Search results for "Placement."
showing 10 items of 1123 documents
Natural course of Fabry disease: changing pattern of causes of death in FOS - Fabry Outcome Survey
2009
Background: Fabry disease is a rare X-linked lysosomal storage disorder characterised by severe multisystemic involvement that leads to major organ failure and premature death in affected men and women. Over the past 7 years, the Fabry Outcome Survey (FOS) has collected data on the natural history of Fabry disease, and the long-term efficacy and safety of enzyme-replacement therapy. This paper provides an update on the first analysis of FOS data. Design: Baseline data on clinical manifestations and causes of death in a cohort of 1453 patients (699 male, 754 female) from 19 countries worldwide were analysed. Causes of death of affected relatives were analysed separately. Results: The most fr…
Clinical characteristics of adults with slowly progressing mucopolysaccharidosis VI: a case series.
2012
To assess clinical features and general health status of adult patients with mucopolysaccharidosis (MPS) VI. This report includes the clinical history of patients older than 18 years with slowly progressing MPS VI and the retrospective analysis of the outcomes of available data collected between September 2003 and October 2008 at the Center of Pediatric and Adolescent Medicine, University Medical Center, Johannes Gutenberg-University of Mainz, Germany. Variables included were urinary glycosaminoglycan (uGAG) level, mutation analysis, body height, forced vital capacity (FVC), 6-minute walk test, echocardiographic findings, the need for craniocervical decompression surgery, orthopaedic findin…
The Applicability of a Checklist for the Diagnosis and Treatment of Exocrine Pancreatic Insufficiency: Results of the Italian Exocrine Pancreatic Ins…
2020
Objective To evaluate a rapid checklist capable of identifying exocrine pancreatic insufficiency in outpatients. Methods Prospective observational study of a multicenter cohort. Results One hundred and two patients were enrolled; 61.8% of the patients had medically-treated benign or malignant pancreatic disease, and 38.2% had a pancreatic resection. Visual examination of the feces was evaluated in 84 patients and it was related to steatorrhea in 51 patients (50.0%). Receiver operating characteristic curves were evaluated for each symptom or clinical sign and four of them (ie, increase in daily bowel movements, number of bowel movements, fatty stools, >10% weight loss) had a satisfactory …
Mortality and cause of death in mucopolysaccharidosis type II-a historical review based on data from the Hunter Outcome Survey (HOS).
2009
Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a progressive, multisystemic disease caused by a deficiency of iduronate-2-sulfatase. Patients with the severe form of the disease have cognitive impairment and typically die in the second decade of life. Patients with the less severe form do not experience significant cognitive involvement and may survive until the fifth or sixth decade of life. We studied the relationship of both severity of MPS II and the time period in which patients died with age at death in 129 patients for whom data were entered retrospectively into HOS (Hunter Outcome Survey), the only large-scale, multinational observational study of patients with MPS II.…
Spontaneous echo contrast imaging in infective endocarditis: a predictor of complications?
1992
Infective endocarditis is associated with significant morbidity and mortality. Valvular destruction and congestive heart failure are more common in patients with echocardiographically detectable vegetations. In addition, spontaneous platelet aggregation is increased when vegetations are present on cardiac valves. The aim of the study was to assess the prognostic value of spontaneous echo contrast (SEC) imaging, as SEC is supposed to reflect red blood cell aggregates stimulated by platelet activity. We studied 293 patients with clinical signs of infective endocarditis. Vegetations, attached to the aortic or mitral valve, were found in 130 patients (44.4%) who were followed for a mean period …
Enzyme Replacement Therapy Stabilized White Matter Lesion Progression in Fabry Disease
2014
<b><i>Background:</i></b> The central nervous system manifestations in Fabry disease (FD) include progressive white matter lesions (WMLs) and stroke. Due to progressive microvascular involvement, men and women with FD over 35 years of age develop WMLs. Moreover, the prevalence of stroke has been estimated to be 12 times higher in FD compared with the general population. Enzyme replacement therapy (ERT) is available and has shown beneficial effects on renal, cardiac, and peripheral nerve function in FD, but the ERT effect on the progression of WMLs, or the reduction in cerebrovascular events, remains unknown. <b><i>Methods:</i></b> The WML burd…
Quantitative computed tomography-assisted osteodensitometry of the pelvis after press-fit cup fixation: a prospective ten-year follow-up.
2011
Background: As a follow-up of a previously reported three-year study, we analyzed the periprosthetic acetabular cortical and cancellous bone density changes at ten years after implantation of a press-fit cup. Methods: Prospective clinical, radiographic, and quantitative computed tomography examinations were performed within ten days and at mean periods of one, three, and ten years after total hip arthroplasty with a press-fit cup, a femoral stem with a tapered design, and alumina-alumina pairing. Periacetabular cortical and cancellous bone density (mg CaHA/mL) in the cranial, ventral, and dorsal regions about the cup were measured for twenty-four hips in vivo. Results: All acetabular cups s…
Cervical Spondylotic Myelopathy: When and Why the Cervical Corpectomy?
2020
Background: Cervical spondylotic myelopathy (CSM) is a degenerative disease that represents the most common spinal cord disorder in adults. The best treatment option has remained controversial. We performed a prospective study to evaluate the clinical, radiographic, and neurophysiologic outcomes for anterior cervical corpectomy in the treatment of CSM. Methods: From January 2011 to January 2017, 60 patients with CSM were prospectively enrolled in the present study. The patients were divided according to the modified Japanese Orthopaedic Association scale (mJOA) score into 2 groups: group A, patients with mild to moderate CSM (mJOA score ≥13); and group B, patients with severe myelopathy (mJ…
Bone Marrow Concentrate and Bovine Bone Mineral for Sinus Floor Augmentation
2011
Purpose: The purpose of this work was to evaluate the potential of substituting autogenous bone (AB) by bone marrow aspirate concentrate (BMAC). Both AB and BMAC were tested in combination with a bovine bone mineral (BBM) for their ability of new bone formation (NBF) in a multicentric, randomized, controlled, clinical and histological noninferiority trial.Materials and Methods: Forty-five severely atrophied maxillary sinus from 26 patients were evaluated in a partial cross-over design. As test arm, 34 sinus of 25 patients were augmented with BBM and BMAC containing mesenchymal stem cells. Eleven control sinus from 11 patients were augmented with a mixture of 70% BBM and 30% AB. Biopsies wer…
Lifetime exercise and disk degeneration: an MRI study of monozygotic twins.
1997
Participation in some competitive sports has been shown to increase disk degeneration; however, the long-term effects of recreational physical activities are unclear. We investigated the effects of endurance exercise and power sports on disk degeneration in monozygotic male twins with contrasting lifetime exercise histories. The effects of endurance exercise were studied in 22 discordant twin pairs (mean lifetime frequencies of 3.9 vs 1.1 times/wk), and the effects of power sports were investigated in 12 discordant pairs (2,300 vs 200 h of weightlifting). The age range of the twins was from 35 to 69 yr. No differences in MRI findings between co-twins discordant for endurance exercise were f…