339 research outputs found
The Jervell and Lange-Nielsen syndrome; atrial pacing combined with Ă-blocker therapy, a favorable approach in young high-risk patients with long QT syndrome?
BackgroundPatients with Jervell and Lange-Nielsen syndrome (JLNS) exhibit severe phenotypes that are characterized by congenital deafness, very long QT intervals, and high risk of life-threatening arrhythmias. Current treatment strategies include high doses of beta-blocker medication, left cardiac sympathetic denervation, and ICD placement, which is challenging in young children.ObjectiveThe purpose of this study was to evaluate the safety and effect of pacing in addition to beta-blocker treatment in children with JLNS.MethodsAll genetically confirmed patients with JLNS born since 1999 in Norway were included in the study. Data on history of long QT syndromeârelated symptoms, QT interval, and beta-blocker and pacemaker treatment were recorded.ResultsA total of 9 patients with QT intervals ranging from 510 to 660 ms were identified. Eight patients developed long QT syndromeârelated symptoms, and 1 patient died before diagnosis. The survivors received beta-blocker medication. Seven patients also received a pacemaker; 1 had a ventricular lead and 6 had atrial leads. The patient with the ventricular lead died during follow-up. The 6 patients with atrial leads survived without events at a mean follow-up of 6.9 years after pacemaker implantation. Two patients received prophylactic upgrade to a 2-chamber ICD.ConclusionNo arrhythmic events occurred in 6 very young JLNS patients who received atrial pacing in combination with increased doses of beta-blockers during 7-year follow-up. If confirmed in additional patients, this treatment strategy may prevent life-threatening arrhythmias in this high-risk patient group and may act as a bridge to insertion of a 2-chamber ICD when left cardiac sympathetic denervation is not available
Quasicontinuum -decay of Zr: benchmarking indirect () cross section measurements for the -process
Nuclear level densities (NLDs) and -ray strength functions
(SFs) have been extracted from particle- coincidences of the
Zr()Zr and Zr()Zr
reactions using the Oslo method. The new Zr SF data, combined
with photonuclear cross sections, cover the whole energy range from ~MeV up to the giant dipole resonance at ~MeV. The wide-range SF data display structures at ~MeV, compatible with a superposition of the spin-flip
resonance and a pygmy resonance. Furthermore, the SF shows a
minimum at ~MeV and an increase at lower -ray
energies. The experimentally constrained NLDs and SFs are shown to
reproduce known () and Maxwellian-averaged cross sections for
Zr using the {\sf TALYS} reaction code, thus serving as a benchmark
for this indirect method of estimating () cross sections for Zr
isotopes.Comment: 10 pages and 9 figure
Extraction of thermal and electromagnetic properties in 45Ti
The level density and gamma-ray strength function of 45Ti have been
determined by use of the Oslo method. The particle-gamma coincidences from the
46Ti(p,d gamma)45Ti pick-up reaction with 32 MeV protons are utilized to obtain
gamma-ray spectra as function of excitation energy. The extracted level density
and strength function are compared with models, which are found to describe
these quantities satisfactorily. The data do not reveal any single-particle
energy gaps of the underlying doubly magic 40Ca core, probably due to the
strong quadruple deformation
Observation of Thermodynamical Properties in the Dy, Er and Yb Nuclei
The density of accessible levels in the (He,) reaction has
been extracted for the Dy, Er and Yb nuclei. The
nuclear temperature is measured as a function of excitation energy in the
region of 0 -- 6 MeV. The temperature curves reveal structures indicating new
degrees of freedom. The heat capacity of the nuclear system is discussed within
the framework of a canonical ensemble.Comment: 12 pages, 4 figures include
- âŠ