ABSTRACT
AIMS OF THE STUDY: Systemic amyloidoses are rare protein-folding diseases with heterogeneous, often nonspecific clinical presentations. To better understand systemic amyloidoses and to apply state-of-the-art diagnostic pathways and treatment, the interdisciplinary Amyloidosis Network was founded in 2013 at University Hospital Zurich. In this respect, a registry was implemented to study the characteristics and life expectancy of patients with amyloidosis within the area covered by the network. Patient data were collected retrospectively for the period 2005-2014 and prospectively from 2015 onwards. METHODS: Patients aged 18 years or older diagnosed with any subtype of systemic amyloidosis were eligible for inclusion if they were treated in one of the four referring centres (Zurich, Chur, St Gallen, Bellinzona). Baseline data were captured at the time of diagnosis. Follow-up data were assessed half-yearly for the first two years, then annually. RESULTS: Between January 2005 and March 2020, 247 patients were screened, and 155 patients with confirmed systemic amyloidosis were included in the present analysis. The most common amyloidosis type was light-chain (49.7%, n = 77), followed by transthyretin amyloidosis (40%, n = 62) and amyloid A amyloidosis (5.2%, n = 8). Most patients (61.9%, n = 96) presented with multiorgan involvement. Nevertheless, single organ involvement was seen in all types of amyloidosis, most commonly in amyloid A amyloidosis (75%, n = 6). The median observation time of the surviving patients was calculated by the reverse Kaplan-Meier method and was 3.29 years (95% confidence interval [CI] 2.33-4.87); it was 4.87 years (95% CI 3.14-7.22) in light-chain amyloidosis patients and 1.85 years (95% CI 1.48-3.66) in transthyretin amyloidosis patients, respectively. The 1-, 3- and 5-year survival rates were 87.0% (95% CI 79.4-95.3%), 68.5% (95% CI 57.4-81.7%) and 66.0% (95% CI 54.6-79.9%) respectively for light-chain amyloidosis patients and 91.2% (95% CI 83.2-99.8%), 77.0% (95% CI 63.4-93.7%) and 50.6% (95% CI 31.8-80.3%) respectively for transthyretin amyloidosis patients. There was no significant difference between the two groups (p = 0.81). CONCLUSION: During registry set-up, a more comprehensive work-up of our patients suffering mainly from light-chain amyloidosis and transthyretin amyloidosis was implemented. Survival rates were remarkably high and similar between light-chain amyloidosis and transthyretin amyloidosis, a finding which was noted in similar historic registries of international centres. However, further studies are needed to depict morbidity and mortality as the amyloidosis landscape is changing rapidly.
Subject(s)
Amyloid Neuropathies, Familial , Amyloidosis , Humans , Amyloid Neuropathies, Familial/diagnosis , Amyloid Neuropathies, Familial/metabolism , Amyloid Neuropathies, Familial/therapy , Registries , Retrospective Studies , Serum Amyloid A Protein , Switzerland/epidemiology , AdultABSTRACT
PURPOSE: The aim of this study was to assess safety and efficacy of vertebral body stenting (VBS) by analyzing (1) radiographic outcome, (2) clinical outcome, and (3) perioperative complications in patients with vertebral compression fractures treated with VBS at minimum 6-month follow-up. METHODS: In this retrospective cohort study, 78 patients (61 ± 14 [21-90] years; 67% female) who have received a vertebral body stent due to a traumatic, osteoporotic or metastatic thoracolumbar compression fracture at our hospital between 2012 and 2020 were included. Median follow-up was 0.9 years with a minimum follow-up of 6 months. Radiographic and clinical outcome was analyzed directly, 6 weeks, 12 weeks, 6 months postoperatively, and at last follow-up. RESULTS: Anterior vertebral body height of all patients improved significantly by mean 6.2 ± 4.8 mm directly postoperatively (p < 0.0001) and remained at 4.3 ± 5.1 mm at last follow-up compared to preoperatively (p < 0.0001). The fracture kyphosis angle of all patients improved significantly by mean 5.8 ± 6.9 degrees directly postoperatively (p < 0.0001) and remained at mean 4.9 ± 6.9 degrees at last follow-up compared to preoperatively (p < 0.0001). The segmental kyphosis angle of all patients improved significantly by mean 7.1 ± 7.6 degrees directly postoperatively (p < 0.0001) and remained at mean 2.8 ± 7.8 degrees at last follow-up compared to preoperatively (p = 0.03). Back pain was ameliorated from a preoperative median Numeric Rating Scale value of 6.5 to 3.0 directly postoperatively and further bettered to 1.0 six months postoperatively (p = 0.0001). Revision surgery was required in one patient after 0.4 years. CONCLUSION: Vertebral body stenting is a safe and effective treatment option for osteoporotic, traumatic and metastatic compression fractures.
Subject(s)
Fractures, Compression , Kyphosis , Spinal Fractures , Humans , Female , Male , Spinal Fractures/diagnostic imaging , Spinal Fractures/surgery , Spinal Fractures/complications , Fractures, Compression/diagnostic imaging , Fractures, Compression/surgery , Vertebral Body , Retrospective Studies , Thoracic Vertebrae/diagnostic imaging , Thoracic Vertebrae/surgery , Thoracic Vertebrae/injuries , Lumbar Vertebrae/diagnostic imaging , Lumbar Vertebrae/surgery , Lumbar Vertebrae/injuries , Kyphosis/surgery , Stents/adverse effectsABSTRACT
BACKGROUND: Theta burst stimulation (TBS) is a non-invasive brain stimulation method. Various stimulation protocols have been proposed, for instance, stimulation at 50 Hz with pattern at 5 Hz, or at 30 Hz with pattern at 6 Hz. To identify better stimulation parameters for behavioral applications, we investigated the effects of 50-Hz continuous TBS (cTBS) on the sense of agency (SoA), and compared them with a previously published study with 30-Hz cTBS. METHODS: Based on power analysis from a previous sample using two applications of 30-Hz cTBS, we recruited 20 healthy subjects in a single-blind, Vertex-controlled, randomized, crossover trial. Participants were stimulated with one application of 50-Hz cTBS over the right posterior parietal cortex (rPPC), a key area for agency processing, and the vertex, in a random order. A behavioral task targeting the SoA was done before and after stimulation. After controlling for baseline differences across samples, we studied the effect of stimulation in the two protocols separately. RESULTS: Compared to the previously published 30-Hz protocol, 50-Hz cTBS over the rPPC did not reveal significant changes in the SoA, similar to sham Vertex stimulation. CONCLUSION: One application of 50-Hz cTBS was not sufficient to elicit behavioral effects, compared to two applications of 30-Hz cTBS, as previously described. This may be due to a mechanism of synaptic plasticity, consolidated through consecutive stimulation cycles. Our results are relevant for future studies aiming at modulating activity of the rPPC in cognitive domains other than agency, and in patients affected by abnormal agency, who could benefit from treatment options based on TBS.