Search

INTRODUCTION: The osteogenesis imperfecta (OI) is a phenotypically heterogeneous hereditary connective tissue disease. The mild forms of OI tends to show minimum fragility on bones and normal height which could only be manifested as an early osteoporosis or serious postmenopausal bone density loss. The severe clinic picture shows multiple fractures with minimum trauma and bone deformity. The most severe form causes perinatal death. CASE REPORT: A breastfed infant boy of two years and seven months, born by natural birth, very small for gestational age, first born of a teenage mother and a father with OI. He was, first hospitalized for weight and growth control. No pathological findings were recognized. After the discharge, he did not attend to the genetic control. At one year old, he was sent with a right femur fracture after a minimum trauma. He was hospitalized suspecting OI and possible domestic violence. A first genetic evaluation did not match with OI, then endocrinology requested a complete radiological study. The biochemical study was normal. Then, he presented left femur and right tibia fracture. The genetic reevaluation, found multiple dysmorphic characteristic and late size development, which confirmed a mild form of OI. A biphosphonates treatment was initiated. DISCUSSION: The OI is a frequent disease with heterogeneous manifestations. The diagnosis is simple, bases on bone fragility, extra skeletal manifestations and familiar history. An early diagnosis and treatment could minimize complications and maximize mobility and functional capacity. [ABSTRACT FROM AUTHOR]

Magnetizing the fuel in inertial confinement fusion relaxes ignition requirements by reducing thermal conductivity and changing the physics of bum product confinement. Diagnosing the level of fuel magnetization during bum is critical to understanding target performance in magneto-inertial fusion (MIF) implosions. In pure deuterium fusion plasma, 1.01 MeV tritons are emitted during deuterium-deuterium fusion and can undergo secondary deuterium-tritium reactions before exiting the fuel. Increasing the fuel magnetization elongates the path lengths through the fuel of some of the tritons, enhancing their probability of reaction. Based on this feature, a method to diagnose fuel magnetization using the ratio of overall deuterium-tritium to deuterium-deuterium neutron yields is developed. Analysis of anisotropies in the secondary neutron energy spectra further constrain the measurement. Secondary reactions also are shown to provide an upper bound for the volumetric fuel-pusher mix in MIF. The analysis is applied to recent MIF experiments [M. R. Gomez et al., Phys. Rev. Lett. 113, 155003 (2014)] on the Z Pulsed Power Facility, indicating that significant magnetic confinement of charged bum products was achieved and suggesting a relatively low-mix environment. Both of these are essential features of future ignition-scale MIF designs. [ABSTRACT FROM AUTHOR]

This Letter presents results from the first fully integrated experiments testing the magnetized liner inertial fusion concept [S. A. Slutz et at, Phys. Plasmas 17, 056303 (2010)], in which a cylinder of deuterium gas with a preimposed 10 T axial magnetic field is heated by Z beamlet, a 2.5 kJ, 1 TW laser, and magnetically imploded by a 19 MA, 100 ns rise time current on the Z facility. Despite a predicted peak implosion velocity of only 70 km/s, the fuel reaches a stagnation temperature of approximately 3 keV, with Te ≈ Ti, and produces up to 2 × 1012 thermonuclear deuterium-deuterium neutrons. X-ray emission indicates a hot fuel region with full width at half maximum ranging from 60 to 120 μn over a 6 mm height and lasting approximately 2 ns. Greater than 1010 secondary deuterium-tritium neutrons were observed, indicating significant fuel magnetization given that the estimated radial areal density of the plasma is only 2 mg/cm². [ABSTRACT FROM AUTHOR]