Myocardial tissue characterization in patients with hereditary gelsolin (AGel) amyloidosis using novel cardiovascular magnetic resonance techniques

Gelsolin (AGel) amyloidosis is a hereditary condition with common neurological effects. Myocardial involvement, especially strain, T1, or extracellular volume (ECV), in this disease has not been investigated before. Local myocardial effects and possible amyloid accumulation were the targets of interest in this study. Fifty patients with AGel amyloidosis were enrolled in the study. All patients underwent cardiovascular magnetic resonance imaging, including cine imaging, T1 mapping, tagging, and late gadolinium enhancement (LGE) imaging at 1.5 T. Results for volumetry, myocardial feature-tracking strain, rotation, torsion, native T1, ECV, and LGE were investigated. The population mean native T1 values in different segments of the left ventricle (LV) varied between 1003 and 1080 ms. Myocardial mean T1 was 1031 ± 37 ms. T1 was highest in the basal plane of the LV (1055 ± 40 ms), similarly to ECV (30.0% ± 4.4%). ECV correlated with native T1 in all LV segments (p Peer reviewe

Lasten yksityisen hoidon tuen kaksi vuosikymmentä

Tässä tutkimuksessa tarkastellaan yksityisen hoidon tuen kehitystä tukimuodon käyttöön-otosta vuonna 1997 vuoteen 2017. Tutkimusaineiston muodostavat 1–5-vuotiaiden lasten perheet, joiden kaikki lapset ovat siirtyneet kodin ulkopuoliseen hoitoon, joko kunnalliseen tai yksityiseen varhaiskasvatukseen. Tutkimuksessa selvitetään millaiset perheet käyttävät yksityisen hoidon tukea sekä yksityisen hoidon tuella hankittujen yksityisten varhaiskasvatuspalveluiden hintaa perheille. Lisäksi tutkimuksessa arvioidaan yksityisen hoidon tuelle hallituksen esityksessä HE 208/1996 asetettujen tavoitteiden toteutumista. Yksityisen hoidon tuki otettiin käyttöön vuonna 1997. Yksityisen hoidon tuella perheet voivat ostaa varhaiskasvatuspalveluita yksityisiltä palveluntuottajilta. Kunta voi halutessaan maksaa perheille kuntalisää yksityisen hoidon tuen lakisääteisten osien, hoitorahan ja hoitolisän, li-säksi. 2000-luvun aikana kuntalisää tarjoavien kuntien lukumäärä kasvoi, mikä osittain no-peutti yksityisten varhaiskasvatuspalveluiden yleistymistä. Vuodesta 2009 lähtien kunnat ovat voineet tarjota perheille palveluseteliä yksityisten varhaiskasvatuspalveluiden ostamiseen. Pienituloisille perheille suunnatun yksityisen hoidon tuen hoitolisän merkitys on vähentynyt, kun taas kuntalisän merkitys on kasvanut. Vuosina 1997–1998 noin 30 prosenttia tukea saavista perheistä sai hoitolisää, vuonna 2000 hoitolisää sai 24 prosenttia perheistä ja vuodesta 2010 eteenpäin enää alle 10 prosenttia perheistä. Toisaalta 2000-luvun alussa kuntalisää sai noin 60 prosenttia yksityisen hoidon tukea saavista perheistä. Vuonna 2017 kuntalisää sai 88 prosenttia perheistä. Tulosten perusteella yksityisen hoidon tuki on ollut koko 2000-luvun suurituloisille perheille keskittynyt tukimuoto, mutta toisaalta sosioekonomiset erot yksityisen hoidon tuen käytössä eivät ole kasvaneet. Koko 2000-luvun ajan noin 40 prosenttia yksityisen hoidon tukea käyttäneistä perheistä kuului ylimpään tuloviidennekseen ja 10 prosenttia alimpaan tuloviidennekseen. Yksityisen hoidon tuella ostettiin keskimäärin kalliimpia varhaiskasvatuspalveluita 2010-lu-vulla kuin 2000-luvun alussa. Vuonna 2007 yhden lapsen hoidon keskihinta oli 642 euroa kuu-kaudessa vuoden 2017 hinnoissa, mutta vuonna 2017 keskihinta oli 860 euroa kuukaudessa lapselta. Suurituloiset perheet ostivat keskimäärin kalliimpia palveluita kuin pienituloiset perheet.nonPeerReviewedVertaisarvioimato

Cardiac manifestations in Finnish gelsolin amyloidosis patients

Introduction Finnish gelsolin amyloidosis (AGel amyloidosis) is an inherited systemic amyloidosis with well-known ophthalmological, neurological and cutaneous symptoms. Additionally, cardiomyopathies, conduction disorders and need of cardiac pacemakers occur in some patients. This study focuses on electrocardiographic (ECG) findings in AGel amyloidosis and their relation to cardiac magnetic resonance (CMR) changes. We also assessed whether ECG abnormalities were associated with pacemaker implantation and mortality. Materials and methods In this cohort study, 51 genetically verified AGel amyloidosis patients (mean age 66 years) without cardiac pacemakers underwent 12-lead ECG and CMR imaging with contrast agent in 2017. Patients were followed-up for 3 years. Results Conduction disturbances were found in 22 patients (43%). Nine (18%) presented with first-degree atrioventricular block, six (12%) with left anterior hemiblock, seven (14%) with left or right bundle branch block and two (4%) with non-specific intraventricular conduction delay. Low QRS voltage was present in two (4%) patients. Late gadolinium enhancement (LGE) concentrating on the interventricular septum and inferior parts of the heart was present in 19 (86%) patients with conduction abnormalities. During the follow-up, only one patient received a pacemaker, and one patient died. Discussion Conduction disorders and septal LGE are common in AGel amyloidosis, whereas other ECG and CMR findings typically observed in most common cardiac amyloidosis types were rare. Septal pathology seen in CMR may interfere with the cardiac conduction system in AGel amyloidosis, explaining conduction disorders, although pacemaker therapy is rarely required.Peer reviewe

Sydänamyloidoosi

English summaryPeer reviewe

The type of the functional cardiovascular response to upright posture is associated with arterial stiffness: a cross-sectional study in 470 volunteers

In a cross-sectional study we examined whether the haemodynamic response to upright posture could be divided into different functional phenotypes, and whether the observed phenotypes were associated with known determinants of cardiovascular risk. METHODS: Volunteers (n = 470) without medication with cardiovascular effects were examined using radial pulse wave analysis, whole-body impedance cardiography, and heart rate variability analysis. Based on the passive head-up tilt induced changes in systemic vascular resistance and cardiac output, the principal determinants of blood pressure, a cluster analysis was performed. RESULTS: The haemodynamic response could be clustered into 3 categories: upright increase in vascular resistance and decrease in cardiac output were greatest in the first (+45 % and -27 %, respectively), smallest in the second (+2 % and -2 %, respectively), and intermediate (+22 % and -13 %, respectively) in the third group. These groups were named as 'constrictor' (n = 109), 'sustainer' (n = 222), and 'intermediate' (n = 139) phenotypes, respectively. The sustainers were characterized by male predominance, higher body mass index, blood pressure, and also by higher pulse wave velocity, an index of large arterial stiffness, than the other groups (p < 0.01 for all). Heart rate variability analysis showed higher supine and upright low frequency/high frequency (LF/HF) ratio in the sustainers than constrictors, indicating increased sympathovagal balance. Upright LF/HF ratio was also higher in the sustainer than intermediate group. In multivariate analysis, independent explanatory factors for higher pulse wave velocity were the sustainer (p < 0.022) and intermediate phenotypes (p < 0.046), age (p < 0.001), body mass index (p < 0.001), and hypertension (p < 0.001). CONCLUSIONS: The response to upright posture could be clustered to 3 functional phenotypes. The sustainer phenotype, with smallest upright decrease in cardiac output and highest sympathovagal balance, was independently associated with increased large arterial stiffness. These results indicate an association of the functional haemodynamic phenotype with an acknowledged marker of cardiovascular risk. TRIAL REGISTRATION: ClinicalTrials.gov NCT01742702.BioMed Central open acces

Association of resting heart rate with cardiovascular function: a cross-sectional study in 522 Finnish subjects

ad-up tilt. Methods Hemodynamics were recorded using whole-body impedance cardiography and continuous radial pulse wave analysis in 522 subjects (age 20–72 years, 261 males) without medication influencing HR or BP, or diagnosed diabetes, coronary artery, renal, peripheral arterial, or cerebrovascular disease. Correlations were calculated, and results analysed according to resting HR tertiles. Results Higher resting HR was associated with elevated systolic and diastolic BP, lower stroke volume but higher cardiac output and work, and lower systemic vascular resistance, both supine and upright (p < 0.05 for all). Subjects with higher HR also showed lower supine and upright aortic pulse pressure and augmentation index, and increased resting pulse wave velocity (p < 0.001). Upright stroke volume decreased less in subjects with highest resting HR (p < 0.05), and cardiac output decreased less in subjects with lowest resting HR (p < 0.009), but clear hemodynamic differences between the tertiles persisted both supine and upright. Conclusions Supine and upright hemodynamic profile associated with higher resting HR is characterized by higher cardiac output and lower systemic vascular resistance. Higher resting HR was associated with reduced central wave reflection, in spite of elevated BP and arterial stiffness. The increased cardiac workload, higher BP and arterial stiffness, may explain why higher HR is associated with less favourable prognosis in populations.BioMed Central open acces

Final report on dissemination, regulation, standardization, exploitation & training : D6.3

In D6.1 deliverable project dissemination, exploitation and training plans, as well as standardization & regulatory approach strategy was presented. The D6.2 reported on the necessary updates of these strategies and the actions taken by the partners in line with them, as well as the obtained results. In this D6.3 deliverable, a full set of project dissemination activities, standardization & regulatory contributions as well as an operator’s “cook book” outlining steps necessary for full deployment of ON functionality and services, are presented.Deliverable D6.3 del projecte OneFITPostprint (author’s final draft

Business scenarios, technical challenges and system requirements - D2.1

Deliverable D2.1 del projecte Europeu OneFIT (ICT-2009-257385)Preprin

Formulation, implementation considerations, and first performance evaluation of algorithmic solutions - D4.1

Deliverable D4.1 del projecte Europeu OneFIT (ICT-2009-257385)This deliverable contains a first version of the algorithmic solutions for enabling opportunistic networks. The presented algorithms cover the full range of identified management tasks: suitability, creation, QoS control, reconfiguration and forced terminations. Preliminary evaluations complement the proposed algorithms. Implementation considerations towards the practicality of the considered algorithms are also included.Preprin