diaphragm weakness

Chest MRI May Help Monitor Pompe Progression, Treatment Efficacy

The function of the diaphragm, a major muscle in breathing, is significantly reduced over a year in adults with Pompe disease relative to healthy people, a small study showed. These deficits, detected through a chest MRI test, were not accompanied by significant changes in lung function tests. In addition,…

Muscle Fat Replacement Linked to Respiratory Impairment

Fat replacement of back, abdominal, and trunk muscles in late-onset Pompe disease (LOPD) correlates with diaphragm weakness and worsening respiratory function, a long-term clinical study has determined. “These results suggest that identification of fat replacement in these areas … should…

Chest MRI May Capture Respiratory Muscle Weakness at Early Stages

A chest MRI can be used to detect early signs of respiratory muscle weakness in people with Pompe disease, even when lung function is still within a normal range, a study reported. According to its investigators, the findings highlight the importance of these imaging tests to rapidly identify patients…