ORCID Profile
0000-0002-9253-9921
Current Organisation
Medizinische Universität Wien
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Publisher: BMJ
Date: 10-11-2016
Publisher: Informa UK Limited
Date: 04-05-2018
Publisher: Wiley
Date: 28-03-2019
DOI: 10.1002/AJMG.A.61105
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 2012
DOI: 10.2215/CJN.03130411
Abstract: Fabry disease is a rare X-linked disease with multisystemic manifestations. This study investigated the effectiveness of long-term enzyme replacement therapy with agalsidase alfa in Fabry nephropathy treatment. In this observational study, data on patients receiving agalsidase alfa (0.2 mg/kg every other week) were extracted from the Fabry Outcome Survey, an international registry of patients with Fabry disease. Serum creatinine and estimated GFR (eGFR) at baseline and after ≥5 years of treatment were assessed 24-hour urinary protein excretion and BP measurements were also reviewed. The eGFR was calculated using the Chronic Kidney Disease Epidemiology Collaboration formula. Patients with an eGFR ml/min per 1.73 m 2 were excluded. Renal function was assessed in 208 patients (mean enzyme replacement therapy, 7.4 years range, 5.0–11.2 years). Mean yearly change in eGFR was −2.2 ml/min per 1.73 m 2 in men and −0.7 ml/min per 1.73 m 2 in women (95% confidence limits, −2.8 −1.7 and −1.4 0.0, respectively). Patients with 24-hour protein excretion g/24 h had poorer renal function at baseline and follow-up compared with patients with protein excretion of 500–1000 mg/24 h or with proteinuria mg/24 h. Renal function was worse in patients with baseline arterial hypertension, and there was a more rapid yearly decline compared with normotensive patients. This study suggests that long-term agalsidase alfa therapy is able to stabilize the rate of Fabry nephropathy progression in women and is associated with a mild to moderate decline of renal function in men.
Publisher: BMJ
Date: 10-11-2010
Publisher: Massachusetts Medical Society
Date: 26-12-2013
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 27-10-2004
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 04-2000
DOI: 10.1097/00000539-200004000-00003
Abstract: Infraclavicular and internal jugular central venous access are techniques commonly used for temporary transvenous pacing. However, the procedure still has a considerable complication rate, with a high risk/benefit ratio because of insertion difficulties and pacemaker malfunction. To enlarge the spectrum of alternative access sites, we prospectively evaluated the right supraclavicular route to the subclavian/innominate vein for emergency ventricular pacing with a transvenous flow-directed pacemaker as a bedside procedure. For 19 mo, 17 consecutive patients with symptomatic bradycardia, cardiac arrest, or torsade de pointes requiring immediate bedside transvenous pacing were enrolled in the study. The success rate, insertional complications, pacemaker performance, and patients' outcomes were recorded. Supraclavicular venipuncture was successful in all patients, in 16 of 17 at the first attempt. Adequate ventricular pacing was achieved within 1 to 5 min (median, 2 min) after venipuncture and within 10 s to 4 min (median, 30 s) after lead insertion (</=30 s in 15 of 17 patients). The median pacing threshold was 1 mA (range, 0.7 to 2.5 mA). No procedure-related complications were recorded. Throughout the pacing period of 1538 h (median: 62 h, range, 1-280 h) two reversible malfunctions caused by inadvertent lead dislodgement after 122 and 171 h were recorded in one patient the pacemaker had to be removed because of local infection after 14 days of pacing. We conclude that the right supraclavicular route is an easy, safe, and effective first approach for transvenous ventricular pacing and might provide a useful alternative to traditional puncture sites, even in a preclinical setting. Temporary transvenous cardiac pacing can yield high complication rates especially under emergency conditions. We investigated emergency pacing via the right supraclavicular access in 17 consecutive hemodynamically compromised patients and found good safety, efficacy, and a low complication rate.
Publisher: SAGE Publications
Date: 19-09-2012
DOI: 10.5301/JN.5000214
Abstract: During Fabry disease, progressive glycosphingolipid deposition in the kidney causes gradual deterioration of renal function with proteinuria, uremia and hypertension. This results in end-stage renal disease (ESRD) which is one of the leading causes of morbidity and premature mortality in affected patients. Given the excellent graft and patient survival generally nowadays, kidney transplantation is the first choice to correct renal dysfunction and improve the overall prognosis of patients with renal failure because of Fabry disease. The benefit of enzyme-replacement therapy (ERT) in kidney transplanted Fabry patients has been controversially discussed and long-term trials focusing on the effectiveness of agalsidase in this patient population are needed.
No related grants have been discovered for Gere Sunder-Plassmann.