80 Questions
What hormone levels should be monitored in patients with hypogonadism?
FSH, LH, testosterone, and estrogen
How often should patients on chelation therapy have their hormone levels monitored?
Every month
What test is used to diagnose diabetes mellitus?
Fasting glucose or oral glucose tolerance test
What is the recommended frequency for performing liver MRI in patients with frequent transfusions or high serum ferritin levels?
At baseline only
What is the recommended frequency for performing cardiac MRI in patients with iron overload?
Only if LIC is ≥10 mg/g dry weight
What hormone levels should be monitored in patients with hypothyroidism?
Free thyroxine (FT4) and TSH
What is the recommended frequency for performing hormone assessments in patients not receiving iron chelation therapy?
Every 24 months
What is the recommended frequency for performing hormone assessments in patients receiving iron chelation therapy?
Every 12 months
What is the recommended frequency for performing exams in patients with evidence of iron overload?
Annually
What electrolytes should be monitored in patients with hypoparathyroidism?
Calcium, phosphate, and magnesium
What is the primary purpose of monitoring adult patients with α- or β-thalassemia from the time of diagnosis?
To track disease progression over time
What is recommended to be performed at every visit (Q3 months) for adult patients with α- or β-thalassemia?
Complete blood count, liver and kidney function test, and physical exam
Which specialist should a patient with abnormal assessments be referred to?
Endocrinologist or bone health specialist
What laboratory test is recommended to assess iron overload in adult patients with α- or β-thalassemia?
Liver MRI for LIC
What does an elevated TRV indicate?
Pulmonary hypertension
What is recommended to assess cardiac function in adult patients with α- or β-thalassemia?
ECHO, including TRV
Which of the following is a symptom of hemolytic crisis?
All of the above
What is recommended to assess osteoporosis and bone disease in adult patients with α- or β-thalassemia?
DEXA scan
What is the frequency of routine assessments recommended for adult patients with α- or β-thalassemia?
Every 3 months
What is the purpose of referring a female to a reproductive endocrinologist?
To assess for fertility and need for reproductive assistance
Which test is used to assess liver iron concentration?
LIC
What is the purpose of collecting a baseline assessment for every patient with α- or β-thalassemia?
To track disease progression over time
What is recommended to assess extramedullary hematopoiesis in adult patients with α- or β-thalassemia?
MRI C/T/L spine with contrast
What is the purpose of monitoring for arrhythmias and abnormal Holter test results?
To refer the patient to a cardiologist
What is the complication that can commonly develop from infections resulting in fever?
Worsening anemia
What is the purpose of performing a physical exam on adult patients with α- or β-thalassemia?
To assess for splenomegaly
Which hormone is related to reproductive health?
FSH
What is recommended to assess endocrinopathy in adult patients with α- or β-thalassemia?
25-hydroxyvitamin D
What is the name of the test used to assess bone health?
DEXA
When should a patient be counseled on the risk of worsening anemia?
When infections resulting in fever occur
What is a potential complication of thrombocytosis following splenectomy?
Thrombosis and vascular events
Why should patients with chronic, severe iron overload or hepatitis receive a baseline AFP, ultrasound, and FibroScan?
To monitor for liver fibrosis, cirrhosis, and HCC
What is the recommended frequency for skin inspection in patients with leg ulcers?
At every visit
What is a common symptom of Deep Vein Thrombosis (DVT)?
Swelling and tenderness to touch in the involved leg
Why is annual screening for HCV, HBV, and HIV recommended in certain patients?
For patients who have received blood transfusions in the previous 12 months
What is a common symptom of Pulmonary Embolism (PE)?
Dyspnea followed by chest pain
Why should patients with splenectomy follow vaccination recommendations?
To prevent infections
What is a common symptom of choledocholithiasis?
Upper right quadrant or epigastric pain, nausea, vomiting, and worsening jaundice
What is a recommended laboratory test for patients with clinical suspicion of liver dysfunction?
Liver function tests
What guidelines should be referred to for infection risk and course of action during emergency-related febrile events in splenectomized individuals?
CDC guidelines
What hormones should be monitored in patients with hypogonadism?
FSH, LH, testosterone, and estrogen
What test is used to diagnose hypothyroidism?
Free thyroxine (FT4) and TSH
What electrolytes should be monitored in patients with hypoparathyroidism?
PTH, calcium, phosphate, and magnesium
How often should liver MRI be performed in patients with frequent transfusions or high serum ferritin levels?
At baseline and every 24 months or every 12 months with abnormality
What is the recommended frequency for performing hormone assessments in patients on chelation therapy?
Every 12 months
What is the recommended frequency for performing cardiac MRI in patients with iron overload?
Every 24 months or every 12 months with abnormality
How often should patients on deferiprone have their iron levels monitored?
Every 2 weeks
What is the recommended frequency for performing exams in patients with evidence of iron overload?
Annually
What is the recommended frequency for performing hormone assessments in patients not on chelation therapy?
Every 24 months
What is the primary purpose of monitoring adult patients with α- or β-thalassemia from the time of diagnosis?
To detect and manage complications
What should a patient be referred to when ECHO or MRI indicates low ejection fraction or evidence of diastolic dysfunction?
A cardiologist
What should a patient be referred to when monitoring indicates abnormal hormone levels?
An endocrinologist or bone health specialist
What is the purpose of performing a DEXA scan in adult patients with α- or β-thalassemia?
To assess osteoporosis and bone disease
What should a patient be referred to when monitoring indicates arrhythmias or abnormal Holter test results?
A cardiologist
What is the complication that can commonly develop from infections resulting in fever?
Worsening anemia
What is the purpose of monitoring for hemolytic crisis?
To detect symptoms of worsening fatigue, jaundice, and/or dark-colored urine
What should a female patient be referred to when assessing for fertility and need for reproductive assistance?
A reproductive endocrinologist
What is the purpose of performing an ECHO in adult patients with α- or β-thalassemia?
To assess cardiac function
What should a patient be referred to when monitoring indicates evidence of pulmonary hypertension?
A cardiologist
What is the purpose of monitoring for liver iron concentration?
To assess liver iron overload
What is the recommended frequency of routine assessments for adult patients with α- or β-thalassemia?
Every 3 months (Q3 months)
What laboratory tests are recommended to assess iron overload in adult patients with α- or β-thalassemia?
Serum ferritin and liver MRI for LIC
What test is used to assess cardiac function in adult patients with α- or β-thalassemia?
ECHO, including TRV
What is recommended to assess osteoporosis and bone disease in adult patients with α- or β-thalassemia?
DEXA scan
What is the purpose of collecting a baseline assessment for every patient with α- or β-thalassemia?
To track disease progression over time and allow healthcare providers to make informed decisions about treatment.
What is recommended to assess extramedullary hematopoiesis in adult patients with α- or β-thalassemia?
MRI C/T/L spine with contrast
Why are patients with chronic, severe iron overload or hepatitis recommended to receive a baseline AFP, ultrasound, and FibroScan?
To assess liver health and detect potential complications.
What is the purpose of monitoring for arrhythmias and abnormal Holter test results in adult patients with α- or β-thalassemia?
To detect potential cardiac complications.
Why should patients with splenectomy follow vaccination recommendations?
To prevent infection and reduce the risk ofpostsplenectomy infection.
What is the recommended frequency of performing liver function tests (CMP) in adult patients with α- or β-thalassemia?
Every 3 months (Q3 months)
What steps should be taken regarding vaccinations for splenectomized individuals?
Follow vaccination recommendations according to CDC guidelines.
What symptoms should patients be counseled on regarding Deep Vein Thrombosis (DVT)?
Swelling, pain, warmth, tenderness to touch, and redness in the involved leg.
What is the purpose of performing laboratory tests for liver function and imaging in patients with clinical suspicion of liver dysfunction?
To diagnose liver dysfunction, specifically liver fibrosis, cirrhosis, and Hepatocellular Carcinoma (HCC).
Why should patients with chronic, severe iron overload or hepatitis receive a baseline AFP, ultrasound, and FibroScan?
To assess liver fibrosis, cirrhosis, and Hepatocellular Carcinoma (HCC) risks.
What is the recommended frequency for performing skin inspection in patients with leg ulcers?
At every visit.
Why is annual screening for HCV, HBV, and HIV recommended in certain patients?
For patients who have received blood transfusions in the previous 12 months.
What is a potential complication of thrombocytosis following splenectomy?
Thrombosis and vascular events.
What is a common symptom of choledocholithiasis?
Right upper quadrant or epigastric pain, nausea, vomiting, and worsening jaundice.
What guidelines should be referred to for infection risk and course of action during emergency-related febrile events in splenectomized individuals?
CDC guidelines.
What is a common symptom of Pulmonary Embolism (PE)?
Dyspnea followed by chest pain, and cough.
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.
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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