 3 months</p> Signup and view all the answers

    What is the purpose of referring a female to a reproductive endocrinologist?

    <p>To assess for fertility and need for reproductive assistance</p> Signup and view all the answers

    Which test is used to assess liver iron concentration?

    <p>LIC</p> Signup and view all the answers

    What is the purpose of collecting a baseline assessment for every patient with α- or β-thalassemia?

    <p>To track disease progression over time</p> Signup and view all the answers

    What is recommended to assess extramedullary hematopoiesis in adult patients with α- or β-thalassemia?

    <p>MRI C/T/L spine with contrast</p> Signup and view all the answers

    What is the purpose of monitoring for arrhythmias and abnormal Holter test results?

    <p>To refer the patient to a cardiologist</p> Signup and view all the answers

    What is the complication that can commonly develop from infections resulting in fever?

    <p>Worsening anemia</p> Signup and view all the answers

    What is the purpose of performing a physical exam on adult patients with α- or β-thalassemia?

    <p>To assess for splenomegaly</p> Signup and view all the answers

    Which hormone is related to reproductive health?

    <p>FSH</p> Signup and view all the answers

    What is recommended to assess endocrinopathy in adult patients with α- or β-thalassemia?

    <p>25-hydroxyvitamin D</p> Signup and view all the answers

    What is the name of the test used to assess bone health?

    <p>DEXA</p> Signup and view all the answers

    When should a patient be counseled on the risk of worsening anemia?

    <p>When infections resulting in fever occur</p> Signup and view all the answers

    What is a potential complication of thrombocytosis following splenectomy?

    <p>Thrombosis and vascular events</p> Signup and view all the answers

    Why should patients with chronic, severe iron overload or hepatitis receive a baseline AFP, ultrasound, and FibroScan?

    <p>To monitor for liver fibrosis, cirrhosis, and HCC</p> Signup and view all the answers

    What is the recommended frequency for skin inspection in patients with leg ulcers?

    <p>At every visit</p> Signup and view all the answers

    What is a common symptom of Deep Vein Thrombosis (DVT)?

    <p>Swelling and tenderness to touch in the involved leg</p> Signup and view all the answers

    Why is annual screening for HCV, HBV, and HIV recommended in certain patients?

    <p>For patients who have received blood transfusions in the previous 12 months</p> Signup and view all the answers

    What is a common symptom of Pulmonary Embolism (PE)?

    <p>Dyspnea followed by chest pain</p> Signup and view all the answers

    Why should patients with splenectomy follow vaccination recommendations?

    <p>To prevent infections</p> Signup and view all the answers

    What is a common symptom of choledocholithiasis?

    <p>Upper right quadrant or epigastric pain, nausea, vomiting, and worsening jaundice</p> Signup and view all the answers

    What is a recommended laboratory test for patients with clinical suspicion of liver dysfunction?

    <p>Liver function tests</p> Signup and view all the answers

    What guidelines should be referred to for infection risk and course of action during emergency-related febrile events in splenectomized individuals?

    <p>CDC guidelines</p> Signup and view all the answers

    What hormones should be monitored in patients with hypogonadism?

    <p>FSH, LH, testosterone, and estrogen</p> Signup and view all the answers

    What test is used to diagnose hypothyroidism?

    <p>Free thyroxine (FT4) and TSH</p> Signup and view all the answers

    What electrolytes should be monitored in patients with hypoparathyroidism?

    <p>PTH, calcium, phosphate, and magnesium</p> Signup and view all the answers

    How often should liver MRI be performed in patients with frequent transfusions or high serum ferritin levels?

    <p>At baseline and every 24 months or every 12 months with abnormality</p> Signup and view all the answers

    What is the recommended frequency for performing hormone assessments in patients on chelation therapy?

    <p>Every 12 months</p> Signup and view all the answers

    What is the recommended frequency for performing cardiac MRI in patients with iron overload?

    <p>Every 24 months or every 12 months with abnormality</p> Signup and view all the answers

    How often should patients on deferiprone have their iron levels monitored?

    <p>Every 2 weeks</p> Signup and view all the answers

    What is the recommended frequency for performing exams in patients with evidence of iron overload?

    <p>Annually</p> Signup and view all the answers

    What is the recommended frequency for performing hormone assessments in patients not on chelation therapy?

    <p>Every 24 months</p> Signup and view all the answers

    What is the primary purpose of monitoring adult patients with α- or β-thalassemia from the time of diagnosis?

    <p>To detect and manage complications</p> Signup and view all the answers

    What should a patient be referred to when ECHO or MRI indicates low ejection fraction or evidence of diastolic dysfunction?

    <p>A cardiologist</p> Signup and view all the answers

    What should a patient be referred to when monitoring indicates abnormal hormone levels?

    <p>An endocrinologist or bone health specialist</p> Signup and view all the answers

    What is the purpose of performing a DEXA scan in adult patients with α- or β-thalassemia?

    <p>To assess osteoporosis and bone disease</p> Signup and view all the answers

    What should a patient be referred to when monitoring indicates arrhythmias or abnormal Holter test results?

    <p>A cardiologist</p> Signup and view all the answers

    What is the complication that can commonly develop from infections resulting in fever?

    <p>Worsening anemia</p> Signup and view all the answers

    What is the purpose of monitoring for hemolytic crisis?

    <p>To detect symptoms of worsening fatigue, jaundice, and/or dark-colored urine</p> Signup and view all the answers

    What should a female patient be referred to when assessing for fertility and need for reproductive assistance?

    <p>A reproductive endocrinologist</p> Signup and view all the answers

    What is the purpose of performing an ECHO in adult patients with α- or β-thalassemia?

    <p>To assess cardiac function</p> Signup and view all the answers

    What should a patient be referred to when monitoring indicates evidence of pulmonary hypertension?

    <p>A cardiologist</p> Signup and view all the answers

    What is the purpose of monitoring for liver iron concentration?

    <p>To assess liver iron overload</p> Signup and view all the answers

    What is the recommended frequency of routine assessments for adult patients with α- or β-thalassemia?

    <p>Every 3 months (Q3 months)</p> Signup and view all the answers

    What laboratory tests are recommended to assess iron overload in adult patients with α- or β-thalassemia?

    <p>Serum ferritin and liver MRI for LIC</p> Signup and view all the answers

    What test is used to assess cardiac function in adult patients with α- or β-thalassemia?

    <p>ECHO, including TRV</p> Signup and view all the answers

    What is recommended to assess osteoporosis and bone disease in adult patients with α- or β-thalassemia?

    <p>DEXA scan</p> Signup and view all the answers

    What is the purpose of collecting a baseline assessment for every patient with α- or β-thalassemia?

    <p>To track disease progression over time and allow healthcare providers to make informed decisions about treatment.</p> Signup and view all the answers

    What is recommended to assess extramedullary hematopoiesis in adult patients with α- or β-thalassemia?

    <p>MRI C/T/L spine with contrast</p> Signup and view all the answers

    Why are patients with chronic, severe iron overload or hepatitis recommended to receive a baseline AFP, ultrasound, and FibroScan?

    <p>To assess liver health and detect potential complications.</p> Signup and view all the answers

    What is the purpose of monitoring for arrhythmias and abnormal Holter test results in adult patients with α- or β-thalassemia?

    <p>To detect potential cardiac complications.</p> Signup and view all the answers

    Why should patients with splenectomy follow vaccination recommendations?

    <p>To prevent infection and reduce the risk ofpostsplenectomy infection.</p> Signup and view all the answers

    What is the recommended frequency of performing liver function tests (CMP) in adult patients with α- or β-thalassemia?

    <p>Every 3 months (Q3 months)</p> Signup and view all the answers

    What steps should be taken regarding vaccinations for splenectomized individuals?

    <p>Follow vaccination recommendations according to CDC guidelines.</p> Signup and view all the answers

    What symptoms should patients be counseled on regarding Deep Vein Thrombosis (DVT)?

    <p>Swelling, pain, warmth, tenderness to touch, and redness in the involved leg.</p> Signup and view all the answers

    What is the purpose of performing laboratory tests for liver function and imaging in patients with clinical suspicion of liver dysfunction?

    <p>To diagnose liver dysfunction, specifically liver fibrosis, cirrhosis, and Hepatocellular Carcinoma (HCC).</p> Signup and view all the answers

    Why should patients with chronic, severe iron overload or hepatitis receive a baseline AFP, ultrasound, and FibroScan?

    <p>To assess liver fibrosis, cirrhosis, and Hepatocellular Carcinoma (HCC) risks.</p> Signup and view all the answers

    What is the recommended frequency for performing skin inspection in patients with leg ulcers?

    <p>At every visit.</p> Signup and view all the answers

    Why is annual screening for HCV, HBV, and HIV recommended in certain patients?

    <p>For patients who have received blood transfusions in the previous 12 months.</p> Signup and view all the answers

    What is a potential complication of thrombocytosis following splenectomy?

    <p>Thrombosis and vascular events.</p> Signup and view all the answers

    What is a common symptom of choledocholithiasis?

    <p>Right upper quadrant or epigastric pain, nausea, vomiting, and worsening jaundice.</p> Signup and view all the answers

    What guidelines should be referred to for infection risk and course of action during emergency-related febrile events in splenectomized individuals?

    <p>CDC guidelines.</p> Signup and view all the answers

    What is a common symptom of Pulmonary Embolism (PE)?

    <p>Dyspnea followed by chest pain, and cough.</p> Signup and view all the answers

    Study Notes

    Monitoring Recommendations for Adult Non-Transfusion-Dependent Thalassemia Patients

    • Assessment schedule is based on recommendations from leading physicians and Thalassaemia International Federation Guidelines.
    • Monitoring allows healthcare providers to track disease progression over time.

    Routine Assessments at Every Visit (Q3 Months)

    • Medical history, including quality of life assessment.
    • Physical exam.
    • Laboratory tests:
      • Complete blood count.
      • Serum ferritin.
      • Liver and kidney function test (CMP).

    Monitoring for Listed Complications

    • Extramedullary hematopoiesis:
      • MRI C/T/L spine with contrast as clinically indicated.
    • Splenomegaly:
      • Physical exam/ultrasound.
    • Iron overload:
      • Liver MRI for LIC.
      • Cardiac T2* MRI.
      • Cardiac function:
        • ECHO, including TRV.
        • Holter or equivalent.
    • Osteoporosis and bone disease:
      • DEXA scan.
      • 25-hydroxyvitamin D.
    • Endocrinopathy:
      • FSH, LH, testosterone, and estrogen.
      • Hypogonadism, hypothyroidism, and hypoparathyroidism tests.
      • Diabetes mellitus:
        • Fasting glucose or oral glucose tolerance test.

    Conditional Interventions Based on Findings

    • Refer to a cardiologist if:
      • ECHO or MRI indicates low ejection fraction, diastolic dysfunction, or pulmonary hypertension.
      • Monitoring indicates arrhythmias or abnormal Holter test results.
    • Refer to an endocrinologist or bone health specialist if:
      • Monitoring indicates abnormal assessments.
    • Refer females to a reproductive endocrinologist to assess for fertility and need for reproductive assistance.

    Consider Additional Potential Complications

    • Hemolytic crisis:
      • Counsel patients on the risk of worsening anemia and its symptoms.
      • Emergency management is required.
    • Splenectomized individuals:
      • Counsel on the risk of infections and follow vaccination recommendations.
      • Thrombocytosis following splenectomy may contribute to thrombosis and vascular events.
    • Thrombosis and vascular events:
      • Counsel on classical symptoms of DVT and PE.
      • Consider obtaining a baseline AFP, ultrasound, and FibroScan in patients with chronic, severe iron overload or hepatitis.
    • Liver fibrosis, cirrhosis, and HCC:
      • Counsel on risk and consider obtaining a baseline AFP, ultrasound, and FibroScan.
      • Perform laboratory tests for liver function and imaging with clinical suspicion.
    • Choledocholithiasis:
      • Counsel on symptoms and perform laboratory tests for liver function and imaging with clinical suspicion.
    • HCV, HBV, and HIV:
      • Annual screening (serology and, if positive, PCR) in patients who have received blood transfusions in the previous 12 months.
    • Leg ulcers:
      • Perform skin inspection at every visit.

    Monitoring Recommendations for Adult Non-Transfusion-Dependent Thalassemia Patients

    • Assessment schedule is based on recommendations from leading physicians and Thalassaemia International Federation Guidelines.
    • Monitoring allows healthcare providers to track disease progression over time.

    Routine Assessments at Every Visit (Q3 Months)

    • Medical history, including quality of life assessment.
    • Physical exam.
    • Laboratory tests:
      • Complete blood count.
      • Serum ferritin.
      • Liver and kidney function test (CMP).

    Monitoring for Listed Complications

    • Extramedullary hematopoiesis:
      • MRI C/T/L spine with contrast as clinically indicated.
    • Splenomegaly:
      • Physical exam/ultrasound.
    • Iron overload:
      • Liver MRI for LIC.
      • Cardiac T2* MRI.
      • Cardiac function:
        • ECHO, including TRV.
        • Holter or equivalent.
    • Osteoporosis and bone disease:
      • DEXA scan.
      • 25-hydroxyvitamin D.
    • Endocrinopathy:
      • FSH, LH, testosterone, and estrogen.
      • Hypogonadism, hypothyroidism, and hypoparathyroidism tests.
      • Diabetes mellitus:
        • Fasting glucose or oral glucose tolerance test.

    Conditional Interventions Based on Findings

    • Refer to a cardiologist if:
      • ECHO or MRI indicates low ejection fraction, diastolic dysfunction, or pulmonary hypertension.
      • Monitoring indicates arrhythmias or abnormal Holter test results.
    • Refer to an endocrinologist or bone health specialist if:
      • Monitoring indicates abnormal assessments.
    • Refer females to a reproductive endocrinologist to assess for fertility and need for reproductive assistance.

    Consider Additional Potential Complications

    • Hemolytic crisis:
      • Counsel patients on the risk of worsening anemia and its symptoms.
      • Emergency management is required.
    • Splenectomized individuals:
      • Counsel on the risk of infections and follow vaccination recommendations.
      • Thrombocytosis following splenectomy may contribute to thrombosis and vascular events.
    • Thrombosis and vascular events:
      • Counsel on classical symptoms of DVT and PE.
      • Consider obtaining a baseline AFP, ultrasound, and FibroScan in patients with chronic, severe iron overload or hepatitis.
    • Liver fibrosis, cirrhosis, and HCC:
      • Counsel on risk and consider obtaining a baseline AFP, ultrasound, and FibroScan.
      • Perform laboratory tests for liver function and imaging with clinical suspicion.
    • Choledocholithiasis:
      • Counsel on symptoms and perform laboratory tests for liver function and imaging with clinical suspicion.
    • HCV, HBV, and HIV:
      • Annual screening (serology and, if positive, PCR) in patients who have received blood transfusions in the previous 12 months.
    • Leg ulcers:
      • Perform skin inspection at every visit.

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    Related Documents

    Agios 2024 Thalassaemia Monitoring Guide PDF

    Description

    This quiz assesses knowledge on monitoring and assessment recommendations for adult non-transfusion-dependent thalassemia patients. It covers routine assessments and laboratory tests necessary for disease management.

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