Phosphaturic Mesenchymal Tumor.

Phosphaturic mesenchymal tumors (PMTs) are neoplasms associated with tumor-induced osteomalacia. Patients typically present with pathologic fractures in the setting of chronic hypophosphatemic hyperphosphaturic osteomalacia, as well as gradual muscle weakness, bone pain, and difficulty walking. Because of their rarity and nonspecific symptomatology, phosphaturic mesenchymal tumors often go undiagnosed for years. Even when discovered on imaging, the tumors can be diagnostically challenging for radiologists. Phosphaturic mesenchymal tumors often tend to be small and can be located nearly anywhere in the body, and, therefore, can mimic many other tumors. This case highlights the imaging and pathologic markers of a phosphaturic mesenchymal tumor, often found in a patient with tumor-induced osteomalacia.

Therapists' perspectives on a new portable hand telerehabilitation platform for home-based personalized treatment of stroke patients.

OBJECTIVE: Patients who have sustained a stroke suffer from residual motor impairments. Stroke can limit their ability to employ their impaired upper limb properly. Hand function is particularly one of the most frequently persisting consequences of stroke. This paper introduces a new portable hand telerehabilitation platform (PHTP) for home-based personalized treatment of stroke patients. The aims of this study are (1) to document the iterative design and development process of the PHTP, and (2) to explore the therapists' perspectives on implementing home-based treatment of stroke patients. MATERIALS AND METHODS: Local therapists were involved early in designing and developing the PHTP. We collected views of 84 therapists practicing in North America via an online survey. RESULTS: Therapists' perspectives on the introduced prototype support the use of the PHTP to provide home-based telerehabilitation for stroke patients. The System Usability Scale score was 70 for the PHTP, indicating that the platform is usable. The rest of the qualitative results obtained from custom questionnaires showed consistency in the platform design, high perceived usability and good acceptability among the therapists' community. CONCLUSIONS: In sum, the results encourage and support fine-tuning of the PHTP, commercializing it, and conducting prospective clinical studies.

Thermionic microwave gun for terahertz and synchrotron light sources.

Conventional thermionic microwave and radio frequency (RF) guns can offer high average beam current, which is important for synchrotron light and terahertz (THz) radiation source facilities, as well as for industrial applications. For example, the Advanced Photon Source at Argonne National Laboratory is a national synchrotron-radiation light source research facility that utilizes thermionic RF guns. However, these existing thermionic guns are bulky, difficult to handle and install, easily detuned, very sensitive to thermal expansion, and due for a major upgrade and replacement. In this paper, we present the design of a new, more stable, and reliable gun with optimized electromagnetic performance, improved thermal engineering, and a more robust cathode mounting technique, which is a critical step to improve the performance of existing and future light sources, industrial accelerators, and electron beam-driven THz sources. We will also present a fabricated gun prototype and show results of high-power and beam tests.

The sex differences of the behavior response to early Life immune stimulation: Microglia and astrocytes involvement.

It is well known that inflammatory challenge during the prenatal period results in permanent changes in glial cells and behavior in adulthood. However, it is unknown whether inflammatory challenge during the infantile period may have permanent sexually-dimorphic effects on microglia and astrocytes in vivo, which in turn may be associated with sex differences in adult behavior. In this study, we have evaluated whether postnatal injection of lipopolysaccharide (LPS; 250µg/kg, i.p. on postnatal day 14) induces depressive and less anxiety-like behaviors, glial cell activation, pro-inflammatory cytokine (TNF-alpha) secretion and sexually dimorphic responses in adulthood. Postnatal day 14 (P14) male and female Wistar rats received an intraperitoneal (ip) injection of LPS or PBS. Three months later, animals were tested in the Open Field (OF), the Elevated Plus Maze (EPM) and the Forced Swimming Test (FST) to assess the level of anxiety and depression-like behavior. Hippocampal proinflammatory cytokine TNF-alpha concentration and the number of astrocytes and microglia were estimated in the dentate gyrus, CA1, and CA3 in two regions of the hippocampus (ventral and dorsal). Our results showed that the administration of LPS resulted in less anxiety and depression-like behavior in males but not in females. However, the LPS-administration increased the number of microglia in the dorsal and ventral hippocampus areas in females more than male, while no significant differences in TNFα level had been detected between the LPS-rats treated and their controls. Interestingly, LPS resulted in an increase in the number of astrocytes in both areas of the hippocampus in a female. While in a male, our results showed a decrease in astrocytes number in the dorsal hippocampus, but no significant differences observed in ventral hippocampus. These findings indicate that an immune challenge in infantile rats induces a ventral and dorsal hippocampus damage in female more than in male, without affecting significantly the affective behavior changes in the female. The results also showed that small changes in the male hippocampus can affect the behavior and induce a depression-like behavior.

Nfkb1 is a haploinsufficient DNA damage-specific tumor suppressor.

NF-κB proteins play a central and subunit-specific role in the response to DNA damage. Previous work identified p50/NF-κB1 as being necessary for cytotoxicity in response to DNA alkylation damage. Given the importance of damage-induced cell death for the maintenance of genomic stability, we examined whether Nfkb1 acts as a tumor suppressor in the setting of alkylation damage. Hprt mutation analysis demonstrates that Nfkb1(-/-) cells accumulate more alkylator-induced, but not ionizing radiation (IR)-induced, mutations than similarly treated wild-type cells. Subsequent in vivo tumor induction studies reveal that following alkylator treatment, but not IR, Nfkb1(-/-) mice develop more lymphomas than similarly treated Nfkb1(+/+) animals. Heterozygous mice develop lymphomas at an intermediate rate and retain functional p50 in their tumors, indicating that Nfkb1 acts in a haploinsufficient manner. Analysis of human cancers, including therapy-related myeloid neoplasms, demonstrates that NFKB1 mRNA expression is downregulated compared with control samples in multiple hematological malignancies. These data indicate that Nfkb1 is a haploinsufficient, pathway-specific tumor suppressor that prevents the development of hematologic malignancy in the setting of alkylation damage.

Impact of chronic renal failure on anti-tuberculosis treatment outcomes.

SETTING: National Referral Centre for Tuberculosis (TB), Tehran, Iran. OBJECTIVE: To determine the impact of chronic renal failure (CRF) on TB treatment outcomes. DESIGN: A retrospective study was conducted among adult TB patients with CRF and age- and sex-matched TB controls without CRF treated at the National Research Institute of Tuberculosis and Lung Disease from 2004 to 2011. Multivariate analysis was performed to determine the impact of CRF on drug-induced hepatitis (DIH), treatment failure and all-cause mortality. RESULTS: A total of 55 TB cases with CRF and 165 TB cases without CRF were included in the study. Baseline demographic and clinical characteristics were similar, except that TB cases with CRF were more likely to be of Iranian nationality (94.5% vs. 83%, P = 0.04). During anti-tuberculosis treatment, 40 (18.2%) patients developed DIH, none failed treatment and 15 (6.8%) died. Patients with CRF were more likely to develop DIH (27.3% vs. 15.2%, P = 0.04) and to die during treatment (16.4% vs. 3.6%, P = 0.001). CRF remained significantly associated with all-cause mortality (HR 4.87, 95%CI 1.73-13.65) in multivariate analysis, whereas the relationship with DIH was not. CONCLUSION: TB patients with CRF are at increased risk of death. More intensive monitoring of patients with CRF should be considered by the National TB Programme.

A multicenter retrospective study investigating the role of interventional bronchoscopic techniques in the management of endobronchial lipomas.

BACKGROUND: Pulmonary lipomas are rare benign tumors that are usually found endobronchially. Undiagnosed, they can lead to the serious late sequelae associated with endobronchial obstruction. In the majority of cases, they are located in the first three subdivisions of the tracheobronchial tree, and therefore, they are amenable to endoscopic techniques for diagnostic and therapeutic purposes. OBJECTIVES: It was our aim to retrospectively study the bronchoscopic management and follow-up of a large series of endobronchial lipomas, as well as defining the demographic and endoscopic characteristics of patients. METHODS: A retrospective multicenter study was performed to identify all cases of lipomas that were treated endoscopically in 4 institutions in the period from 1981 to 2002. Demographic, radiological, endoscopic and histological data were collected. RESULTS: Thirty-eight patients were included in the study; 81.6% of cases were males and the average age was 63.5 +/- 15.2 years. The majority of the patients were symptomatic (63.2%). Lipomas were located proximally in 18 cases (47.4%) and distally in 20 subjects (52.6%). Specimens obtained by rigid bronchoscopy were diagnostic in all cases. Thirty-six out of 38 patients underwent therapeutic rigid bronchoscopy. Laser and mechanical debulking was performed in 29 cases (76.3%), cryotherapy and mechanical debulking in 7 subjects (18.4%), and mechanical debulking alone in 2 cases (5.3%). No cases of recurrence occurred during the follow-up period. CONCLUSIONS: This study demonstrates that endoscopic techniques are effective for the diagnosis and treatment of endobronchial lipomas when there is no evidence of severe distal bronchiectasis. This should be the treatment of choice after a full clinicoradiological evaluation.

Socioeconomic status of Iranian living unrelated kidney donors: a multicenter study.

Renal transplantation is the best treatment modality for end-stage renal disease (ESRD) patients. In Iran, a living unrelated donor (LURD) transplant program was started in 1988, because of the shortage of living related and cadaver donors. In this study, we evaluated the socioeconomic status of LURD in Iran. We enrolled 478 LURDs from 30 transplant centers between October 2005 and March 2006. The demographic data, education, employment, motivation, and satisfaction status were asked. Economic status was assessed using criteria of the statistics center of Iran. Ninety-six percent of donors were referred from the Kidney Foundation of Iran (KFI). The mean age of the donors was 27 +/- 4.8 years and 85% were men (n=408). Fifty-one percent were smokers, 82% married, and 79% the breadwinner; 29% were unemployed; 2.7%, 90.8%, and 6.5% were illiterate, school graduates, and university graduates, respectively. Coercion was documented for only one donor. Financial issues were the most frequent motive. Among them, 91% were satisfied with the donation. Finally, 53% suggested kidney donation to others. Of donors, 62% were living below the poverty line. In the Iran model, the KFI is a bridge that connects recipients and donors. As a result, there is no middle man or broker introducing donors to recipients. The transplantation team knows nothing about money transactions between recipient and donor. Most donors were satisfied with the donation. However, establishment of a government-regulated program for social support of donors, such as lifelong health insurance may be a compensation for donors.

Exponential gain and saturation of a self-amplified spontaneous emission free-electron laser.

Self-amplified spontaneous emission in a free-electron laser has been proposed for the generation of very high brightness coherent x-rays. This process involves passing a high-energy, high-charge, short-pulse, low-energy-spread, and low-emittance electron beam through the periodic magnetic field of a long series of high-quality undulator magnets. The radiation produced grows exponentially in intensity until it reaches a saturation point. We report on the demonstration of self-amplified spontaneous emission gain, exponential growth, and saturation at visible (530 nanometers) and ultraviolet (385 nanometers) wavelengths. Good agreement between theory and simulation indicates that scaling to much shorter wavelengths may be possible. These results confirm the physics behind the self-amplified spontaneous emission process and forward the development of an operational x-ray free-electron laser.

[Hypoxemia secondary to inferior vena cava return into left atrium]. / Hypoxémie secondaire à un abouchement de la veine cave inférieure dans l'oreillette gauche.

The case of a right-to-left shunt-induced hypoxemia with an abnormal return of the inferior vena cava (AIVCR) into the left atrium (LA) is reported in a 30-year-old male with cyanosis and polycythemia. The chest X ray and the lung CT scan was normal. Spirometry was normal but the transfert-CO coefficient (KCO) was lowered. Hypoxemia was observed at rest and worsening during exercise. The alveolo-arterial oxygen tension difference under hyperoxia was increased (56 kPa). Contrast echocardiography (CEch) suggested the presence of an AIVCR with a right-to-left shunt only observed by the inferior route. The inferior vena cava (IVC) angiography and the magnetic resonance imaging demonstrated an AIVCR characterized by a direct drainage of IVC in the left atrium. The good tolerance can be explained by the association of AIVCR with an inter-auricular septal defect resulting in a left-to-right shunt which partially corrected the right-to-left shunt. After surgical treatment, arterial blood gases normalized, KCO remained low and CEch became negative.