Abnormal language pathway in children with Angelman syndrome

Angelman syndrome is a genetic disorder characterized by pervasive developmental disability with failure to develop speech. We examined the basis for severe language delay in patients with Angelman syndrome by diffusion tensor imaging. Magnetic resonance imaging/diffusion tensor imaging was performed in 7 children with genetically confirmed Angelman syndrome (age 70 ± 26 months, 5 boys) and 4 age-matched control children to investigate the microstructural integrity of arcuate fasciculus and other major association tracts. Six of 7 children with Angelman syndrome had unidentifiable left arcuate fasciculus, while all control children had identifiable arcuate fasciculus. The right arcuate fasciculus was absent in 6 of 7 children with Angelman syndrome and 1 of 4 control children. Diffusion tensor imaging color mapping suggested aberrant morphology of the arcuate fasciculus region. Other association tracts, including uncinate fasciculus, inferior fronto-occipital fasciculus, inferior longitudinal fasciculus, and corticospinal tract, were identifiable but manifested decreased fractional anisotropy in children with Angelman syndrome. Increased apparent diffusion coefficient was seen in all tracts except uncinate fasciculus when compared to control children. Patients with Angelman syndrome have global impairment of white matter integrity in association tracts, particularly the arcuate fasciculus, which reveals severe morphologic changes. This finding could be the result of a potential problem with axon guidance during brain development, possibly due to loss of UBE3A gene expression.     

Outcome and prognostic features in anaplastic ganglioglioma: analysis of cases from the SEER database

Anaplastic ganglioglioma (AGG) are rare central nervous system tumours. Patient and treatment factors associated with outcome are poorly defined and limited to small retrospective case series and single case reports. Using the Surveillance, Epidemiology, and End Results (SEER) cancer registry, we investigated potential clinicopathological factors that can affect outcome in patients with anaplastic ganglioglioma. Patients with anaplastic ganglioglioma diagnosed between 1973 and 2007 were identified from the SEER database. Kaplan-Meier survival analysis and Cox models were used to examine the effect of variables on overall survival. The variables analysed included patient age at diagnosis, gender, race, tumour location, uni-focal or multi-focal tumour, surgical resection and the use of adjuvant radiotherapy. Fifty-eight patients were identified, with a median age at diagnosis of 25.5 years. Ninety-three percent of patients underwent surgery and 36% received adjuvant radiotherapy. The median overall survival was 28.5 months. The most common tumour site was the temporal lobe (27%). Univariate and multivariate analysis identified surgery and uni-focal disease as important predictors of overall survival. Adjuvant radiotherapy did not influence overall survival. This study represents the largest analysis of anaplastic ganglioglioma to date. Furthermore it also emphasises the role of national tumour databases for furthering our understanding of rare brain tumours and determining management options.     

Biolarvicidal and pupicidal potential of silver nanoparticles synthesized using Euphorbia hirta against Anopheles stephensi Liston (Diptera: Culicidae)

Vector control is a critical requirement in epidemic disease situations, as is an urgent need to develop new and improved mosquito control methods that are economical and effective yet safe for nontarget organisms and the environment. Mosquitoes transmit serious human diseases, causing millions of deaths every year. Use of synthetic insecticides to control vector mosquitoes has caused physiological resistance and adverse environmental effects in addition to high operational cost. Insecticides of synthesized natural products for vector control have been a priority in this area. In the present study, activity of silver nanoparticles (AgNPs) synthesized using Euphorbia hirta (E. hirta) plant leaf extract against malarial vector Anopheles stephensi (A. stephensi) was determined. Range of concentrations of synthesized AgNPs (3.125, 6.25, 12.5, 25, and 50?ppm) and methanol crude extract (50, 100, 150, 200, and 250?ppm) were tested against larvae of A. stephensi. The synthesized AgNPs from E. hirta were highly toxic than methanolic crude extract against malarial vector, A. stephensi. The synthesized AgNPs were characterized by UV-vis spectrum, scanning electron microscopy (SEM), and X-ray diffraction. SEM analyses of the synthesized showed that AgNPs, measuring 30-60?nm in size, were clearly distinguishable. The synthesized AgNPs showed larvicidal effects after 24?h of exposure; however, the highest larval mortality was found in the synthesized AgNPs against the first to fourth instar larvae and pupae of values LC(50) (10.14, 16.82, 21.51, and 27.89?ppm, respectively), LC(90) (31.98, 50.38, 60.09, and 69.94?ppm, respectively), and the LC(50) and LC(90) values of pupae of 34.52 and 79.76?ppm, respectively. Methanol extract exhibited the larval toxicity against the first to fourth instar larvae and pupae of values LC(50) (121.51, 145.40, 169.11, and 197.40?ppm, respectively), LC(90) (236.44, 293.75, 331.42, and 371.34?ppm, respectively), and the LC(50) and LC(90) values of pupae of 219.15 and 396.70?ppm, respectively. No mortality was observed in the control. These results suggest that synthesized silver nanoparticles are a rapid, eco-friendly, and single-step approach; the AgNPs formed can be potential mosquito larvicidal agents.     

Mosquito larvicidal, pupicidal, adulticidal, and repellent activity of Artemisia nilagirica (Family: Compositae) against Anopheles stephensi and Aedes aegypti

Mosquito-borne diseases have an economic impact, including loss in commercial and labor outputs, particularly in countries with tropical and subtropical climates; however, no part of the world is free from vector-borne diseases. The aim of the present study, to evaluate the larvicidal, pupicidal, repellent, and adulticidal activities of methanol crude extract of Artemisia nilagirica were assayed for their toxicity against two important vector mosquitoes, viz., Anopheles stephensi and Aedes aegypti (Diptera: Culicidae). The fresh leaves of A. nilagirica were washed thoroughly in tap water and shade dried at room temperature (28?±?2?°C) for 5?C8?days. The air-dried materials were powdered separately using commercial electrical blender. From the plants, 500?g powdered was macerated with 1.5?L organic solvents of methanol sequentially for a period of 72?h each and filtered. The larval and pupal mortality was observed after 24?h of exposure; no mortality was observed in the control group. The first- to fourth-instar larvae and pupae of A. stephensi had values of LC₅₀?=?272.50, 311.40, 361.51, 442.51, and 477.23?ppm, and the LC₉₀?=?590.07, 688.81, 789.34, 901.59, and 959.30?ppm; the A. aegypti had values of LC₅₀?=?300.84, 338.79, 394.69, 470.74, and 542.11?ppm, and the LC₉₀?=?646.67, 726.07, 805.49, 892.01, and 991.29?ppm, respectively. The results of the repellent activity of plant extract of A. nilagirica plants at five different concentrations of 50, 150, 250, 350, and 450?ppm were applied on skin of fore arm in man and exposed against adult female mosquitoes. In this observation, the plant crude extract gave protection against mosquito bites without any allergic reaction to the test person, and also, the repellent activity is dependent on the strength of the plant extracts. The adult mortality was found in methanol extract of A. nilagirica, with the LC₅₀ and LC₉₀ values of 205.78 and 459.51?ppm for A. stephensi, and 242.52 and 523.73?ppm for A. aegypti, respectively. This result suggests that the leaf extract have the potential to be used as an ideal eco-friendly approach for the control of vector mosquito as target species.     

Fahr's syndrome: report of two cases

Two cases (one female and one male) of Fahr's syndrome are reported here. Both of them had neuropsychiatric disturbances and intracranial calcifications in bilateral basal ganglia and cerebral hemispheres. CT scan helped in establishing the diagnosis. Both of them were treated with antipsychotic drugs and responded to some extent.     

Duodenojejunal intussusception in Peutz-Jeghers syndrome: Report of a case

Recurrent intussusception is one of the common modes of presentation in patients with Peutz-Jeghers syndrome. This report presents a case of Peutz-Jeghers syndrome with synchronous duodenojejunal and jejunojejunal intussusceptions due to hamartomatous polyps. Very few cases of duodenojejunal intussusception in Peutz-Jeghers syndrome patients have so far been reported in the literature.     

Transarterial radioembolization with Yttrium-90 for regional management of hepatocellular cancer: the early results of a nontransplant center

Selective arterial radioembolization with Yttrium-90 (Y-90) microspheres has shown promise for regional management of hepatocellular cancer (HCC). Our objective was to report our early experience with this treatment modality from a nontransplant center. Treatment of patients with HCC was discussed in a multidisciplinary tumor board. Patients with unresectable disease resulting from high lesion number, ill location of the tumor, poor hepatic reserve, or medical comorbidities were offered Y-90 treatment. Liver treatment was either lobar or tumor-targeted. Response to therapy was assessed by CT scan obtained within 3 months using Response Evaluation Criteria in Solid Tumors criteria. During 2007 to 2009, 40 Y-90 radioembolizations were performed in 20 patients with age that ranged from 16 to 87 years; four patients were 80 years old or older. After the first therapy, CT assessment of the treated area showed stable disease (n=15), partial response (n=3), and progression (n=2). Of the two patients who progressed, one was retreated with a subsequent complete response. The other patient died of progressive disease. The most common side effects were mild fatigue, anorexia, and nausea. In summary, our nontransplant center experience shows that Y-90 radioembolization is a well-tolerated treatment in select patients with unresectable HCC with an associated high rate of local tumor control.     

The relationships of nutrients, routes of delivery, and immunocompetence

Malnutrition has marked consequences on surgical outcomes. Adequate nutrition is important for the proper functioning of all organ systems, particularly the immune system. Determination of the type and amount of nutrient supplementation and the appropriate route of nutrient delivery is essential to bolster the immune system and enhance the host's response to stress. Correct administration of immunonutrients could lead to reductions in patient morbidity following major surgery, trauma, and critical illness.