A rare neurocutaneous syndrome and a rare association

Among the many causes of ataxia, Ataxia -telangictasia is an autosomal recessive1, multi system disease affecting the skin, nervous system & immune system. It's prevalence has been estimated at 1 to 2 per 100,000 It is a neurodegenerative disorder2 in which there is progressive cerebellar ataxia, occulocutaneous telangictasias, chronic sinopulmonary disease & high incidence of malignancy associated with variable humoral & cellular immuno deficiency. The abnormal gene has been mapped to the long arm of chromosome 11 (ATM gene)3 . Carriers has increased sensitivity to ionizing radiation4. Telangicatsias tends to develop between the ages of 3-6 years. It also affects the ears, eye lids immunologic abnormality is selective absence of IgA found in 50-80 % ,IgG may also be affected. The T cells percentage is reduced in total & T Helper (CD4) phenotype, with normal or increased percentages of the Suppressor ( CD8). Hodgkin's, non Hodgkin's lymphoma5 & leukemia6  develops in 10 % of cases6 . Other variable body tumors may occur. These patients may become critically ill and need intense care to prevent serious complications or death7. The syndrome is associated with abnormal IgA levels with a higher risk of blood malignancies like lymphoma or leukaemia, patients may have telangiectasia else were in the body specially the upper respiratory tract. The is a case report about one of this rare neuro- cutaneous syndromes which was reported in Khartoum Sudan. Consent from the parents was taken for the purpose of publication and teaching.Keywords: Ataxia telangiectasia, Sudan, Rare associatio

Activation Energy of Surface Diffusion and Terrace Width Dynamics During the Growth of in (4×3) on Si (100) - (2×1) by Femtosecond Pulsed Laser Deposition

The nucleation and growth of indium on a vicinal Si (100) - (2×1) surface at high temperature by femtosecond pulsed laser deposition was investigated by in situ reflection high energy electron diffraction (RHEED). RHEED intensity relaxation was observed for the first ∼2 ML during the growth of In (4×3) by step flow. From the temperature dependence of the rate of relaxation, an activation energy of 1.4±0.2 eV of surface diffusion was determined. The results indicate that indium small clusters diffused to terrace step edges with a diffusion frequency constant of (1.0±0.1) × 1011 s-1. The RHEED specular beam split peak spacing, which is characteristic of a vicinal surface, was analyzed with the growth temperature to obtain the average terrace width. Gradual reduction in the terrace width during growth of In (4×3) was observed with In coverage and is attributed to the detachment of In atoms from terrace edges. At a substrate temperature of 405 °C, the average terrace width decreased from 61±10 Å, which corresponds to the vicinal Si(100) surface, to an equilibrium value of 45±7 Å after deposition of ∼23 ML. Further In coverage showed a transition of the RHEED pattern from (4×3) to (1×1) and the growth of rounded In islands (average height of ∼1 nm and width of ∼25 nm), as examined by ex situ atomic force microscopy. © 2008 American Institute of Physics. [DOI: 10.1063/1.2909923

Atomic Hydrogen Cleaning of InP(100): Electron Yield and Surface Morphology of Negative Electron Affinity Activated Surfaces

Atomic hydrogen cleaning of the InP(100) surface has been investigated using quantitative reflection high-energy electron diffraction. The quantum efficiency of the surface when activated to negative electron affinity was correlated with surface morphology. The electron diffraction patterns showed that hydrogen cleaning is effective in removing surface contaminants, leaving a clean, ordered, and (2×4)-reconstructed surface. After activation to negative electron affinity, a quantum efficiency of ∼6% was produced in response to photoactivation at 632 nm. Secondary electron emission from the hydrogen-cleaned InP(100)-(2×4) surface was measured and correlated to the quantum efficiency. The morphology of the vicinal InP(100) surface was investigated using electron diffraction. The average terrace width and adatom-vacancy density were measured from the (00) specular beam at the out-of-phase condition. With hydrogen cleaning time, there was some reduction in the average terrace width. The surface quality was improved with hydrogen cleaning, as indicated by the increased (00) spot intensity-to-background ratio at the out-of-phase condition, and improved quantum efficiency after activation to negative electron affinity. © 2002 American Institute of Physics. [DOI: 10.1063/1.1429796

Quantum Trajectory Analysis of the Two-Mode Three-Level Atom Microlaser

We consider a single atom laser (microlaser) operating on three-level atoms interacting with a two-mode cavity. The quantum statistical properties of the cavity field at steady state are investigated by the quantum trajectory method which is a Monte Carlo simulation applied to open quantum systems. It is found that a steady state solution exists even when the detailed balance condition is not guaranteed. The differences between a single mode microlaser and a two-mode microlaser are highlighted. The second-order correlation function g^2(T) of a single mode is studied and special attention is paid to the one-photon trapping state, for which a simple formula is derived for its correlation function. We show the effects of the velocity spread of the atoms used to pump the microlaser cavity on the second-order correlation function, trapping states, and phase transitions of the cavity field

Atomic Hydrogen Cleaning of InP(100) for Preparation of a Negative Electron Affinity Photocathode

Atomic hydrogen cleaning is used to clean InP(100) negative electron affinity photocathodes. Reflection high-energy electron diffraction patterns of reconstructed, phosphorus-stabilized, InP(100) surfaces are obtained after cleaning at ∼400 °C. These surfaces produce high quantum efficiency photocathodes (∼8.5%), in response to 632.8 nm light. Without atomic hydrogen cleaning, activation of InP to negative electron affinity requires heating to ∼530 °C. At this high temperature, phosphorus evaporates preferentially and a rough surface is obtained. These surfaces produce low quantum efficiency photocathodes (∼0.1%). The use of reflection high-energy electron diffraction to measure the thickness of the deposited cesium layer during activation by correlating diffraction intensity with photoemission is demonstrated. © 1998 American Institute of Physics

Coherent Phonons in Bismuth Film Observed by Ultrafast Electron Diffraction

The generation of coherent phonons in polycrystalline bismuth film excited with femtosecond laser pulse is observed by ultrafast time-resolved electron diffraction. The dynamics of the diffracted intensities from the (110), (202), and (024) lattice planes show pronounced oscillations at 130-150 GHz. The origin of these coherent acoustic phonons is discussed in view of optical phonon decay into two acoustic phonons. Different drop times in the intensity of the diffraction orders are observed and interpreted as anisotropy in the energy transfer rate of coherent optical phonons

Scientific investigations in the Gulf of Mexico and Caribbean Sea during the 1974-1975 Calypso cruise, parts 1 and 2

The distribution and concentrations of the standing crop of phytoplankton and nutrient salts in the Gulf of Mexico and the Caribbean Sea were investigated to provide ground truth for correlating temperature and chlorophyll-a measurements with observations from NASA U-2 aircraft equipped with specially designed sensors for measuring ocean color phenomena. The physical, chemical, and biological data obtained is summarized. Sampling procedures and methods used for determining plant pigments, species composition of phytoplankton, nutrient salt analysis, and the euphotic zones are described

Functional bioglass/carbon nanocomposite scaffolds from vat photopolymerization of a novel preceramic polymer-based nanoemulsion

Silicone polymers are already known as feedstock for a variety of silicate bioceramics, in the form of scaffolds with complex shapes, obtained by Vat Photopolymerization. Printing is enabled by using silicone blended with photocurable acrylates. The development of a particular silicate composition that functions as a glass or glass-ceramic precursor is possible by the addition of suitable oxide fillers (especially carbonate powders), suspended in the polymer blend. Oxides, from the fillers, easily react with silica left by the thermal transformation of the silicone. The fillers, however, also determine complications in Vat Photopolymerization, due to light scattering; in addition, local oxide concentrations generally impede the obtainment of glassy products. The present paper illustrates a simple solution to these issues, based on the inclusion of a Ca salt in nano-emulsion within a silicone-containing blend. Homogeneous printed samples are later converted into crack-free, fully amorphous ceramic composites, by firing at only 700 °C. The glass matrix, resembling 70S30C (70 % SiO2 and 30 % CaO) bioglass, is achieved according to the quasi-molecular CaO distribution. The secondary phase, promoted by treatment in N2 atmosphere and consisting of pyrolytic carbon, provides a marked photothermal effect

Anti-VEGFR-2 Kinase Effects of Cyclo (Nα-dinicotinoyl)-bis-[(L-valinyl)- L-lysine] and its Anticancer Activities Against Different Cancer Cell Lines

The current work aimed at preparing a cyclo (Nα-dinicotinoyl)-bis-[(L-valinyl)-L-lysine] from previously established synthetic routs. The derivative was investigated for its potential anticancer activities as well as its possible mechanism of action. The prepared compound showed variable anticancer activities against all tested cell lines. Furthermore, it showed very promising activities in terms of obtained IC50 values compared to known used drugs. The mechanism of action studies showed that the prepared tripeptide may act on cancerous cells through its inhibitory action on tyrosine kinase pathway. Animal model experiments proved the potential of the synthesized tripeptide as an anticancer agent against PC3 cancer cells