Atypical presentation of neuronal ceroid lipofuscinosis type 8 in a sibling pair and review of the eye findings and neurological features.

Purpose:To report atypical presentation of neuronal ceroid lipofuscinoses type 8 (CLN8) to the eye clinic and review clinical features of CLN8. Observations:Detailed eye exam by slit lamp exam, indirect ophthalmoscopy, fundus photography, optical coherence tomography, visual fields and electroretinogram (ERG). Molecular genetic testing using Next Generation Sequencing panel (NGS) and array Comparative Genomic Hybridization (aCGH).The siblings in this study presented to the eye clinic with retinitis pigmentosa and cystoid macular edema, and a history of seizures but no severe neurocognitive deficits or regression. Genetic testing identified a c.200C > T (p.A67V) variant in the CLN8 gene and a deletion encompassing the entire gene. Electron microscopy of lymphocytes revealed fingerprint inclusions in both siblings. Conclusions:and Importance: Pathogenic variants in CLN8 account for the retinitis pigmentosa and seizures in our patients however, currently, they do not have regression or neurocognitive decline. The presentation of NCL can be very diverse and it is important for ophthalmologists to consider this in the differential diagnosis of retinal disorders with seizures or other neurological features. Molecular genetic testing of multiple genes causing isolated and syndromic eye disorders using NGS panels and aCGH along with additional complementary testing may often be required to arrive at a definitive diagnosis

Fasilitas Budidaya Bunga Potong Dan Pasar Bunga Di Tomohon “Arsitektur Lansekap”

Kota Tomohon merupakan salah satu kota yang terletak di Provinsi Sulawesi Utara yang sangat kaya akan kehidupan sosial-budaya serta sumber daya alam yang memiliki potensi yang baik dalam bidang pertanian. Saat ini mas yarakat kota Tomohon telah membudidayakan bunga potong . Namun saat ini, bunga potong yang di jual, sebagian tidak berasal dari Kota Tomohon melainkan dari beberapa daerah lain di Indonesia seperti pulau jawa yang di pesan dan di kirim ke Tomohon. Hal ini disebabkan karena keterbatasan memperoleh bibit bunga potong dan belum tersedianya wadah yang representatif dan memadai dalam memelihara bunga potong. Oleh karena itu, perancangan Fasilitas Budidaya Bunga Potong dan Pasar Bunga di Kota Tomohon ini diharapkan dapat memenuhi kebutuhan bagi petani bunga potong dalam pembudidayan bunga potong, bagi penjual bunga potong dan bagi wisatawan dalam berkreasi serta menikmati alam yang ada disekitar objek Fasilitas Budidaya Bunga Potong dan Pasar Bunga di Tomohon. Arsitektur Lansekap merupakan tema yang diangkat dalam perancangan Fasilitas Budidaya Bunga Potong dan Pasar Bunga tersebut. Pemahaman Arsitektur Lansekap yaitu ilmu dan seni perencanaan dan perancangan serta pengaturan dari pada lahan, penyusunan elemen-elemen alam dan buatan melalui aplikasi ilmu pengetahuan dan budaya, dengan memperhatikan keseimbangan kebutuhan pelayanan dan pemeliharaan sumber daya, hingga pada akhirnya dapat tersajikan suatu lingkungan yang estetis dan fungsional. Fungsi dari Arsitektur Lansekap lebih kepada perencanaan ruang luar, dimana lansekap ini akan menjadi penghubung antara manusia dengan alam. Melalui perencanaan Fasilitas Budidaya Bunga Potong dan Pasar Bunga di Tomohon ini diharapkan bisa menjadi fasilitas budidaya bunga potong, tempat jual beli bunga dan menjadi fasilitas pariwisata di Kota Tomoho

Molecular phylogenies disprove a hypothesized C4 reversion in Eragrostis walteri (Poaceae)

Background and Aims The main assemblage of the grass subfamily Chloridoideae is the largest known clade of C4 plant species, with the notable exception of Eragrostis walteri Pilg., whose leaf anatomy has been described as typical of C3 plants. Eragrostis walteri is therefore classically hypothesized to represent an exceptional example of evolutionary reversion from C4 to C3 photosynthesis. Here this hypothesis is tested by verifying the photosynthetic type of E. walteri and its classification. Methods Carbon isotope analyses were used to determine the photosynthetic pathway of several E. walteri accessions, and phylogenetic analyses of plastid rbcL and ndhF and nuclear internal transcribed spacer DNA sequences were used to establish the phylogenetic position of the species. Results Carbon isotope analyses confirmed that E. walteri is a C3 plant. However, phylogenetic analyses demonstrate that this species has been misclassified, showing that E. walteri is positioned outside Chloridoideae in Arundinoideae, a subfamily comprised entirely of C3 species. Conclusions The long-standing hypothesis of C4 to C3 reversion in E. walteri is rejected, and the classification of this species needs to be re-evaluate

Pengaruh Pengalaman Audit, Keahlian Audit dan Tekanan Ketaatan terhadap Audit Judgment Bpk RI Perwakilan Provinsi Sulawesi Utara

This study aimed to examine the effect of audit experience, audit expertise, and obedience pressure to audit judgment taken by auditor. The sample of this research are auditors who worked on Badan Pemeriksa Keuangan (BPK) RI Representative of Nort Sulawesi Province. The sample was conducted by purposive sampling method. Collecting data was conducted by questionnare distributed directly to auditors as much 50 and only 38 questionnare replayed. Data analysis used multiple linear regression method with SPSS (Statistical Product and Service Solution). The result of this research showed that audit experience significantly affect audit judgment taken by auditor, obedience pressure significantly affect audit judgment taken by auditor, but audit expertise didn\u27t significantly affect audit judgment taken by auditor, because the audit expertise in variabel measurement only use indicator : adetailed knowledge of the financial statements, have the ability to perform an audit in accordance with the regulations and have many types of certification / recognition of the expertise of technical training (does not represent an operational definition of variables). So that the results are less present facts on the ground

Determining the Locations of Dust Sources in FeLoBAL Quasars

We conduct a spectroscopic search of quasars observed by the Sloan Digital Sky Survey (SDSS) with broad absorption line (BAL) troughs due to Mg II and troughs due to Fe II that simultaneously exhibit strong Balmer narrow emission lines (NELs). We find that in a redshift range of 0.4 less than or equal to z less than or equal to 0.9 approximately 23 of the 70 Mg II BALs and 4 of a subset of 15 Fe II BALs exhibit strong Balmer emission. We also find significant fractions of Mg II BALs (approximately 23%) and those Mg II BALs with Fe II troughs (approximately 27%) have strong continuum reddening, E(B - V) greater than or equal to 0.1. From measurements of the Balmer decrement in three objects, we find similarly significant reddening of the NEL region in three of the four objects; the NELs in the fourth object are not measurable. We also include one object in this study not taken from the SDSS sample that shows Fe II absorption and strong narrow emission, but due to measurement uncertainty and low continuum reddening the comparison is consistent but inconclusive. We find a trend in both the Mg II and Fe II BAL samples between the NEL reddening and continuum reddening. Because the narrow line reddening is consistent with the continuum reddening in every object in the two SDSS samples, it suggests that the reddening sources in these objects likely exist at larger radial distances than the narrow line regions from the central nucleus.Comment: 40 manuscript pages, accepted in ApJ (July

Delivering a “Dose of Hope”: A Faith-Based Program to Increase Older African Americans’ Participation in Clinical Trials

Background: Underrepresentation of older-age racial and ethnic minorities in clinical research is a significant barrier to health in the United States, as it impedes medical research advancement of effective preventive and therapeutic strategies. Objective: The objective of the study was to develop and test the feasibility of a community-developed faith-based intervention and evaluate its potential to increase the number of older African Americans in clinical research. Methods: Using a cluster-randomized design, we worked with six matched churches to enroll at least 210 persons. We provided those in the intervention group churches with three educational sessions on the role of clinical trials in addressing health disparity topics, and those in the comparison group completed surveys at the same timepoints. All persons enrolled in the study received ongoing information via newsletters and direct outreach on an array of clinical studies seeking participants. We evaluated the short-, mid-, and longer-term effects of the interventional program on clinical trial-related outcomes (ie, screening and enrollment)

Can microsatellite data allow identification of oleaster Plio-Pleistocene refuge zones in the Mediterranean Basin?

International audienc

Continuous EB welding of the reinforcement of the CMS conductor

The Compact Muon Solenoid (CMS) is one of the general-purpose detectors to be provided for the LHC project at CERN. The design field of the CMS superconducting magnet is 4 T, the magnetic length is 12.5 m and the free bore is 6 m. In order to withstand the electro-mechanical forces during the operation of the CMS magnet, the superconducting cable embedded in a 99.998% pure aluminum matrix is reinforced with two sections of aluminum alloy EN AW-6082 assembled by continuous Electron Beam Welding (EBW). A dedicated production line has been designed by Techmeta, a leading company in the field of EBW. The production line has a total length of 70 m. Non-stop welding of each of the 20 lengths of 2.5 km, required to build the coil, will last 22 hours. EBW is the most critical process involved in the production line. The main advantage of the EBW process is to minimize the Heat Affected Zone; this is particularly important for avoiding damage to the superconducting cable located only 4.7 mm from the welded joints. Two welding guns of 20 kW each operate in parallel in a vacuum chamber fitted with dynamic airlocks. After welding, the conductor is continuously machined on the four faces and on each corner to obtain the required dimensions and surface finish. Special emphasis has been put on quality monitoring. All significant production parameters are recorded during operation and relevant samples are taken from each produced length for destructive testing purposes. In addition, a continuous phased array ultrasonic checking device is located immediately after the welding unit for the continuous welding quality control, along with a dimension laser measurement unit following the machining. (8 refs)

Trudy Rice\u27s Story: Nursing and Race in Oregon History

The article presents an interview with Gertrude Rice, known as Trudy, an Oregon-based African American registered nurse (RN) since 1968. Topics include her nursing education at Portland Community College (PCC), her employment at Oregon\u27s Holladay Park Hospital and in the State Health Division, and her experiences as an African American supervisor

Bodyweight Perceptions among Texas Women: The Effects of Religion, Race/Ethnicity, and Citizenship Status

Despite previous work exploring linkages between religious participation and health, little research has looked at the role of religion in affecting bodyweight perceptions. Using the theoretical model developed by Levin et al. (Sociol Q 36(1):157–173, 1995) on the multidimensionality of religious participation, we develop several hypotheses and test them by using data from the 2004 Survey of Texas Adults. We estimate multinomial logistic regression models to determine the relative risk of women perceiving themselves as overweight. Results indicate that religious attendance lowers risk of women perceiving themselves as very overweight. Citizenship status was an important factor for Latinas, with noncitizens being less likely to see themselves as overweight. We also test interaction effects between religion and race. Religious attendance and prayer have a moderating effect among Latina non-citizens so that among these women, attendance and prayer intensify perceptions of feeling less overweight when compared to their white counterparts. Among African American women, the effect of increased church attendance leads to perceptions of being overweight. Prayer is also a correlate of overweight perceptions but only among African American women. We close with a discussion that highlights key implications from our findings, note study limitations, and several promising avenues for future research