Erdheim Chester disease: Cerebral involvement in childhood

Erdheim Chester disease (ECD) is an uncommon, non-Langerhans form of histiocytosis, characterized by long bone sclerosis. To date, only approximately some 100 cases have been reported. The diagnosis is based on its peculiar radiological and pathological features. ECD disease is rarely expected preoperatively. Although the ECD is an adult illness it can rarely be seen in the childhood period. We reported the case of a 10-year-old boy who presented headache, paraparesis and with diabetes inspidus for 6 years. As far as we know. the case presented here is the first published report of intracranial involvement and unilateral bone sclerosis with ECD in childhood. (c) 2006 Elsevier B.V. All rights reserved

Temperature dependent energy relaxation time in AlGaN/AlN/GaN heterostructures

The two-dimensional (2D) electron energy relaxation in Al0.25Ga0.75N/AlN/GaN heterostructures was investigated experimentally by using two experimental techniques; Shubnikov-de Haas (SdH) effect and classical Hall Effect. The electron temperature (T-e) of hot electrons was obtained from the lattice temperature (T-L) and the applied electric field dependencies of the amplitude of SdH oscillations and Hall mobility. The experimental results for the electron temperature dependence of power loss are also compared with the current theoretical models for power loss in 2D semiconductors. The power loss that was determined from the SdH measurements indicates that the energy relaxation of electrons is due to acoustic phonon emission via unscreened piezoelectric interaction. In addition, the power loss from the electrons obtained from Hall mobility for electron temperatures in the range T-e > 100 K is associated with optical phonon emission. The temperature dependent energy relaxation time in Al0.25Ga0.75N/AlN/GaN heterostructures that was determined from the power loss data indicates that hot electrons relax spontaneously with MHz to THz emission with increasing temperatures. (c) 2012 Elsevier Ltd. All rights reserved

Investigation of Camphor Effects on Fusarium graminearum and F. culmorum at Different Molecular Levels

Fusarium graminearum and F. culmorum are phytopathogens, which cause destructive diseases in cereals. Epidemics of these phytopathogens are caused by mycotoxin contamination and the reduction of crop quality. In this study, the alteration due to in vitro camphor treatment on F. culmorum 9F and F. graminearum H11 isolates was investigated in terms of epigenetic, cellular, and transcription levels. Camphor with different concentrations (0.2, 0.4, 0.8, 1, 2, and 4 mu g/mu L) was applied to potato dextrose agar (PDA) growth media. The minimum inhibitory concentration (MIC) and the half maximal inhibitory concentration (IC50) were calculated as 2 and 1 mu g/mu L respectively. hog1, mst20, CAT, POD, mgv1, stuA, and tri5 genes, which are related to various cellular processes and pathogenesis, were examined by qPCR assay. qPCR analysis showed that camphor treatment leads to the downregulation of tri5 expression but the upregulation of the remaining genes. Apoptosis and oxidative stress were confirmed via acridine orange/ethidium bromide (AO/EB) and dichlorofluorescin diacetate (DCF-DA) staining, respectively. Moreover, coupled restriction enzyme digestion-random amplification (CRED-RA) assay, used for DNA methylation analysis, was carried out to evaluate epigenetic alterations. The decrease in genomic template stability (GTS) values, which resulted due to the alterations in random amplified polymorphic DNA (RAPD) profiles caused by camphor treatment, were detected as 97.60% in F. culmorum 9F and 66.27% in F. graminearum H-11. The outer and inner methylated cytosine profiles are determined by CRED-RA assay as type I-IV epigenetic alterations. The outcomes indicated that camphor could lead to alterations at several molecular levels of F. graminearum and F. culmorum

Three Patients With Lafora Disease: Different Clinical Presentations and a Novel Mutation

WOS: 000353584100016PubMed ID: 25015673Lafora disease is a rare, fatal, autosomal recessive hereditary disease characterized by epilepsy, myoclonus and progressive neurological deterioration. Diagnosis is made by polyglucosan inclusion bodies (Lafora bodies) shown in skin biopsy. Responsible mutations of Lafora disease involves either the EPM2A or NHLRC1 (EPM2B) gene. Mutations in the NHLRC1 gene are described as having a more benign clinical course and a later age of death compared with EPM2A mutations. We report 2 genetic mutations and clinical courses of Lafora disease in 3 adolescents with homozygote NHLRC1 mutation and novel homozygous EPM2A mutation

Temperature dependent energy relaxation time in AlGaN/AlN/GaN heterostructures

The two-dimensional (2D) electron energy relaxation in Al0.25Ga0.75N/AlN/GaN heterostructures was investigated experimentally by using two experimental techniques; Shubnikov-de Haas (SdH) effect and classical Hall Effect. The electron temperature (T-e) of hot electrons was obtained from the lattice temperature (T-L) and the applied electric field dependencies of the amplitude of SdH oscillations and Hall mobility. The experimental results for the electron temperature dependence of power loss are also compared with the current theoretical models for power loss in 2D semiconductors. The power loss that was determined from the SdH measurements indicates that the energy relaxation of electrons is due to acoustic phonon emission via unscreened piezoelectric interaction. In addition, the power loss from the electrons obtained from Hall mobility for electron temperatures in the range T-e > 100 K is associated with optical phonon emission. The temperature dependent energy relaxation time in Al0.25Ga0.75N/AlN/GaN heterostructures that was determined from the power loss data indicates that hot electrons relax spontaneously with MHz to THz emission with increasing temperatures. (c) 2012 Elsevier Ltd. All rights reserved