Probable Noonan syndrome in a boy without PTPN11 mutation, manifesting unusual complications.

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A subset of ocular adnexal marginal zone lymphomas may arise in association with IgG4-related disease

We previously suggested a relationship between ocular immunoglobulin (Ig)G4-related disease (IgG4-RD) and marginal zone lymphomas (MZLs). However, the cytokine background associated with these disorders and whether it differs between ocular adnexal MZLs with (IgG4-associated MZL) and without (IgG4-negative MZL) numerous IgG4+ plasma cells are unknown. In this study, we identified the mRNA expression pattern of Th2 and regulatory T-cell (Treg) cytokines in IgG4-RD and in IgG4-associated MZL and IgG4-negative MZL using real-time polymerase chain reaction analysis. Ocular IgG4-RD and IgG4-associated MZL exhibited significantly higher expression ratios of interleukin (IL)-4/β-actin, IL-10/β-actin, IL-13/β-actin, transforming growth factor (TGF) β1/β-actin, and FOXP3/β-actin than did IgG4-negative MZL (p < 0.05). This finding further supports our prior observations that a significant subset of ocular MZLs arises in the setting of IgG4-RD. Furthermore, the presence of a different inflammatory background in IgG4-negative MZLs suggests that IgG4-associated MZLs may have a different pathogenesis

A Retrospective Study in the Diagnosis of 301 Jaundiced Cases

In the past 12 years, 301 patients with a total serum bilirubin over 2 mg/dl (reference interval 0.2-0.8 mg/dl) were admitted to the Second Department of Surgery, Nagasaki University School of Medicine, Japan. The purpose of this paper is to analyse the jaundiced cases and to evaluate the diagnostic accuracy of the following imaging techniques: Computed Tomography (CT), Ultrasonography (US), Drip Infusion Cholangiography (DIC), Endoscopic Retrograde Cholangio- pancreatography (ERCP), Percutaneous transhepatic Cholangiography (PTC) and Selective Celiac Angiography (SCAG). Of the 301 patients, 63 had carcinoma of the bile duct, 48 carcinoma of the pancreas, 26 carcinoma of the gallbladder, 16 hepatoma, 8 carcinoma of the ampulla of Vater, 83 cholelithiasis, 27 parenchymal liver disease, 9 congenital bile duct disease, 5 chronic pancreatitis, 14 other diseases, and 2 had no final diagnosis. CT was attempted in 33 of 170 patients with malignancy, and 22 of 129 patients with benignancy. A correct finding was obtained in 23 (69.7%) of the 33 patients and 18 (81.8%) of the 22 patients, respectively. US gave a correct finding in 28 (59.6 %) of 47 patients with malignancy, and 36 (69.2%) of 52 patients with benignancy. DIC gave a correct finding in 1 (5.6%) of 18 patients with malignancy, and 16 (42.1%) of 38 patients with benignancy. ERCP gave a correct finding in 33 (76.7%) of 43 patients with malignancy, and 38 (74.5%) of 51 patients with benignancy. PTC gave a correct finding i

Reconstruction of a pelvic floor defect using a pedicled tensor fascia lata flap: a new technique to prevent radiation injury for pediatric patients with advanced pelvic tumors

BACKGROUND: In the treatment of pelvic tumors, pelvic floor defects owing to a wide excision tend to increase the occurrence of such morbidities as radiation injury. The reconstruction of these defects would minimize the risk of such morbidities. Authors introduce a new technique for repairing a pelvic floor defect using a tensor fascia lata flap. METHODS: Two boys, 4 years old and 10 months old, presenting with pelvic rhabdomyosarcoma underwent a tumor extirpation associated with a wide excision of the pelvic organs. After the removal of the tumor, a tensor fascia lata flap was designed on the right thigh. The pedicled rotation flap was subcutaneously elevated, guided to the intraperitoneal cavity, and was fixed to cover the superior aperture of the lesser pelvis. RESULTS: The flaps functioned well, and postoperative radiation therapies consisting of 45 and 41.4 Gy to the lesser pelvic cavity were carried out without any complications. As a result, the necessary postoperative protocol combination therapies could be successfully performed in a timely manner. CONCLUSION: The pedicled tensor fascia lata flap is considered to be an alternative option for the stable repair of pelvic floor defects to prevent radiation injury.Without Figure

The ASTRO-H X-ray Observatory

The joint JAXA/NASA ASTRO-H mission is the sixth in a series of highly successful X-ray missions initiated by the Institute of Space and Astronautical Science (ISAS). ASTRO-H will investigate the physics of the high-energy universe via a suite of four instruments, covering a very wide energy range, from 0.3 keV to 600 keV. These instruments include a high-resolution, high-throughput spectrometer sensitive over 0.3-2 keV with high spectral resolution of Delta E < 7 eV, enabled by a micro-calorimeter array located in the focal plane of thin-foil X-ray optics; hard X-ray imaging spectrometers covering 5-80 keV, located in the focal plane of multilayer-coated, focusing hard X-ray mirrors; a wide-field imaging spectrometer sensitive over 0.4-12 keV, with an X-ray CCD camera in the focal plane of a soft X-ray telescope; and a non-focusing Compton-camera type soft gamma-ray detector, sensitive in the 40-600 keV band. The simultaneous broad bandpass, coupled with high spectral resolution, will enable the pursuit of a wide variety of important science themes.Comment: 22 pages, 17 figures, Proceedings of the SPIE Astronomical Instrumentation "Space Telescopes and Instrumentation 2012: Ultraviolet to Gamma Ray

The Quiescent Intracluster Medium in the Core of the Perseus Cluster

Clusters of galaxies are the most massive gravitationally-bound objects in the Universe and are still forming. They are thus important probes of cosmological parameters and a host of astrophysical processes. Knowledge of the dynamics of the pervasive hot gas, which dominates in mass over stars in a cluster, is a crucial missing ingredient. It can enable new insights into mechanical energy injection by the central supermassive black hole and the use of hydrostatic equilibrium for the determination of cluster masses. X-rays from the core of the Perseus cluster are emitted by the 50 million K diffuse hot plasma filling its gravitational potential well. The Active Galactic Nucleus of the central galaxy NGC1275 is pumping jetted energy into the surrounding intracluster medium, creating buoyant bubbles filled with relativistic plasma. These likely induce motions in the intracluster medium and heat the inner gas preventing runaway radiative cooling; a process known as Active Galactic Nucleus Feedback. Here we report on Hitomi X-ray observations of the Perseus cluster core, which reveal a remarkably quiescent atmosphere where the gas has a line-of-sight velocity dispersion of 164+/-10 km/s in a region 30-60 kpc from the central nucleus. A gradient in the line-of-sight velocity of 150+/-70 km/s is found across the 60 kpc image of the cluster core. Turbulent pressure support in the gas is 4% or less of the thermodynamic pressure, with large scale shear at most doubling that estimate. We infer that total cluster masses determined from hydrostatic equilibrium in the central regions need little correction for turbulent pressure.Comment: 31 pages, 11 Figs, published in Nature July

Hitomi (ASTRO-H) X-ray Astronomy Satellite

The Hitomi (ASTRO-H) mission is the sixth Japanese x-ray astronomy satellite developed by a large international collaboration, including Japan, USA, Canada, and Europe. The mission aimed to provide the highest energy resolution ever achieved at E  >  2  keV, using a microcalorimeter instrument, and to cover a wide energy range spanning four decades in energy from soft x-rays to gamma rays. After a successful launch on February 17, 2016, the spacecraft lost its function on March 26, 2016, but the commissioning phase for about a month provided valuable information on the onboard instruments and the spacecraft system, including astrophysical results obtained from first light observations. The paper describes the Hitomi (ASTRO-H) mission, its capabilities, the initial operation, and the instruments/spacecraft performances confirmed during the commissioning operations for about a month