Search results for "Replacement"
showing 10 items of 561 documents
Cardiac disease in patients with mucopolysaccharidosis: presentation, diagnosis and management
2011
The mucopolysaccharidoses (MPSs) are inherited lysosomal storage disorders caused by the absence of functional enzymes that contribute to the degradation of glycosaminoglycans (GAGs). The progressive systemic deposition of GAGs results in multi-organ system dysfunction that varies with the particular GAG deposited and the specific enzyme mutation(s) present. Cardiac involvement has been reported in all MPS syndromes and is a common and early feature, particularly for those with MPS I, II, and VI. Cardiac valve thickening, dysfunction (more severe for left-sided than for right-sided valves), and hypertrophy are commonly present; conduction abnormalities, coronary artery and other vascular in…
Use of topical tranexamic acid in primary total hip arthroplasty. Efficiency and safety. Our experience.
2019
Abstract Background and aim Intravenous tranexamic acid has been shown to reduce bleeding and the need for transfusions in total hip arthroplasty, although it has a theoretical risk of producing thromboembolic phenomena. Recently some papers have been published using the topical application of tranexamic acid, but the ideal administration protocol has not yet been clearly defined. The aim of this paper was to demonstrate that our protocol of topical tranexamic acid is effective and safe. Methods Prospective data collection from a case series of 80 primary hip arthroplasties, in which the following topical tranexamic acid protocol is used: 1.5 g diluted to a total volume of 60 ml were admini…
Fabry disease: overall effects of agalsidase alfa treatment
2004
Background Fabry disease is a rare X-linked disorder caused by deficient activity of the lysosomal enzyme α-galactosidase A. Progressive accumulation of the substrate globotriaosylceramide in cells throughout the body leads to major organ failure and premature death. The Fabry Outcome Survey (FOS) is a European outcomes database which was established to collect data on the natural history of this little-known disease and to monitor the long-term efficacy and safety of enzyme replacement therapy (ERT) with agalsidase alfa. This paper presents the first analysis of the FOS database on the effects of ERT on renal function, heart size, pain and quality of life. Design The effects of 1 and 2 y…
Use of gabapentin to reduce chronic neuropathic pain in Fabry disease.
2003
The effect of the anticonvulsant gabapentin on neuropathic pain was studied in six male patients with Fabry disease, aged 15-45 years. After 4 weeks of treatment, pain, as measured using the Brief Pain Inventory, was decreased compared with baseline. Treatment was generally well tolerated. This study indicates that gabapentin should be considered as a treatment option for the neuropathic pain of Fabry disease.
Morbidity in 565 Type 2 Diabetic Patients According to Stage of Nephropathy
1998
Between 1988 and 1992, 565 type 2 diabetic patients were examined for nephropathy and diabetes-associated diseases during hospital treatment. Stages of nephropathy were defined as no clinical sign of nephropathy (N = 280), microalbuminuria (N = 38), overt proteinuria (N = 105), impaired renal function (N = 55), and chronic dialysis therapy (N = 87). In dialyzed patients, HbA1c averaged 6.8%, and, in the other groups, HbA1c was between 7.6% and 8.3% (normal range, 3.8%-6.1%). Cataract was not associated with the severity of nephropathy. Stroke was most common in the stage of renal insufficiency (34%). The following complications, as found in medical history or as current event, showed a sign…
Evaluation of total alloplastic temporo-mandibular joint replacement with two different types of prostheses : a three-year prospective study
2015
Background Temporo-Mandibular Joint (TMJ) replacement has been used clinically for years. The objective of this study was to evaluate outcomes achieved in patients with two different categories of TMJ prostheses. Material and Methods All patients who had a TMJ replacement (TMJR) implanted during the study period from 2006 through 2012 were included in this 3-year prospective study. All procedures were performed using the Biomet Microfixation TMJ Replacement System, and all involved replacing both the skull base component (glenoid fossa) and the mandibular condyle. Results Fifty-seven patients (38 females and 19 males), involving 75 TMJs with severe disease requiring reconstruction (39 unila…
Changes in plasma and urine globotriaosylceramide levels do not predict Fabry disease progression over 1 year of agalsidase alfa.
2013
Globotriaosylceramide concentrations were assessed as potential predictors of change from baseline after 12 months by estimated glomerular filtration rate and left-ventricular mass index using pooled data from three randomized, placebo-controlled agalsidase alfa trials and open-label extensions of patients with Fabry disease.Males (aged 18 years or older) with Fabry disease received agalsidase alfa (0.2 mg/kg every other week for 12 months). A backward-elimination approach evaluated potential predictors (baseline estimated glomerular filtration rate and left-ventricular mass index; age at first dose; baseline and change from baseline at 12 months of globotriaosylceramide (urine, plasma); ur…
Cardiac manifestations of Anderson-Fabry disease: results from the international Fabry outcome survey.
2007
Aims Anderson–Fabry disease (AFD) is an uncommon X-linked disorder caused by deficient activity of the lysosomal enzyme α-galactosidase A. The Fabry Outcome Survey is a European database designed to monitor the long-term efficacy and safety of enzyme replacement therapy (ERT) with agalsidase alfa. The aim of this study was to determine the prevalence and characteristics of cardiac disease in AFD patients. Methods and results Clinical and laboratory data were available in 714 patients from 11 countries (mean age 35 ± 17 years, 369 women, 336 treated). The prevalence of angina was 23 vs. 22%; palpitations and arrhythmias 27 vs. 26%; exertional dyspnoea 23 vs. 23%; and syncope 2 vs. 4%, in wom…
Five times sit-to-stand test in subjects with total knee replacement: Reliability and relationship with functional mobility tests
2017
The objective was to determine the inter-observer and test/retest reliability of the "Five-repetition sit-to-stand" (5STS) test in patients with total knee replacement (TKR). To explore correlation between 5STS and two mobility tests. A reliability study was conducted among 24 (mean age 72.13, S.D. 10.67; 50% were women) outpatients with TKR. They were recruited from a traumatology unit of a public hospital via convenience sampling. A physiotherapist and trauma physician assessed each patient at the same time. The same physiotherapist realized a 5STS second measurement 45-60min after the first one. Reliability was assessed with intraclass correlation coefficients (ICCs) and Bland-Altman plo…
Gender-, age- and time-dependent dosing of growth hormone in adults - real-world data from a decade of clinical practice in Germany.
2017
We evaluated treatment patterns and gender-dependent dosing of growth hormone (GH) substitution in adults with GH deficiency (AGHD). Data on GH dose were collected (2003-2013) from 509 GH-treated patients (mean age: 48.9 years; 47% female) enroled in the observational German NordiWin study (NCT01543880). The impact of gender, age, treatment duration and calendar year on GH treatment patterns was evaluated by multiple regression analysis. Mean (SD) baseline GH dose (mg/day) was similar between females (0.25 [0.19] and males (0.24 [0.15]), but increased with treatment duration (at year 10, 0.55 [0.48] and 0.31 [0.09] in females and males, respectively), reflecting patient dose titration. GH d…