A typical case of myoclonic epilepsy with ragged red fibers (MERRF) and the lessons learned.

Mitochondrial diseases have a special predilection to involve the brain in view of its high metabolic demand and the tendency for the formation of excitatory neurotransmitters when there is deficiency of intracellular ATP. These diseases have a great phenotypic variation and need a high degree of suspicion. However, some specific syndromes are well defined, both genotypically and phenotypically. Some of the drugs are potentially fatal mitochondrial poisons and an insight into that may be lifesaving as well as prevent serious morbidities.We report a typical case of myoclonic epilepsy with ragged red fibers (MERRF) with classical phenotype and genotype. There was rapid multiaxial deterioration with the introduction of sodium valproate which partly reversed on introducing mitochondrial cocktail and withdrawal of the offending drug.Sodium valproate, phenobarbitone, chloramphenicol and many anti-viral agents are mitochondrial poisons that increase the morbidity and mortality in patients with mitochondrial disease. More harm to the patient can be avoided with insight into this information.

Differing effects of resibufagenin on cinobufatalin- versus marinobufagenin-induced preeclampsia in a rodent model.

OBJECTIVE: Previous studies from this laboratory have demonstrated that the bufodienolide, marinobufagenin, causes a syndrome in the pregnant rat that resembles human preeclampsia. Furthermore, marinobufagenin urinary excretion is elevated in approximately 85% of preeclamptic patients. Resibufagenin, an antagonist to marinobufagenin, completely prevents the syndrome (hypertension, proteinuria, and intrauterine growth restriction) if given from early pregnancy. STUDY DESIGN: We investigated the effects of another bufodienolide, cinobufatalin, to determine if it, likewise, could induce the rat "preeclamptic" syndrome, which it did. We then examined whether resibufagenin could prevent the syndrome due to cinobufatalin. RESULTS: Resibufagenin improved hypertension but not proteinuria, and did not prevent uterine growth restriction. CONCLUSION: We conclude that more than one bufodienolide may induce the preeclamptic syndrome and that each may require a specific antagonist to prevent (or treat) the syndrome.

The role of selenium and zinc oxide nanoparticles on mitigating side effects of obesity in rats.

Obesity and related diseases represent greatest threats to human health. Nanoparticles (NPs) serve to reduce toxicity; reinforce bioactivity and improve targeting. This study was intended to investigate the antiobesity and antioxidant activities of selenium and zinc oxide nanoparticles. METHODS: Twenty four adult male rats were divided into four groups, group1 control rats fed normal diet and the other three groups were fed high fat diet (HFD) for 10 weeks to induce obesity and injected intraperitoneally with saline, SeNPs (30µg/kg b.wt) and ZnONPs (5mg/kg b.wt) respectively on the last two weeks of feeding (9th and 10th). RESULTS: HFD increased body weight, oxidative stress as indicated by elevated lipid peroxidation and decreased glutathione and catalase levels, increased significantly serum lipid fractions, leptin, liver enzymes, creatinine and uric acid. While causing a substantial decrease in HDL-C and thyroid hormone T4 levels. The results confirmed that treatment with SeNPs and ZnONPs significantly reduced body weight, MDA and improved liver and kidney functions, ameliorated serum lipid fractions level and significantly increased glutathione, catalase, HDL-C and thyroid hormone. CONCLUSION: SeNPs and ZnONPs significantly mitigate hyperlipidemia and oxidative stress. So, they might be potential candidate for obesity amelioration.

An intense stratospheric jet on Jupiter.

The Earth's equatorial stratosphere shows oscillations in which the east-west winds reverse direction and the temperatures change cyclically with a period of about two years. This phenomenon, called the quasi-biennial oscillation, also affects the dynamics of the mid- and high-latitude stratosphere and weather in the lower atmosphere. Ground-based observations have suggested that similar temperature oscillations (with a 4-5-yr cycle) occur on Jupiter, but these data suffer from poor vertical resolution and Jupiter's stratospheric wind velocities have not yet been determined. Here we report maps of temperatures and winds with high spatial resolution, obtained from spacecraft measurements of infrared spectra of Jupiter's stratosphere. We find an intense, high-altitude equatorial jet with a speed of approximately 140 m s(-1), whose spatial structure resembles that of a quasi-quadrennial oscillation. Wave activity in the stratosphere also appears analogous to that occurring on Earth. A strong interaction between Jupiter and its plasma environment produces hot spots in its upper atmosphere and stratosphere near its poles, and the temperature maps define the penetration of the hot spots into the stratosphere.

Predictive Value of the Pattern of ß-Catenin Expression for Pathological Response to Neoadjuvant Chemotherapy in Breast Cancer Patients.

PURPOSE: This study aimed to explore the association of ß-catenin expression pattern with pathological response after neoadjuvant chemotherapy in breast cancer (BC) patients. MATERIALS AND METHODS: In this retrospective exploratory study, data for 50 BC patients who received neoadjuvant chemotherapy were recorded. ß-catenin expression in tumours was assessed using immunohistochemistry and classified as either membranous or cytoplasmic according to the pattern of staining. Distributions of different clinico-pathological parameters according to ß-catenin expression were assessed using the Chi-square test. Logistic regression analysis was used to assess any relation of the pattern of ß-catenin expression with the pathological response. RESULTS: Cytoplasmic ß-catenin expression was detected in 34% of BCs. Among our cases, 52% were hormonal receptor (HR)-positive, 24% were HER2-positive, 74% were clinical stage III and 74% received both anthracycline and taxane-based chemotherapy. Patients with cytoplasmic expression were more commonly younger than 40 years at diagnosis (cytoplasmic, 41.2% vs. no cytoplasmic expression, 12.1%, p=0.03). By doing t-test, cytoplasmic ß-catenin expression was linked with a higher body mass index compared to membranous-only expression (mean± SD 33.0 ± 4.47 vs. 29.6 ±6.01, respectively, p=0.046). No significant associations were found between ß-catenin expression and other parameters such as HR and HER2 status, or clinical stage. Complete pathological response (pCR) rate was twice as great in patients with membranous expression but without statistical signi cance (membranous- only, 33.3% vs. cytoplasmic, 17.6%, OR=2.3, 95% CI= 0.55-9.87, p=0.24). CONCLUSIONS: This study suggests that cytoplasmic ß-catenin expression may be linked with lower probability of achieving pCR after neoadjuvant chemotherapy. These data need to be validated in a larger cohort of patients.

Fast pyrolysis char - Assessment of alternative uses within the bioliq® concept.

Experiments with a process development unit for fast pyrolysis of biomass residues of 10kgh(-1) have been performed to quantify the impact of two different product recovery options. Wheat straw, miscanthus and scrap wood have been used as feedstock. A separate recovery of char increases the organic oil yield as compared to a combined recovery of char and organic condensate (OC). Furthermore, it allows for an alternative use of the byproduct char which represents an important product fraction for the high ash biomass residues under consideration. The char produced shows little advantage over its biomass precursor when considered as energy carrier due to its high ash content. Significant value can be added by demineralizing and activating the char. The potential to increase the economic feasibility of fast pyrolysis is shown by an assessment of the bioliq® process chain.

GnRH and oxytocin have nonidentical effects on the cellular LH response by gonadotrophs at pro-oestrus.

For full fertility in the female ovulation is necessary, which is dependent on the production of a surge of LH. An understanding of the processes which result in the high levels of LH requires delineation of the activities of individual component cells. In this study the responses of gonadotrophs to two signalling hypothalamic peptides, GnRH and oxytocin, were investigated. A cell immunoblot method was used to identify and distinguish between cells which secrete LH and those which contain LH but do not secrete the glycohormone. Rats were killed on the morning of pro-oestrus, the pituitary collected and the cells dispersed onto a protein-binding membrane for study. Cells were then incubated with GnRH and oxytocin, after which the membranes including the attached cells were stained by immunocytochemistry for LH. GnRH increased the total number of immunopositive cells which were present in a concentration-dependent manner. The most prominent change after 2 h incubation was in the number of secreting cells, whereas after 4 h there was also a marked increase in numbers of nonsecreting cells. Oxytocin also increased the total number of immunopositive cells in a concentration-responsive manner, however the profile of action of oxytocin was different from that observed for GnRH. Oxytocin had a relatively greater effect on numbers of immunopositive nonsecreting cells. Thus, the results reveal the potential for gonadotrophs to be flexibly and appropriately modulated by selected hypothalamic peptides. When cells were preincubated with oxytocin prior to GnRH there was not an additive increase in the numbers of immunopositive cells, suggesting that the two agonists act, in a nonidentical manner, on similar cells. The increase in the total number of immunopositive cells implies that there was a production of LH or post-translational processing, induced by exposure to GnRH or oxytocin. The results confirmed the heterogeneity of gonadotrophs and the existence of functionally distinguishable subpopulations, and revealed a difference between the effects of GnRH and oxytocin on expression and secretion of LH.

Inhibition of growth of a murine squamous cell carcinoma by a cyclooxygenase inhibitor increases leukotriene B4 production.

OBJECTIVE: To determine the role of metabolites of arachidonic acid in the growth of squamous cell carcinomas of the head and neck. DESIGN: Investigation of the effect of a cyclooxygenase inhibitor, piroxicam, on the growth of squamous cell carcinoma in a murine model. INTERVENTION: C3H/HeJ mice bearing squamous cell carcinoma (SCCVII) were treated with piroxicam (0.08 mg/d, orally) for 30 days beginning 1 day before tumor inoculation. MAIN OUTCOME MEASURES: Decrease in tumor volumes and tumor growth rates. RESULTS: Significant inhibition of tumor growth (P = .002) and final tumor weight (P = .0007) was noted in the group receiving piroxicam therapy. Prostaglandin E2 levels in the tumor tissue were unrelated to treatment or tumor size. Increased levels of leukotriene B4 were observed in the piroxicam-treated group (P = .03), and larger tumors were associated with decreased leukotriene B4 levels (P = .0001). CONCLUSIONS: Cyclooxygenase inhibitors may be effective in the treatment of some squamous cell carcinomas. The therapeutic effect of cyclooxygenase inhibitors may result from shunting into the lipoxygenase pathway of arachidonic acid metabolism.

Titan's atmospheric temperatures, winds, and composition.

Temperatures obtained from early Cassini infrared observations of Titan show a stratopause at an altitude of 310 kilometers (and 186 kelvin at 15 degrees S). Stratospheric temperatures are coldest in the winter northern hemisphere, with zonal winds reaching 160 meters per second. The concentrations of several stratospheric organic compounds are enhanced at mid- and high northern latitudes, and the strong zonal winds may inhibit mixing between these latitudes and the rest of Titan. Above the south pole, temperatures in the stratosphere are 4 to 5 kelvin cooler than at the equator. The stratospheric mole fractions of methane and carbon monoxide are (1.6 +/- 0.5) x 10(-2) and (4.5 +/- 1.5) x 10(-5), respectively.

Temperatures, winds, and composition in the saturnian system.

Stratospheric temperatures on Saturn imply a strong decay of the equatorial winds with altitude. If the decrease in winds reported from recent Hubble Space Telescope images is not a temporal change, then the features tracked must have been at least 130 kilometers higher than in earlier studies. Saturn's south polar stratosphere is warmer than predicted from simple radiative models. The C/H ratio on Saturn is seven times solar, twice Jupiter's. Saturn's ring temperatures have radial variations down to the smallest scale resolved (100 kilometers). Diurnal surface temperature variations on Phoebe suggest a more porous regolith than on the jovian satellites.

Regulation of C-fos protein in gonadotrope cells by oxytocin and gonadotropin-releasing hormone.

The integrated regulation of luteinizing hormone (LH) from the anterior pituitary gland is vital to the functioning of the ovulatory cycle in the female and consists of several components acting at different time points. The best-studied is the rapid release of LH elicited by gonadotropin-releasing hormone (GnRH). The so-called primary (immediate early) response genes (PRGs), including c-fos, regulate relatively long-term activities, such as mitosis, protein synthesis, protein release and cell differentiation. Regular ovulatory cycles occur as a result of interaction of several peptide factors including the primary factor, GnRH and oxytocin, although GnRH and oxytocin do not have identical activities. We wished to determine whether oxytocin could mediate changes in expression of c-fos protein and compare its effects with those of GnRH. Anterior pituitary glands were collected from female rats at proestrus and a single-cell suspension prepared. Cells were incubated with oxytocin or GnRH at selected concentrations for various times. C-fos protein was extracted and submitted to Western blot analysis. Other cells were stained immunohistochemically for c-fos and LH following incubation with the peptides and fixation. There was an increase in c-fos protein from 15 to 60 min in Western blots of cells from all incubations. After immunohistochemistry, it was observed that both oxytocin (100 nM) and GnRH (100 nM) increased the percentage of cells that expressed c-fos protein (p < 0.001) and of cells that expressed LH (p < 0.001). The responses to the peptides were concentration dependent. We found that neither all LH-containing cells expressed c-fos, nor all c-fos-containing cells immunostained for LH. The effects of the peptides were not the same. High concentrations of GnRH (1 microM) induced the appearance of a higher percent of LH-containing cells having c-fos than did 10 nM GnRH (p < 0.01), whereas a lower percent of LH-containing cells with c-fos were observed when the oxytocin concentration was raised from 10 nM to 1 microM (p < 0.02). It appears, therefore, that the two peptides have different regulatory effects on LH-containing cells, indicating the possibility of specialized function. The results emphasize the suggestion that stimulation of LH secretion is not the sole index of gonadotrope-directed activity by a peptide. Collectively, these results indicate that the peptides oxytocin and GnRH are able to modulate processes that are associated with longer-term activities of gonadotropes and also demonstrate that specific subpopulations of LH-containing gonadotropes are stimulated to express c-fos.

A study of autoimmunity in schistosomiasis.

Circulating auto-antibodies were estimated in sera of ten normal persons and fifty-four schistosomal patients classified into 3 groups: Group I 20 patients with early S. mansoni infection, Group II 20 patients with post schistosomal hepatic fibrosis and Group III 14 patients with post schistosomal glomerulonephritis. A significant high levels of auto antibodies (antinuclear-antismooth muscle and antimitochondrial antibodies) were noticed in Groups II & III. Anti-ds DNA antibodies were significantly higher in Group III than in the other two groups. It was interesting to notice absence of rheumatoid factor in patients with schistosomal glomerulonephritis which may suggest that RF production may have a protective mechanism in patients with schistosomiasis against development of renal disease.

Infrared upconversion for astronomical applications.

The performance of an upconversion system is examined for observation of astronomical sources in the low to middle ir spectral range. Theoretical values for the performance parmeters of an upconversion system for astronomical observations are evaluated in terms of the conversion efficiencies, spectral resolution, field of view minimum detectable source brightness, and source flux. Experimental results of blackbody measurements and molecular absorption spectrum measurements using a lithium niobate upconverter with an argon-ion laser as the pump are presented. Estimates are given of the expected optimum sensitivity of an upconversion device that may be built with presently available components.

Prevalence of Toxoplasma gondii and cytomegalovirus antibodies in patients with chronic renal failure.

This study aimed to determine the prevalence of Toxoplasma gondii and cytomegalovirus (CMV) antibodies in patients with chronic renal failure (CRF) with and without haemodialysis. To achieve this goal, 60 patients with CRF in addition to 20 healthy controls were investigated. Patients were classified into 3 groups, group 1 comprised 20 patients with CRF who haven't any haemodialysis sessions (under conservative treatment) group II comprised 20 patients with CRF due to primary renal diseases already on haemodialysis and group III comprised 20 patients with renal failure secondary to collagen disease or accompanied by liver disease undergoing haemodialysis. Out of 60 patients, Toxoplasma antibodies were detected in 38.3% and in 15% of the normal controls with a statistical significant difference. From the present results, it was noticed high percentages of positivity for Toxoplasma and CMV antibodies in patients with CRF undergoing haemodialysis which was also correlated to the number of the dialysis sessions. So, it can be concluded that CRF patients undergoing haemodialysis should be screened for Toxoplasma and cytomegalovirus before dialysis to prevent the dissemination of these infections through dialysis procedure.

Inversion technique for IR heterodyne sounding of stratospheric constituents from space platforms.

The techniques which have been employed for inversion of IR heterodyne measurements for remote sounding of stratospheric trace constituents usually rely on either geometric effects based on limb-scan observations (i.e., onion peel techniques) or spectral effects by using weighting functions corresponding to different frequencies of an IR spectral line. An experimental approach and inversion technique are discussed which optimize the retrieval of concentration profiles by combining the geometric and the spectral effects in an IR heterodyne receiver. The results of inversions of some synthetic ClO spectral lines corresponding to solar occultation limb scans of the stratosphere are presented, indicating considerable improvement in the accuracy of the retrieved profiles. The effects of noise on the accuracy of retrievals are discussed for realistic situations.

Sensitivity limits of an infrared heterodyne spectrometer for astrophysical applications.

The sensitivity of an ideal heterodyne spectrometer approaches the quantum detection limit provided the local oscillator power is sufficiently large and the shot noise dominates all other sources of noise. The postintegration minimum-detectable-number of photons/sec for an ideal heterodyne system is (B/tau)((1/2)), where B is the IF bandwidth, and tau is the integration time. For astronomical observations, however, a number of factors (Delta(i)) tend to degrade the sensitivity, a fact that becomes significant particularly when the laser power is insufficient. A discussion and an evaluation of the degradation in sensitivity are given for a heterodyne spectrometer employing a HgCdTe photodiode mixer and tunable diode lasers. The minimum detectable source brightness is considered as a function of the mixer parameters, transmission coefficient of the beam splitter, and local oscillator emission powers. The degradation in the minimum detectable line source brightness that results from the bandwidth being a fraction of the line width is evaluated and plotted as a function of the wavelength and bandwidth for various temperature to mass ratios. It is shown that the minimum achievable degradation [pi(i)(Delta(i))] in the sensitivity of a practical astronomical heterodyne spectrometer is ~30. Estimates of SNR's with which ir line emission from astronomical sources of interest may be detected are given.

On employing Leishmania promastigotes for systemic lupus erythematosus diagnosis.

Based on having definite double stranded DNA determinants in the kinetoplast, it was attempted to use Leishmania promastigotes as an IFAT substrate for the detection of serum anti ds-DNA autoantibodies which are the prominent diagnostic marker of SLE. Serological inspection of different Leishmania preparations revealed that Leishmania is not analogous to Crithidia lucilia as an appropriate ds-DNA substrate. It seems likely that the species-specific molecular characters of the kinetoplast-DNA are influencing the reaction. The issue is an evidence of the distinct differentiation of the K-DNA antigenic determinants in haemoflagellates.

Effect of immunosuppression of the virulence of Giardia lamblia cysts.

This study was done to evaluate the pathological effects of Giardia lamblia cysts obtained from immunosuppressed host on the small intestine and liver of immunocompetent hosts. Thirty mice, subdivided into two groups, were infected with G. lamblia cysts in a dose of 5000 cysts/mouse. The first group (10 mice) was inoculated with Giardia cysts obtained from immunocompetent patients and the second group (20 mice) was inoculated with Giardia cysts obtained from already immunosuppressed mice (injected with cyclophosphamide). Stool examination revealed that mice in both groups passed cysts on day 6 +/- 1 after infection. The pathological changes of small intestine in both groups were more or less similar. The changes were in the form of shortening and widening of the villi with cellular inflammatory infiltrate in the lamina propria. The liver of both groups showed no pathological changes. It can be concluded that, although immunosuppression affects the immune system and the defensive mechanism of the host, yet it has no effects on the virulence of the excreted Giardia lamblia cysts.

Causes of minimal hepatic periportal fibrosis present in Egypt.

This study aimed to elucidate the common causes of minimal periportal fibrosis in Egypt. Out of 50 patients having minimal periportal fibrosis, 22 (44%) had schistosomiasis as a sole aetiology, 33 (66%) had combined schistosomiasis with both HBV and HCV. Rare causes were tuberculosis in 4% of the patients and HIV in 2%. No cause could be elicited in 14% of the patients. It is concluded that although schistosomiasis is the commonest cause of minimal hepatic fibrosis in Egypt, yet it is not the sole aetiological factor.