Natural progression of cardiac features and long-term effects of enzyme replacement therapy in Taiwanese patients with mucopolysaccharidosis II.

ABSTRACT:

Background

Cardiac abnormalities have been observed in patients with mucopolysaccharidosis type II (MPS II). The aim of this study was to investigate the cardiac features and natural progression of Taiwanese patients with MPS II, and evaluate the impact of enzyme replacement therapy (ERT) on cardiac structure and function.

Methods

The medical records and echocardiograms of 48 Taiwanese patients with MPS II (median age, 6.9 years; age range, 0.1-27.9 years) were reviewed. The relationships between age and each echocardiographic parameter were analyzed.

Results

The mean z-scores of left ventricular mass index (LVMI), interventricular septum diameter in diastole (IVSd), left ventricular posterior wall diameter in diastole (LVPWd), and aortic diameter were 1.10, 2.70, 0.9

SUBMITTER: Lin HY 

PROVIDER: S-EPMC7903682 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature