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LOPD Treatment Recommendations Quiz

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Questions and Answers

When should patients with LOPD be initiated on Enzyme Replacement Therapy (ERT) according to the AANEM panel recommendations?

Only if they are symptomatic

When they experience limb weakness and difficulty completing ADL

When they have detectable proximal muscle weakness or reduced FVC (correct)

When they use a wheelchair and invasive ventilation

What is the advice for patients who are asymptomatic but have signs of late-onset Pompe disease (LOPD) according to Brazilian clinicians?

Monitor symptoms and start ERT at the onset of any symptoms

Administer ERT for 1 year immediately

Initiate ERT if there is difficulty with activities of daily living (ADL)

Consider ERT if muscle weakness or reduced pulmonary function is observed (correct)

What is the recommended frequency for monitoring for the development of antibodies in patients undergoing ERT according to the AANEM panel recommendations?

Every month

Every 6 months

Every 3 months for up to 2 years, then annually (correct)

Only if symptoms worsen

According to the recommendations, when should patients with severe symptoms be reevaluated after receiving ERT?

After 1 year of treatment Signup and view all the answers

What should be considered before continuing ERT in patients requiring constant invasive ventilation?

Symptom improvement during the first year of treatment Signup and view all the answers

What is the primary factor determining the initiation of ERT in presymptomatic patients with objective signs according to the AANEM panel recommendations?

Proximal muscle weakness Signup and view all the answers

When is it recommended to initiate enzyme replacement therapy (ERT) for presymptomatic patients without objective signs of LOPD?

When they have objective signs of proximal muscle weakness Signup and view all the answers

What should be done for symptomatic patients with LOPD who experience increased limb weakness and difficulty completing ADL?

Consider ERT if they have reduced FVC Signup and view all the answers

What is the recommendation for patients with severe symptoms such as wheelchair use and constant invasive ventilation?

Consider initiation of ERT on a case-by-case basis Signup and view all the answers

What is the protocol for presymptomatic patients with objective signs of LOPD regarding ERT initiation?

Administer ERT immediately Signup and view all the answers

At what point should presymptomatic patients without objective signs initiate ERT according to the recommendations?

At the onset of symptoms or detectable proximal muscle weakness Signup and view all the answers

For which type of patient is the continuation of ERT considered on a case-by-case basis?

For patients requiring constant invasive ventilation Signup and view all the answers

Study Notes

A panel of neuromuscular specialists and a pulmonologist developed treatment recommendations for Late-Onset Pompe Disease (LOPD).
Recommendations for initiating Enzyme Replacement Therapy (ERT) in LOPD patients are based on the onset and severity of symptoms.
ERT initiation criteria include different scenarios for presymptomatic patients without objective signs, presymptomatic patients with objective signs, symptomatic patients, and patients with severe symptoms.
Continuation of ERT can be considered on a case-by-case basis for patients requiring constant invasive ventilation, based on symptom stabilization or improvement.
Regular monitoring, evaluation of ERT effectiveness annually, and testing for IgG antibodies development are recommended by the panel.

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Description

Test your knowledge on treatment recommendations for Late-Onset Pompe Disease (LOPD) developed by an AANEM panel of specialists. Learn about when to initiate enzyme replacement therapy (ERT) based on the onset and severity of symptoms.