Evaluation of Echocardiographic Findings of Mucopolysaccharidosis Cases
Journal Title: European Archives of Medical Research - Year 2019, Vol 35, Issue 3
Abstract
Objective: Mucopolysaccharidosis (MPS) is a lysosomal storage disease in which the degradation of glycosaminoglycans is impaired. Cardiac involvement may occur in different ways in all types of the disease. In this study, we aimed to evaluate the echocardiographic reports of our patients with MPS retrospectively. Methods: Echocardiography reports of 37 patients with MPS were reviewed retrospectively. Results: Cardiac involvement was present in 70.2% our patients and the most commonly involved structure was mitral valve (59.5%). The most common pathology in mitral valve was mitral valve regurgitation (51.4%). Conclusion: Cardiac involvement and complications are frequently seen in MPS. Heart failure, coronary artery involvement and arrhythmias are the main causes of death. Early diagnosis of MPS and early initiation of enzyme replacement therapy may improve cardiac involvement. Progressive valve involvement may require surgical intervention over time. For all these reasons, cardiac evaluation in MPS patients should be performed at least once a year with accompanying electrocardiogram and echocardiography.
Authors and Affiliations
Nafiye Emel Çakar, Meryem Karaca
Keywords
Related Articles
- EP ID EP549064
- DOI 10.4274/eamr.galenos.2019.92486
- Views 160
- Downloads 0
Evaluation of the Effects of COVID-19 Pandemic Among Patients with Inflammatory Bowel Diseases
Objective: The Coronavirus Disease-2019 (COVID-19) pandemic continues to be a concern, especially to people with chronic diseases across the entire world. During this pandemic, we undertook an investigation to assess how...
Two Cases; Extra-articular Synovial Chondromatosis of Flexor Compartments of the Wrist
Synovial chondromatosis is a benign synovial neoplasm. Its main characteristic is the formation of cartilaginous nodules in synovial joints, bursae and tendon sheath. It usually presents between the third and fifth decad...
A Comparative Study of Two Different Accommodative Intraocular Lenses: The Visual and Accommodative Amplitude Outcomes
Objective: To compare distance and near visual acuity along with accommodative amplitude in eyes implanted with the Tetraflex and the Tek-clear accommodating intraocular lenses (AIOLs). Methods: Comparative, prospective...
Cornelia de Lange Syndrome with Hypernatremia: Two Case Reports
Cornelia de Lange syndrome (CdLS), is a genetical syndrome which can be seen as hereditary, or sporadically. Characteristical appearance is very crucial for diagnosis. Although almost all patient shave characteristical f...
PSMA-positive Secondary Tumors in 68Ga-PSMA PET/CT Imaging in Patients with Prostate Cancer
Objective: The objective of this research was to examine the prostate-specific membrane antigen (PSMA)-positive secondary tumor incidence in 68Ga-PSMA positron emission tomography/computed tomography (PET/CT) imaging in...