MRI changes in diaphragmatic motion and curvature in Pompe disease over time.

ABSTRACT:

Objectives

To evaluate changes in diaphragmatic function in Pompe disease using MRI over time, both during natural disease course and during treatment with enzyme replacement therapy (ERT).

Methods

In this prospective study, 30 adult Pompe patients and 10 healthy controls underwent pulmonary function tests and spirometry-controlled MRI twice, with an interval of 1 year. In the sagittal view of 3D gradient echo breath-hold acquisitions, diaphragmatic motion (cranial-caudal ratio between end-inspiration and end-expiration) and curvature (diaphragm height and area ratio) were calculated using a machine learning algorithm based on convolutional neural networks. Changes in outcomes after 1 year were compared between Pompe patients and healthy controls using the Mann-Whitney test.

SUBMITTER: Harlaar L 

PROVIDER: S-EPMC9705445 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature