Search results for " Replacement"

Mutations in the Factor XII Gene in Solitary Cases of Recurrent Angioedema with Normal C1 Inhibitor Induced or Worsened by Oral Contraceptives or Hor…

2012

Downregulation of alpha-galactosidase A upregulates CD77: functional impact for Fabry nephropathy.

2009

Anderson-Fabry disease, an inherited deficiency in the lysosomal enzyme alpha-galactosidase A, is characterized by the progressive accumulation of globotriaosylceramide (Gb3), also known as CD77. We sought to clarify the pathogenesis of Fabry disease by establishing a cell model of this disorder. The expression of alpha-galactosidase A was transiently silenced by RNA interference in HK2 and primary human renal epithelial cells and stably silenced in HK2 cells by retroviral transfection with small hairpin RNA. All of the silenced cells had histological similarities to cells of patients with Fabry disease. The cells had reduced viability, significant accumulation of intracellular Gb3, and a m…

Effects of enzyme replacement therapy with agalsidase alfa on glomerular filtration rate in patients with Fabry disease: preliminary data

2007

Progressive deposition of globotriaosylceramide results in severe complications involving the kidney, heart and brain in both hemizygous male and heterozygous female patients with Fabry disease. Analysis of renal data from FOS - the Fabry Outcome Survey - suggests that enzyme replacement therapy with agalsidase alfa can significantly improve renal function in patients with Fabry disease, at least in those with a mild decrease in glomerular filtration rate, and may also be able to slow down the natural decline in renal function in patients with a moderate reduction in glomerular filtration rate. Conclusion: Initial results from the large cohort of patients within FOS indicate that treatment …

Change in bone mass distribution induced by hormone replacement therapy and high-impact physical exercise in post-menopausal women.

2002

The purpose of this intervention trial was to determine whether changes in bone mass distribution could be observed in postmenopausal women following hormone replacement therapy (HRT) and/or high-impact physical exercise. Eighty healthy women, aged 50-57 years, at5 years after the onset of menopause and with no previous use of HRT, were randomly assigned to one of four groups: HRT; exercise (Ex); HRT + Ex (ExHRT); and control (Co). HRT administration was conducted in a double-blind manner for 1 year using estradiol plus noretisterone acetate (Kliogest). The exercise groups participated in a 1 year progressive training program consisting of jumping and bounding activities. Subjects participa…

The effect of oral hormone replacement therapy on lipoprotein profile, resistance of LDL to oxidation and LDL particle size

2001

Abstract Objectives: To disclose if oral estradiol (E 2 ), alone or in combination with natural progesterone (P) or medroxyprogesterone acetate (MPA), may modify the oxidizability of low density lipoprotein (LDL), and if the effect is achieved at physiological dosages. LDL oxidizability was assessed by the resistance to oxidation by copper and by the particle size profile, since small particles have increased oxidation susceptibility. Methods: Thirty-three women received two consecutive, two-month length doses of 1 and 2 mg/day of oral E 2 . They were then randomly assigned to a fourteen-day treatment of 2 mg/day E 2 plus either 300 mg/day P or 5 mg/day MPA. A parallel group of experiments …

Towards the tailoring of glucocorticoid replacement in adrenal insufficiency: the Italian Society of Endocrinology Expert Opinion.

2020

Context: Glucocorticoid (GC) replacement therapy in patients with adrenal insufficiency (AI) is life saving. After over 50 years of conventional GC treatment, novel formulations are now entering routine clinical practice. Methods: Given the spectrum of medications currently available and new insights into the understanding of AI, the authors reviewed relevant medical literature with emphasis on original studies, prospective observational data and randomized controlled trials performed in the past 35 years. The Expert Opinion of a panel of selected endocrinologists was sought to answer specific clinical questions. The objective was to provide an evidence-supported guide, for the use of GC in…

Retrospective Analysis of Whole-Body Magnetic Resonance Imaging of Bone Manifestations in Long-Term Treated Patients with Gaucher Disease Type 1

2018

We retrospectively assessed bone and visceral manifestations in patients with Gaucher disease type 1 (GD1) with whole-body magnetic resonance imaging (WB-MRI) to determine the effects of different timing in initiating long-term enzyme replacement therapy.In 17 patients with GD1, we performed 2 WB-MRI examinations at a median interval of 13 months. Patients had received enzyme replacement therapy with alglucerase/imiglucerase for a median of 13 years prior to the first examination. MRI results were retrospectively stratified based on treatment initiation into 2 groups: "early" (age ≤12 years, median 5 years) and "late" (during adulthood, median 32 years). We evaluated occurrence of irreversi…

OARSI/OMERACT Initiative to Define States of Severity and Indication for Joint Replacement in Hip and Knee Osteoarthritis. An OMERACT 10 Special Inte…

2011

Objective.To define pain and physical function cutpoints that would, coupled with structural severity, define a surrogate measure of “need for joint replacement surgery,” for use as an outcome measure for potential structure-modifying interventions for osteoarthritis (OA).Methods.New scores were developed for pain and physical function in knee and hip OA. A cross-sectional international study in 1909 patients was conducted to define data-driven cutpoints corresponding to the orthopedic surgeons’ indication for joint replacement. A post hoc analysis of 8 randomized clinical trials (1379 patients) evaluated the prevalence and validity of cutpoints, among patients with symptomatic hip/knee OA.…

Intrarenal venous flow in cardiorenal syndrome: a shining light into the darkness

2018

The aim of this case report is to assess the potential role of intrarenal Doppler ultrasonography as a non‐invasive method to evaluate intrarenal venous flow (IRVF) in acute heart failure (AHF) and concomitant renal dysfunction. We report a case of an 81‐year‐old woman with valvular heart disease (previous mitral valve replacement) that presented with acutely decompensated heart failure and concomitant worsening renal function (WRF). In addition to complete physical examination, laboratory analysis, and echocardiography, IRVF was assessed at baseline and 48 h after the administration of diuretic treatment. At admission, physical examination and echocardiography revealed signs of intravascul…

Effects of balance and proprioceptive training on total hip and knee replacement rehabilitation: A systematic review and meta-analysis.

2017

Abstract Backgound Balance and proprioceptive deficits are frequently persistent after total joint replacement, limiting functionality and involving altered movement patterns and difficulties in walking and maintaining postural control among patients. Research question The goal of this systematic review was to evaluate the short- and mid-term effects of proprioceptive and balance training for patients undergoing total knee and hip replacement. Methods This is a systematic review of literature. MEDLINE, Embase, Cochrane Library, PEDro, and Scopus were the databases searched. The review included randomized clinical trials in which the experimental groups underwent a training aimed at improvin…