A Child with Resistance to Thyroid Hormone without Thyroid Hormone Receptor Gene Mutation: A 20-Year Follow-Up

We report here the 20-year follow-up study of a male subject diagnosed at 15 months of age as a sporadic case of pituitary resistance to thyroid hormone on the combination of clinical hyperthyroidism, elevated serum thyroid hormone (TH) levels and inappropriate thyrotropin (TSH). On d-thyroxine (D-T4) therapy from 30 months of age to 12.5 years, hyperactivity and hyperthyroid signs and symptoms as well as growth abnormalities improved, serum l-thyroxine (L-T4) enantiomer normalized, and basal and stimulated TSH decreased significantly without complete suppression. After 8 years off D-T4, at 20 years of age, clinical status was normal despite persisting high TH levels and inappropriate TSH. Evolution of serum markers of TH action and echocardiography measurements followed up from 15 months to 20 years of age either in basal condition or on triiodothyronine (T3), as well as the sequential determination of bone mineral density suggest differences in the tissue responses to T3: normal in bone with a high remodelling rate, heterogeneity for various hepatic markers, and decreased at heart level. No mutations were found in the coding sequence of TRβ1, TRβ2, TRα1, RXRγ, SMRT, NCoR1, and NCoA1. In this patient the putative long-term effects of the persisting high bone resorption are unknown

Path Tracking Control for Autonomous Driving Applications

Autonomous or self-driving vehicles are becoming a consolidate reality that involves both industrial and academic elds also for its impact in social and governmental communities, well far from automotive engineering. The intent of the present paper is to design an automatic steering control for an autonomous vehicle equipped with steer-by-wire and drive-by-wire technologies. The steering action is calculated to let the vehicle follow a reference path which is stored in a Digital Map properly built to be available in real-time. A Proportional + Derivative (PD) control strategy is deigned based on the Parameter State Approach (PSA) and it is coupled with a Feedforward (FF) term for improving the path tracking control in cornering maneuvers. Some experimental results are shown to demonstrates the ecacy of the controller presented

First direct mass-measurement of the two-neutron halo nucleus 6He and improved mass for the four-neutron halo 8He

The first direct mass-measurement of $^{6}$He has been performed with the TITAN Penning trap mass spectrometer at the ISAC facility. In addition, the mass of $^{8}$He was determined with improved precision over our previous measurement. The obtained masses are $m$($^{6}$He) = 6.018 885 883(57) u and $m$($^{8}$He) = 8.033 934 44(11) u. The $^{6}$He value shows a deviation from the literature of 4$\sigma$. With these new mass values and the previously measured atomic isotope shifts we obtain charge radii of 2.060(8) fm and 1.959(16) fm for $^{6}$He and $^{8}$He respectively. We present a detailed comparison to nuclear theory for $^6$He, including new hyperspherical harmonics results. A correlation plot of the point-proton radius with the two-neutron separation energy demonstrates clearly the importance of three-nucleon forces.Comment: 4 pages, 2 figure

First Penning-trap mass measurement in the millisecond half-life range: the exotic halo nucleus 11Li

In this letter, we report a new mass for $^{11}$Li using the trapping experiment TITAN at TRIUMF's ISAC facility. This is by far the shortest-lived nuclide, $t_{1/2} = 8.8 \rm{ms}$, for which a mass measurement has ever been performed with a Penning trap. Combined with our mass measurements of $^{8,9}$Li we derive a new two-neutron separation energy of 369.15(65) keV: a factor of seven more precise than the best previous value. This new value is a critical ingredient for the determination of the halo charge radius from isotope-shift measurements. We also report results from state-of-the-art atomic-physics calculations using the new mass and extract a new charge radius for $^{11}$Li. This result is a remarkable confluence of nuclear and atomic physics.Comment: Formatted for submission to PR

Phonon anomalies at the valence transition of SmS : An inelasticX-ray scattering study under pressure

The phonon dispersion curve of SmS under pressure was studied by inelastic x-ray scattering around the pressure-induced valence transition. A significant softening of the longitudinal acoustic modes propagating along the [111] direction was observed spanning a wide $q$ region from ($\frac{2\pi}{3a},\frac{2\pi}{3a},\frac{2\pi}{3a}$) up to the zone boundary as SmS becomes metallic. The largest softening occurs at the zone boundary and stays stable up to the highest measured pressure of 80 kbar while a gradual hardening of the low $q$ modes simultaneously appears. This phonon spectrum indicates favorable conditions for the emergence of pressure-induced superconductivity in SmS.Comment: 4 pages, 3 figure

Coarctation of the aorta: pre and postoperative evaluation with MRI and MR angiography; correlation with echocardiography and surgery

Aims: To compare MRI and MRA with Doppler-echocardiography (DE) in native and postoperative aortic coarctation, define the best MR protocol for its evaluation, compare MR with surgical findings in native coarctation. Materials and methods: 136 MR studies were performed in 121 patients divided in two groups: Group I, 55 preoperative; group II, 81 postoperative. In group I, all had DE and surgery was performed in 35 cases. In group II, DE was available for comparison in 71 cases. MR study comprised: spin-echo, cine, velocity-encoded cine (VEC) sequences and 3D contrast-enhanced MRA. Results: In group I, diagnosis of coarctation was made by DE in 33 cases and suspicion of coarctation and/or aortic arch hypoplasia in 18 cases. Aortic arch was not well demonstrated in 3 cases and DE missed one case. There was a close correlation between VEC MRI and Doppler gradient estimates across the coarctation, between MRI aortic arch diameters and surgery but a poor correlation in isthmic measurements. In group II, DE detected a normal isthmic region in 31 out of 35 cases. Postoperative anomalies (recoarctation, aortic arch hypoplasia, kinking, pseudoaneurysm) were not demonstrated with DE in 50% of cases. Conclusions: MRI is superior to DE for pre and post-treatment evaluation of aortic coarctation. An optimal MR protocol is proposed. Internal measurement of the narrowing does not correspond to the external aspect of the surgical narrowin

Discontinuous Transition from a Real Bound State to Virtual Bound State in a Mixed-Valence State of SmS

Golden SmS is a paramagnetic, mixed-valence system with a pseudogap. With increasing pressure across a critical pressure Pc, the system undergoes a discontinuous transition into a metallic, anti-ferromagnetically ordered state. By using a combination of thermodynamic, transport, and magnetic measurements, we show that the pseudogap results from the formation of a local bound state with spin singlet. We further argue that the transition Pc is regarded as a transition from an insulating electron-hole gas to a Kondo metal, i.e., from a spatially bound state to a Kondo virtually bound state between 4f and conduction electrons.Comment: 5 pages, 5 figure

GPM-DPR Observations on TGFs Producing Storms

Unique spaceborne measurements of the three-dimensional structure of convective clouds producing terrestrial gamma ray flashes (TGFs) were performed using both active and passive microwave sensors on board the Global Precipitation Measurement (GPM)-Core Observatory satellite, finding coherent features for nine TGF-producing storms. The delineation of cloud structure using the radar reflectivity factor shows convective cells with significant vertical development and thick layers with high ice content. Compared to other cumulonimbus clouds in the tropics, the TGFs counterparts have higher reflectivity values above 3 and 8 km altitude showing in all cases a cumulonimbus tower and the TGFs locations are very close, or coincident, to these high Z columns, where reflectivity exceeds 50dBz. Using the GPM Microwave Imager radiometer, most thunderstorms show a very strong depression of polarization corrected temperature (PCT) at channel 89GHz, indicating a strong scattering signal by ice in the upper cloud layers. At channel 166GHZ, the difference between vertical and horizontal brightness temperature signal always returns positive values, from 0.2 up to 13.7K indicating a complex structure with randomly/vertically oriented ice particles. The PCT was used to characterize the analyzed storms in terms of hydrometeor types, confirming in 7/9 cases a high likelihood of hail/graupel presence. To perform analysis on the TGFs parent flashes, radio atmospherics data from the Earth Networks Total Lightning Network lightning network were used. Waveform data indicate that all cases are intra-cloud events and TGFs typically take place during the peak of flash rate production. Finally, the analysis of the most intense event is shown

Lin28A and Lin28B Inhibit let-7 MicroRNA Biogenesis by Distinct Mechanisms

SummaryLin28A and Lin28B selectively block the expression of let-7 microRNAs and function as oncogenes in a variety of human cancers. Lin28A recruits a TUTase (Zcchc11/TUT4) to let-7 precursors to block processing by Dicer in the cell cytoplasm. Here we find that unlike Lin28A, Lin28B represses let-7 processing through a Zcchc11-independent mechanism. Lin28B functions in the nucleus by sequestering primary let-7 transcripts and inhibiting their processing by the Microprocessor. The inhibitory effects of Zcchc11 depletion on the tumorigenic capacity and metastatic potential of human cancer cells and xenografts are restricted to Lin28A-expressing tumors. Furthermore, the majority of human colon and breast tumors analyzed exclusively express either Lin28A or Lin28B. Lin28A is expressed in HER2-overexpressing breast tumors, whereas Lin28B expression characterizes triple-negative breast tumors. Overall our results illuminate the distinct mechanisms by which Lin28A and Lin28B function and have implications for the development of new strategies for cancer therapy