Search results for "Amide"
showing 10 items of 3119 documents
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 …
Sirolimus versus cyclosporine in haploidentical stem cell transplantation with posttransplant cyclophosphamide and mycophenolate mofetil as graft‐ver…
2021
Sirolimus has emerged as an alternative to calcineurin inhibitors-based (CNI) graft-versus-host disease (GVHD) prophylaxis. This retrospective study compares the outcome of 133 consecutive adult patients with haematological malignancies undergoing haploidentical stem cell transplantation with posttransplant cyclophosphamide (PTCy) and mycophenolate mofetil (MMF), combined with cyclosporine A (PTCy-CsA-MMF,
Immunochemical characterization of anti-acetylcholine receptor antibodies in primary biliary cirrhosis
1988
Although the presence of anti-mitochondrial antibodies is the main characteristic of primary biliary cirrhosis (PBC), other autoantibodies have been described in this disease. This study employs immunoblot methods to test whether the sera of PBC patients also contain antibodies directed against nicotinic acetylcholine receptors (AChR). We show that the majority of patients' sera indeed react with AChR just as sera of myasthenic patients do. In contrast, however, these anti-AChR antibodies do not lead to significant clinical symptoms of myasthenia. In all cases studied, PBC sera recognized a protein with the molecular weight of the alpha-chain of acetylcholine receptor (40 kDa). In addition,…
Prävention des früh-postoperativen IOD-Anstiegs nach Phakoemulsifikation
2001
Purpose. To compare the ocular hypotensive effect of different antiglaucomatous eye drops. Patients and methods. This double-blind, randomized study included 119 eyes of 119 patients without other ocular pathology undergoing standardized (one surgeon, Healon) small incision cataract surgery with foldable intraocular lens implantation.The patients were assigned to one of five groups: group 1:0.25% timolol in Gelrite (extended efficacy, n=23); group 2: 2% dorzolamide (n=24); group 3: combination of 0.5% timolol plus 2% dorzolamide (n=22); group 4: brimonidine (n=26); group 5: gentamicin (control, n=24). Intraocular pressure (IOP) was measured preoperatively and at 3±1,6±1,9±1,24±3, and 48±3 h…
Microscopic Endonasal Surgery for Repair of CSF Leaks
1993
This manuscript will detail our experience using a microscopic endonasal surgery (MES) approach in the repair of spontaneous, traumatic, and/or iatrogenic cerebrospinal fluid (CSF) fistula into the ethmoid and sphenoid sinuses. The location of the fistula in 22 patients was determined by a direct endoscopic or microscopic visualization, intrathecal fluorescein, or computed tomography (CT) with metrizamide. Several of these patients had been treated unsuccessfully with previous neurosurgical, intracranial, and extracranial approaches to stop the leak. The CSF fistulas in all these patients were repaired with a MES approach, packing the leak with either a septal mucosal graft, or abdominal f…
Die retraktile Mesenteritis*: Diagnostische und therapeutische Aspekte
2008
An 18-year-old boy, who had severe abdominal pain for 18 months associated with marked weight loss, was found to have a stenosed ileal sling on double-contrast radiology of the small intestine. At operation a plate-like tumour was extending from the pancreas to the aortic bifurcation. Histological examination of removed tissue revealed retractile mesenteritis (mesenteric panniculitis; liposclerotic mesenteritis). The symptoms regressed and the patient again gained weight under immunotherapy with 1 mg/kg of prednisone and 2 mg/kg of cyclophosphamide daily. Subsequently, under prednisone alone, there was a recurrence, which responded within five weeks to daily 60 mg prednisone and 125 mg cycl…
Exploring gastric emptying rate in minipigs: Effect of food type and pre-dosing of metoclopramide.
2018
The present study investigated the gastric emptying rate in Gottingen minipigs pre- and post-prandial and evaluated the effect of metoclopramide on the same parameter, using paracetamol as an absorption marker. The pharmacokinetic evaluation of the obtained plasma concentration data for paracetamol demonstrated that the fastest gastric emptying rate was observed in the animals that were allowed access to normal pig food. There was no significant difference in the stomach emptying rate observed between fasted and fed minipigs, when fed either with a FDA standard breakfast or a nutritional energy drink. Pre-dosing minipigs with metoclopramide (0.2 or 0.4 mg/kg) did not demonstrate any effect …
Assessment of Mobilization Cost for Multiple Myeloma Using 2 Different Mobilization Strategies: High-Dose Cyclophosphamide Versus Plerixafor. on Beha…
2016
Abstract Background. Peripheral blood stem cell (PBSC) collection prior to high dose chemotherapy for autologous transplantation (ASCT) is a standard of care, and an attractive alternative to the use of bone marrow cells, for transplantation in Multiple Myeloma (MM). The optimal methodology for mobilizing PBSC has yet to be defined, both G-CSF and GM-CSF can be used; although, the stimulatory effect may be more pronounced when given after high dose cyclophosphamide (usually administered at a dose of 1.5 to 6g/m2 IV for one to two days) and use of Plerixafor, a CXCR4 antagonist (Mozobil®). The latter 2 options are preferred, overall, in France. Indeed, it was shown that the most recent combi…
Combination of Rehabilitative Therapy with Ultramicronized Palmitoylethanolamide for Chronic Low Back Pain: An Observational Study
2019
Abstract Introduction Chronic low back pain (LBP) caused by intervertebral disc herniation was reported in the 2010 Global Burden of Disease study to be the main reason for years lived with disability. It causes significant personal, social, and economic burdens. Many of those who suffer from LBP find conventional medical treatments to be unsatisfactory for treating their pain, so they are increasingly resorting to complementary and alternative medicine (CAM) therapies. Given that the population is aging, there is an urgent need to characterize the combinations of complementary therapies that yield the best outcomes and treatments, even for prolonged periods. This observational study aimed …