Publications by authors named "Z Koeks"
Background: Becker muscular dystrophy (BMD) is an X-linked disorder characterized by slow, progressive muscle damage and muscle weakness. Hallmarks include fibre-size variation and replacement of skeletal muscle with fibrous and adipose tissues, after repeated cycles of regeneration. Muscle histology can detect these features, but the required biopsies are invasive, are difficult to repeat and capture only small muscle volumes.
View Article and Find Full Text PDFArticle Synopsis
- Becker muscular dystrophy (BMD) shows slow progression, highlighting the need for biomarkers to support clinical trials; researchers examined changes in three muscle-enriched biomarkers in BMD patients over four years to evaluate their relation to disease severity and progression.
- The study involved measuring creatine kinase, creatine/creatinine levels, and myostatin in patient serum while assessing functional performance through various tests, revealing a strong correlation between Cr/Crn and myostatin with functional outcomes, but a weak association with creatine kinase.
- Outcomes from 34 patients indicated that specific biomarkers could explain up to 75% of the variance in functional performance, although dystrophin levels did not show a correlation with these biomarkers or patient performance
Background: In Becker muscular dystrophy evidence for neurocognitive and behavioral comorbidity is evolving. More insight into the extend of these problems is of great importance for early detection and remediation in clinical practice.
Objective: In this study we aimed to describe the neurocognitive and behavioral features of a Dutch adult cohort of BMD patients, and to evaluate correlations to motor function outcomes.
Article Synopsis
- - Cardiac issues are the leading cause of death in Becker muscular dystrophy (BMD), making it vital to monitor left ventricular (LV) function, where LV global longitudinal strain (GLS) may offer a more sensitive detection of early myocardial dysfunction compared to standard LV ejection fraction (LVEF).
- - A study involving 40 BMD patients and 21 controls showed that while both LVEF and GLS were impaired in BMD patients, a higher percentage exhibited reduced GLS, highlighting its potential as a key indicator for cardiac health in these patients.
- - Over a follow-up period of about two years, LV GLS showed significant deterioration, whereas LVEF remained stable, suggesting that GLS might be a better tool
Objective: To identify the best quantitative fat-water MRI biomarker for disease progression of leg muscles in Becker muscular dystrophy (BMD) by applying a stepwise approach based on standardized response mean (SRM) over 24 months, correlations with baseline ambulatory tests, and reproducibility.
Methods: Dixon fat-water imaging was performed at baseline (n = 24) and 24 months (n = 20). Fat fractions (FF) were calculated for 3 center slices and the whole muscles for 19 muscles and 6 muscle groups.