Search results for "hypertrophic"
showing 2 items of 42 documents
The Heart in Fabry Disease – from Pathogenesis to Enzyme Replacement Therapy
2010
The cardiovascular involvement in Fabry disease is progressive, and accounts for one of the major reasons for abbreviated life expectancy and increased morbidity. The majority of patients develop signs and symptoms, related to heart failure and arrhythmias. Hypertrophic cardiomyopathy, in later stages combined with myocardial fibrosis, is one of the leading features. Both genders are affected, and females develop severe cardiac Fabry disease approximately 10–15 years later in life than male patients. Diastolic dysfunction and reduced longitudinal mid-wall related systolic function are the mechanical consequences of the myocardial and epithelial changes related to accumulation of the storage…
Significance of ultrasound for the diagnosis of hypertrophic pyloric stenosis
1986
The sonographic features of hypertrophic pyloric stenosis (HPS) were evaluated in a 5-year prospective study (1981–1985) at the University Clinic of Pediatric Surgery in Mainz. In 37 cases real-time ultrasound was performed preoperatively to measure muscle thickness, diameter, and length of the pylorus; the recorded values were compared with those of a control group. Muscular wall thickness proved to be the most reliable parameter, while pyloric canal length was the most difficult measurement to obtain. Diameter alone was not adequate to establish the sonographic diagnosis, which depends on the individually and functionally different luminal width and submucosal thickness. Significant sonog…