A very exotic process of β-delayed fission of 180Tl is studied in detail by using resonant laser ionization with subsequent mass separation at ISOLDE (CERN). In contrast to common expectations, the ...fission-fragment mass distribution of the post-β-decay daughter nucleus 180Hg (N/Z=1.25) is asymmetric. This asymmetry is more surprising since a mass-symmetric split of this extremely neutron-deficient nucleus would lead to two 90Zr fragments, with magic N=50 and semimagic Z=40. This is a new type of asymmetric fission, not caused by large shell effects related to fragment magic proton and neutron numbers, as observed in the actinide region. The newly measured branching ratio for β-delayed fission of 180Tl is 3.6(7) × 10(-3)%, approximately 2 orders of magnitude larger than in an earlier study.
Iascone M, Ciccone R, Galletti L, Marchetti D, Seddio F, Lincesso AR, Pezzoli L, Vetro A, Barachetti D, Boni L, Federici D, Soto AM, Comas JV, Ferrazzi P, Zuffardi O. Identification of de novo ...mutations and rare variants in hypoplastic left heart syndrome.
Hypoplastic left heart syndrome (HLHS) is one of the most severe congenital heart malformations, characterized by underdevelopment of the structures in the left heart–aorta complex. The majority of cases are sporadic. Although multiple genetic loci have been tentatively implicated in HLHS, no gene or pathway seems to be specifically associated with the disease. To elucidate the genetic basis of HLHS, we analyzed 53 well‐characterized patients with isolated HLHS using an integrated genomic approach that combined DNA sequencing of five candidate genes (NKX2‐5, NOTCH1, HAND1, FOXC2 and FOXL1) and genome‐wide screening by high‐resolution array comparative genomic hybridization. In 30 patients, we identified two novel de novo mutations in NOTCH1, 23 rare patients inherited gene variants in NOTCH1, FOXC2 and FOXL1, and 33 rare patients mostly inherited copy‐number variants. Some of the identified variations coexisted in the same patient. The biological significance of such rare variations is unknown, but our findings strengthen the role of NOTCH pathway in cardiac valve development, indicating that HLHS is, at least in part, a ‘valve’ disease. This is the first report of de novo mutations associated with isolated HLHS. Moreover, the coexistence of multiple rare variants suggests in some cases a cumulative effect, as shown for other complex disease.
In deep inelastic multinucleon transfer reactions of 48Ca + 248Cm we observed about 100 residual nuclei with proton numbers between Z=82 and Z=100. Among them, there are five new neutron-deficient ...isotopes: 216U, 219Np, 223Am, 229Am and 233Bk. As separator for the transfer products we used the velocity filter SHIP of GSI while the isotope identification was performed via the α decay chains of the nuclei. These first results reveal that multinucleon transfer reactions together with here applied fast and sensitive separation and detection techniques are promising for the synthesis of new isotopes in the region of heaviest nuclei.
OBJECTIVES
Right ventricular (RV) dysfunction and pulmonary insufficiency (PI) after tetralogy of Fallot (TOF) repair may contribute to early and late morbidity and mortality. RV dysfunction may be ...related to the ventriculotomy employed in the transventricular repair technique, particularly when it is combined with a transannular patch (TAP). Transatrial/transpulmonary (TA/TP) repair without ventriculotomy has been advocated as a method potentially diminishing such adverse events. However, the prevalence and early as well as the late results of these different surgical approaches in Europe have not been studied. To ascertain 'the current prevalence and associated early mortality' of various surgical approaches for repair of TOF, relevant data in the European Association for Cardio-Thoracic Surgery Congenital Database were analysed.
METHODS
The study population was all types of reparative operations (n = 6654) for patients with primary diagnosis of TOF reported between 1999 and 2011.
RESULTS
Overall hospital mortality (HM) was 2.58% (172 of 6654). Repair via ventriculotomy with TAP was the most prevalent technique (n = 3827, 57.5%), with HM 3.11%. Repair via ventriculotomy with non-TAP was performed in 1309 patients (19.7%, HM = 1.53%). Repair without ventriculotomy was performed in 1214 patients (18.2%, HM = 1.48%). Logistic regression analysis showed statistically significant association between HM risk and the type of surgery. In particular, ventriculotomy with TAP is associated with increased mortality risk significantly compared with ventriculotomy with non-TAP (crude odds ratio OR 2.13 (95% confidence interval CI: 1.33-3.63). Similar results were obtained by analysing for operative mortality risk (30-day mortality, OM). Operations that have been performed before 2005 have resulted in increased surgical risk compared with those performed after 2005 (ORs for OM 1.45, 95% CI: 1.03-2.013).
CONCLUSIONS
Overall HM for TOF repair is low. TOF repair by means of ventriculotomy with TAP is the most prevalent approach and is associated with higher mortality. Repair with ventriculotomy but no TAP and repair without ventriculotomy are both less prevalent and with lower mortality. Surgical risk appears to be decreasing over time.
OBJECTIVES
This study focuses on the different surgical and endoscopical treatment alternatives when dealing with severe complications after slide tracheoplasty (STP).
METHODS
Retrospective study of ...patients with symptomatic congenital tracheal stenosis (CTS) admitted to a single institution, between January 1997 and January 2013, surgically treated by means of STP. The following variables were evaluated: demographics, preoperative tracheal stenosis characteristics, associated anomalies and outcome measures.
RESULTS
Cohort included 14 patients (8 males and 6 females) with a mean age of 8.7 months when treated (range, 1–43 m). Eleven patients (78%) showed a long segment CTS (>30% of total tracheal length) and 9 (64%) had associated cardiac or great vessel anomalies (left pulmonary artery sling). Three patients (21%) showed severe postoperative complications that required significant airway reintervention: tracheal resection of a restenotic segment, laser division with balloon dilatation of a residual stenosis and placement of a biodegradable endotracheal stent in an extensive tracheal narrowing. All patients are in good clinical condition with a mean follow-up of 6.3 years (range, 2 months to 16 years).
CONCLUSIONS
STP has become the procedure of choice when dealing with CTS. Although it shows clear advantages compared with other surgical techniques, severe and difficult to manage complications may occur. Surgeons involved in their treatment should be familiar with diverse surgical and endoscopical procedures. Biodegradable airway stenting is a new and promising technique when long and severe post-surgical tracheal stenosis is present.
We performed experimental and theoretical studies of deep inelastic multinucleon transfer reactions in heavy-ion collisions at Coulomb barrier energies. Our goal was to investigate if deep inelastic ...transfer is superior to fragmentation reactions for producing neutron-rich isotopes in the astrophysically interesting region along the closed neutron shell
N
= 126 . Here, we will present our results obtained in reactions of
64
Ni +
207
Pb at 5.0 MeV/nucleon. The experiment was performed at the velocity filter SHIP at GSI Darmstadt. Several transfer products on the neutron-rich side were populated but new isotopes were not observed. A comparison of the measured transfer cross-sections and production yields with those from fragmentation reactions allowed for interesting conclusions.
Objective: Tracheobronchial obstruction is infrequent in the pediatric age group but it is associated with significant morbidity and mortality. The purpose of this study is to review the results of a ...single institution experience with endoscopic stent placement in children with benign tracheobronchial obstruction, and with special concern on safety and clinical effectiveness. Materials and methods: Twenty-one patients with severe airway stenosing disease in which stent placement was performed between 1993 and 2006. Inclusion criteria according to the clinical status were: failure to wean from ventilation, episode of apnea, frequent respiratory infections (>3 pneumonia/year), and severe respiratory distress. Additional criteria for stent placement were: failure of surgical treatment, bronchomalacia, and tracheomalacia refractory to previous tracheostomy. Selection of the type of stent depended on the site of the lesion, the patient’s age, and the stent availability when time of presentation. The following variables were retrospectively evaluated: age, type of obstruction, associated malformations, stent properties, technical and clinical success, complications and related reinterventions, outcome and follow-up period. Results: Thirty-three stents were placed in the trachea (n = 18) and/or bronchi (n = 15) of 21 patients with a median age of 6 months (range, 9 days–19 years). Etiology of the airway obstruction included severe tracheomalacia and/or bronchomalacia in 19 cases (90%), and postoperative tracheal stenosis in two. Twelve children had a total of 20 balloon-expandable metallic stents placed, and 10 had 13 silicone-type stents (one patient had both). In nine patients (42%) more than one device was placed. Stent positioning was technically successful in all but one patient. Clinical improvement was observed in 18 patients (85%) but complications occurred in five of them (27%). Eight patients died during follow-up but only in one case it was related to airway stenting. Thirteen patients (62%) are alive and in good condition with a mean follow-up of 39 months (1–13.8 years). Conclusions: Although the results were based on a small series, placement of stents in the pediatric airway to treat tracheobronchial obstruction seems to be safe and effective. Stenting is a satisfactory therapeutic option when other procedures have failed or are not indicated.
.
Recently, we reported the observation of several new isotopes with proton numbers
in low-energy collisions of
48
Ca +
248
Cm . The peculiarity is that the nuclei were produced in multinucleon ...transfer reactions, a method which is presently discussed as a possible new way to enter so far unknown regions in the upper part of the Chart of Nuclides. For separation of the transfer products we used a velocity filter, the Separator for Heavy Ion Reaction Products SHIP at GSI. The resulting strong background suppression allowed us to detect nuclei with cross-sections down to the sub-nanobarn scale. Beside the new isotopes we identified about 100 further target-like transfer products and determined their cross-sections. The results together with previous measurements strongly indicate that multinucleon transfer reactions are a viable pathway to the production of new transuranium isotopes.