Clear Discrepancy in Neurofibromin Expression between NF1 Pheochromocytoma Cells and Non-Tumorous Adrenal Medullary Cells

Neurofibromatosis type 1 (NF1) is a common inherited disorder with an autosomal dominant trait. We encountered an NF1 patient who showed adrenal pheochromocytoma, and analyzed expression of neurofibromin in an excised specimen. A 54-year-old man showing multiple neurofibromas and café-au-lait spots in the skin was pointed out to have a right adrenal tumor by ultrasonography. Abdominal CT also revealed a right adrenal tumor. He was diagnosed with neurofibromatosis type 1 with no family history. Urine catecholamines, metanephrine, and normetanephrine levels were elevated. MIBG scintigraphy showed positive right adrenal uptake, and so pheochromocytoma was also diagnosed. The patient underwent laparoscopic right adrenalectomy. The excised adrenal specimen of this patient was stained with anti-neurofibromin polyclonal antibody. The NF1 pheochromocytoma was completely negative for neurofibromin protein expression, while the NF1 non-pheochromocytomatous adrenal medulla was neurofibromin-positive in the cytoplasm and nucleus. The clear discrepancy in neurofibromin expression between pheochromocytoma cells and “normal” adrenal medullary cells of the patient may well be explained by Knudson’s two-hit hypothesis.

Mechanical properties of 7475 aluminum alloy sheets with fine subgrain structure by warm rolling

The effect of transition elements on grain refinement of 7475 aluminum alloy sheets produced by warm rolling was investigated. The alloy which contains zirconium instead of chromium showed ultra fine structures with stable subgrains after warm rolling at 350 ℃, followed by solution heat treatment at 480 ℃. The average subgrain diameter was approximately 3 pan. It became clear that zirconium in solution has the effect of stabilizing subgrains due to precipitation of fine Al3Zr compounds during warm rolling. On the other hand, chromium-bearing compounds precipitate before warm rolling and they grow up to relatively large size during warm rolling. The warm rolled sheets with fine subgrains have unique properties compared with conventional 7475 aluminum alloy sheets produced by cold rolling. The warm roiled sheets solution heat treated had subgrain structures through the thickness with a high proportion of low-angle boundary less than 15°. The strength of the warm rolled sheets in T6 condition was about 10% higher than that of conventional 7475 aluminum alloy sheets. As the most remarkable point in the warm rolled sheets, the high Lankford （r） value of 3.5 was measured in the orientation of 45° to rolling direction, with the average r-value of 2.2. The high r-value would be derived from well developed r-fiber textures, especially with the strong {011 }（211） brass component. The warm rolled sheets also had high resistance to SCC. From Kikuchi lines analysis and TEM images, it was found that PFZs were hardly formed along the low- angle boundaries of the warm rolled sheets in T6 condition. This would be a factor to lead to the improvement of resistance to SCC because of reducing the difference in electrochemical property between the grain boundary area and the grain interior.

Effect of Tirapazamine and Mild Temperature Hyperthermia on the Recovery from Radiation-Induced Damage in Pimonidazole-Unlabeled Quiescent Tumor Cell Population

The aim in this study is to examine the effect of tirapazamine (TPZ) and mild temperature hyperthermia (MTH) on the repair of radiation-induced damage in pimonidazole-unlabeled quiescent (Q) tumor cells. Labeling of proliferating (P) cells in C57BL/6J mice bearing EL4 tumors was achieved by continuous administration of 5-bromo-2-deoxyuridine (BrdU). Tumors were irradiated with γ-rays at 1 h after the administration of pimonidazole followed by TPZ treatment or MTH. Twenty-four hours later, assessment of the responses of Q and total (= P + Q) cells were based on the frequencies of micronucleation and apoptosis using immunofluorescence staining for BrdU. The response of the pimonidazole-unlabeled tumor cell fractions was assessed by means of apoptosis frequency using immunofluorescence staining for pimonidazole. With γ-rays only, the pimonidazole-unlabeled cell fraction showed significantly enhanced radio-sensitivity compared with the whole cell fraction more remarkably in Q cells than total cells. However, a significantly greater decrease in radio-sensitivity in the pimonidazole-unlabeled than the whole cell fraction, evaluated using a delayed assay, was more clearly observed in Q cells than total cells. Post-irradiation MTH more remarkably repressed the decrease in radio-sensitivity in the Q cell than the total cells. Post-irradiation TPZ administration produced a large radio-sensitizing effect on both total and Q cells, especially on Q cells. On the other hand, in pimonidazole-unlabeled cell fractions in both total and Q cells, TPZ suppressed the reduction in sensitivity due to delayed assay much more efficiently than MTH, whereas no radio-sensitizing effect was produced. Not only through suppressing the recovery from radiation-induced damage but also through radio-sensitizing effect, post-irradiation TPZ administration is very useful for repressing the increase in the difference in radio-sensitivity due to the delayed assay not only between total and Q tumor cells but also between the pimonidazole-unlabeled and the whole cell fractions within the total and Q tumor cells.

An Isolated Case of Brachyphalangism of the Basal Finger Bones of the Little Finger with Symptoms of Tenosynovitis: A Case Report

Brachydactyly is a general term that refers to disproportionately short fingers and toes and forms a part of the group of limb malformations characterized by bone dysostosis. Brachydactyly usually occurs either as an isolated malformation or as a part of a complex malformation syndrome. Brachydactyly is classified as types A, B, C, D, or E;brachymetatarsus IV;Sugarman brachydactyly;or Kirner deformity. Various types of isolated brachydactyly are rare, except for types A3 and D. We describe a 15-year-old girl with isolated brachyphalangism of the basal finger bones of the little finger with symptoms of tenosynovitis. Tenosynovitis might be caused by growth deviation between the flexor digitorum superficialis and the flexor digitorum profundus. The patient responded very well to surgical treatment.

Finite-difference time-domain simulation of partial discharges in a gas insulated switchgear

It is important to estimate the current magnitude and charge amount of a partial discharge (PD) in a gasinsulatedswitchgear (GIS). In this study, electromagnetic fields generated by a PD in an 84 kV-class three-phase GIS fortesting have been measured with a voltage probe installed on the outer surface of an insulation spacer of the GIS tank,and the corresponding simulations have been carried out using the three-dimensional finite-difference time-domain(FDTD) method. FDTD-computed time-domain voltages across spacers agree reasonably well with the correspondingmeasured ones, and the current magnitude of PD and its charge amount have been estimated.