Search results for " Replacement"
showing 10 items of 539 documents
Hunter disease before and during enzyme replacement therapy.
2011
Mucopolysaccharidosis type II (Hunter disease) is a lysosomal storage disease attributable to X-linked deficiency of the enzyme α-L-iduronate-sulfatase. Because of this deficiency, glycosaminoglycanes accumulate in various tissues and body fluids. We describe three patients representing the broad spectrum of Hunter disease and their response to enzyme replacement therapy. Patient 1 did not manifest central nervous system involvement, patient 2 manifested moderate neurologic disease, and patient 3 had already manifested a severe neurologic course during early infancy. In all patients, improvements in visceral organ size, physical capacity, and gastrointestinal functioning were reported. More…
Growth hormone replacement therapy prevents sarcopenia by a dual mechanism: improvement of protein balance and of antioxidant defenses.
2013
The aim of our study was to elucidate the role of growth hormone (GH) replacement therapy in three of the main mechanisms involved in sarcopenia: alterations in mitochondrial biogenesis, increase in oxidative stress, and alterations in protein balance. We used young and old Wistar rats that received either placebo or low doses of GH to reach normal insulin-like growth factor-1 values observed in the young group. We found an increase in lean body mass and plasma and hepatic insulin-like growth factor-1 levels in the old animals treated with GH. We also found a lowering of age-associated oxidative damage and an induction of antioxidant enzymes in the skeletal muscle of the treated animals. GH…
Nocturnal blood pressure and progression to end-stage renal disease or death in nondiabetic chronic kidney disease stages 3 and 4
2010
Objective The objective was to assess the role of office and ambulatory blood pressure (BP) on the development of end-stage renal disease (ESRD) in nondiabetic chronic renal failure. Design and method Seventy-nine patients [mean age 57 (standard deviation 11) years, 47 men, BMI 28 (4), office BP 151 (25)/92 (14) mmHg, estimated glomerular filtration rate 28 (14) ml/min per 1.73 m3] were included. The causes of renal disease were nephrosclerosis (n = 33), glomerulonephritis (n = 19), interstitial (n = 12) and others (n = 15). The average follow-up was 44 months (range 9-72 months). The primary outcome was a composite of death, from any cause, or the development of ESRD that require initiatio…
ECG-GATED MULTIDETECTOR COMPUTED TOMOGRAPHY FOR THE ASSESSMENT OF THE POSTOPERATIVE ASCENDING AORTA
2009
This study was undertaken to define the role of electrocardiographically (ECG)-gated multidetector computed tomography (MDCT) in the assessment of the postoperative ascending aorta. From November 2006 to June 2007, 21 patients, [11 men, ten women; age +/- standard deviation (SD): 62.7 +/- 10.8 years] with a history of ascending aorta replacement underwent ECG-gated MDCT and were prospectively included in our study. Ascending aorta replacement had been performed with different surgical techniques: Bentall-De Bono (four patients, 19%), Tirone-David (five patients, 23%), and modified Tirone-David with creation of aortic neosinuses (12 patients, 57%). Two patients were excluded from MDCT evalua…
The role of muscular trauma in the development of heterotopic ossification after hip surgery: An animal-model study in rats.
2015
Abstract Background Heterotopic ossification (HO), the formation of bone in soft tissues, is a frequent complication after surgery of the hip and the pelvis. Although the pathophysiological entities responsible for the formation of HO remain largely unclear, muscle trauma is alleged to play a central role in the pathogenic mechanisms underlying HO. However, for this observation, made by many surgeons for decades, no objective evidence has been provided yet. Methods Fifty male Wistar rats were subjected to surgery of the right hip. The femoral canal was reamed in three steps up to 2 mm. Animals formed 2 groups: in group 1 (25 animals) every effort was taken not to injure the muscles. In cont…
Modes of periacetabular load transfer to cortical and cancellous bone after cemented versus uncemented total hip arthroplasty: A prospective study us…
2008
Stress-shielding and periprosthetic bone loss after total hip arthroplasty (THA) may be clinically relevant for high-demand patients. Analysis of cortical and cancellous bone density (BD) changes in vivo after THA is of interest to basic science researchers and joint reconstruction surgeons. An insufficient periprosthetic bone stock may predispose to migration, early mechanical failure, and major problems in revision surgery. We used computed tomography (CT)-assisted osteodensitometry in two prospectively analyzed cohorts after cemented (n = 21) versus noncemented (n = 23) cup fixation. Periacetabular BD (mgCaHa/mL) was determined in five CT scans cranial and five CT scans at the level of t…
Venous thromboembolism in patients hospitalized for hip joint replacement surgery
2020
Venous thromboembolism (VTE) is a potentially life-threatening disease. Major transient risk factors include trauma, surgery, and immobilization. Patients undergoing hip joint replacement (HJR) are characterized by a high risk of postoperative VTE, but data on the time trends of VTE rates in this population are sparse.In an analysis of the German nationwide inpatient sample, we included all hospitalizations for elective primary HJR in Germany from 2005 to 2016. Time trends of the surgical procedure, overall death rates, and VTE rates were analysed, and predictors of VTE identified.Overall, 1,885,839 inpatients with elective primary HJR (59.1% women, 51.4% ≥70 years) were included in the ana…
Anatomical and procedural features associated with aortic root rupture during balloon-expandable transcatheter aortic valve replacement
2013
Background— Aortic root rupture is a major concern with balloon-expandable transcatheter aortic valve replacement (TAVR). We sought to identify predictors of aortic root rupture during balloon-expandable TAVR by using multidetector computed tomography. Methods and Results— Thirty-one consecutive patients who experienced left ventricular outflow tract (LVOT)/annular/aortic contained/noncontained rupture during TAVR were collected from 16 centers. A caliper-matched sample of 31 consecutive patients without annular rupture, who underwent pre-TAVR multidetector computed tomography served as a control group. Multidetector computed tomography assessment included short- and long-axis diameters an…
Gerbode-type left ventricular outflow tract to right atrial fistula complicating prosthetic aortic valve replacement identified by cardiac computed t…
2012
Abstract Acquired left ventricular-right atrial communication ( Gerbode-type defect) is a rare complication of infective endocarditis. Although transesophageal echocardiography remains the technique of choice for the evaluation of complications of endocarditis this case highlights the usefulness of cardiac computed tomography in this scenario, particularly in cases where assessment of coronary anatomy is required before surgery.
Fabry disease: enzyme replacement therapy
2003
Fabry disease is a multisystem disorder associated with wide variability in clinical expression. Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of alpha-galactosidase A. The enzyme defect leads to the systemic accumulation of glycosphingolipids with alpha-galactosyl moieties consisting predominantly of globotriaosylceramide, galabiosylceramide and two additional glycosphingolipids. Four hemizygotes patients with a family history of Fabry disease and deficiency of the enzyme alpha-galactosidase A were selected. Each patient received purified alpha-galactosidase by intravenous infusion (0.2 mg/kg). The infusion was administered every 2 weeks, for 40 min, for a …