Esityö ERP-järjestelmää varten sähköstandardin avulla

ERP (Enterprise Resource Planning) -järjestelmät ovat tietojärjestelmiä, joiden tarkoituksena on integroida yrityksen eri toiminnot ja prosessit tehokkaiksi kokonaisuuksiksi sekä yrityksen sisällä että yritysten välillä. PDM (Product Data Manage-ment) eli tuotetiedon hallinta tarkoittaa ohjelmistoa, jolla hallitaan keskitetysti yrityksen tuotteisiin liittyvää tietoa ja tiedostoja. Opinnäytetyön tarkoituksena oli valmistella ja helpottaa Lahti Precision Oy:n siirtymistä uuteen ERP-järjestelmään. Työssä määriteltiin yrityksessä yleisesti käytetyt sähkökomponentit sekä lisättiin näiden teknisiä tietoja tietokantaan. Tämä työ toimi pohjana kattavan nimikkeistön luomiselle yrityksessä käyttöönotettavaan ja osittain jo käytettyyn PDM-järjestelmään sekä myöhemmin uuteen ERP-järjestelmään. Työn teoriaosuudessa käsitellään toiminnanohjausjärjestelmiä, tuotetiedon hallintaa sekä standardisoimista yleisellä tasolla, mutta myös Lahti Precisionin näkökulmasta. Työn käytännön osuudessa tarkoituksena oli avustaa Lahti Precisionin sähkökomponenttien sekä -laitteiden standardisointia ja siten helpottaa yhtiössä tapahtuvaa siirtymistä ERP-tietojärjestelmään. Standardisoimiseen liittyi myös esimerkiksi komponenttien mitoittaminen ja muun siihen liittyvän tiedon kerääminen kattavan ja selkeän tietokannan muodostamiseksi. Tietokanta siirretään myöhemmin PDM-järjestelmään. Tarkoituksena oli myös saada mitoitetut komponentit helposti käytettäväksi CADS Planner Electric -suunnitteluohjelmistolla tehtäviin keskuskaappien layout-kuviin. Yrityksen sähkösuunnittelijoille suunnatun kyselyn perusteella kävi ilmi, että uuden ERP-järjestelmän hankkiminen on lähes kaikkien tiedossa, mutta yleinen tietämys järjestelmän konseptista on puutteellista. Komponenttien standardisointi on koettu hyödylliseksi, mutta CADS-ohjelmiston suunnittelua helpottavien toimintojen käyttö on ollut vähäistä.Enterprise Resource Planning (ERP) is a system which is intended to manage all the information and functions of a company, thereby creating an effective entirety. Product Data Management is a function that is responsible for the creation, management and publication of product data. The purpose of this thesis was to prepare and assist Lahti Precision Ltd in the transition to a new ERP system by standardizing the commonly used electrical components and expanding their technical data to create a more versatile database. The database is used as a basis for the future PDM and ERP systems. In the theoretical part, the ERP systems, Product Data Management and standardization are described mostly in a general way but also from the viewpoint of Lahti Precision. The practical part consists of standardizing Lahti Precision's electrical components and equipment, which serves as a preparation to the implementation of a new ERP system. This standardizing also includes gathering important technical data, most importantly external sizes, of the components to create a comprehensive and clear database. The sizes of the components were attached to the items, and therefore they can more easily be used in designing layouts of electrical cabinets. The results of a questionnaire aimed at the electrical designers of the company indicate that the standardization was helpful but the newly implemented functions to ease layout designing were not used much. The questionnaire also revealed that the personnel were mostly aware of the plans for a new ERP system but general knowledge of the system was insufficient

Leaves, berries and herbivorous larvae of bilberry Vaccinium myrtillus as sources of metals in food chains at a Cu-Ni smelter site

Ericaceous dwarf shrubs, such as bilberry, Vaccinium myrtillus, have an important role in nutrient cycling of boreal forests, but in metal polluted environments they also form a link between heavy metal pool of the soil, primary consumers and upper trophic levels. From the viewpoint of metal transfer in a food chain, we document metallic element (As, Ca, Cd, Co, Cu, Mn, Mo, Ni, Pb, Se, Zn) concentrations in leaves, berries and herbivorous larvae of V. myrtillus around a Finnish copper-nickel smelter and compare those with levels in relatively unpolluted reference sites, and with levels documented in soil and feces (a proxy of dietary levels) of an insectivorous bird, the pied flycatcher, Ficedula hypoleuca. Herbivorous larvae of the autumnal moth, Epirrita autumnata (Lepidoptera: Geometridae), grown experimentally on V. myrtillus, showed slower growth rate but not higher mortality in the polluted area. In general, metal levels in leaves, berries and larvae were higher in the polluted area and comparable to those reported at other smelter sites in Europe. The levels of the main toxic metals (As, Cd, Cu, Ni, Pb) followed the general pattern: soil > bird feces > leaves > larvae = berries, and levels in V. myrtillus, E. autumnata and F. hypoleuca reflected soil metal levels. The lowest levels were found in those matrices that are most important sources of food for birds and humans, i.e. leaf-eating larvae and berries, reducing a risk of toxic effects.</p

GRINL1A Complex Transcription Unit Containing GCOM1, MYZAP, and POLR2M Genes Associates with Fully Penetrant Recessive Dilated Cardiomyopathy

Background: Familial dilated cardiomyopathy (DCM) is a monogenic disorder typically inherited in an autosomal dominant pattern. We have identified two Finnish families with familial cardiomyopathy that is not explained by a variant in any previously known cardiomyopathy gene. We describe the cardiac phenotype related to homozygous truncating GCOM1 variants.Methods and Results: This study included two probands and their relatives. All the participants are of Finnish ethnicity. Whole-exome sequencing was used to test the probands; bi-directional Sanger sequencing was used to identify the GCOM1 variants in probands' family members. Clinical evaluation was performed, medical records and death certificates were obtained. Immunohistochemical analysis of myocardial samples was conducted. A homozygous GCOM1 variant was identified altogether in six individuals, all considered to be affected. None of the nine heterozygous family members fulfilled any cardiomyopathy criteria. Heart failure was the leading clinical feature, and the patients may have had a tendency for atrial arrhythmias.Conclusions: This study demonstrates the significance of GCOM1 variants as a cause of human cardiomyopathy and highlights the importance of searching for new candidate genes when targeted gene panels do not yield a positive outcome.Peer reviewe

Case reports of two pedigrees with recessive arrhythmogenic right ventricular cardiomyopathy associated with homozygous Thr335Ala variant in DSG2

TAUSTA Arytmogeeninen oikean kammion kardiomyopatia (ARVC) on perinnöllinen sydänlihassairaus, johon liittyy sydämen kammioiden lihaskudoksen korvautumista rasva- ja sidekudoksella. Tämä johtaa toistuviin, rasituksessa ilmeneviin kammioperäisiin rytmihäiriöihin, kollapseihin, sydämen vajaatoimintaan sekä äkkikuolemiin. Sairauden periytymistapa on useimmiten autosomaalisesti vallitseva, ja sen penetranssi on vaihteleva. Resessiivisesti periytyvät tautimuodot ovat harvinaisia, ja ne voivat liittyä systeemiseen ihosairauteen. Desmosomaalisten geenien mutaatioiden on ajateltu pääasiallisesti selittävän taudin synnyn. Tarkka molekyyligeneettinen etiologia on kuitenkin edelleen suurilta osin selvittämättä, mikä yhdessä kliinisen kuvan heterogeenisyyden kanssa hankaloittaa taudinmääritystä. AINEISTO JA MENETELMÄT Esittelemme kaksi perhettä (n=20), joiden ARVC-indeksipotilaista löysimme desmogleiini-2 (DSG2) missense-variantin c.1003A > G, p.(Thr335Ala) käyttämällä seuraavan sukupolven sekvensointipaneeleita. Indeksipotilaiden lasten ja sisarusten kantajuutta selvitimme Sanger-sekvensoinnilla. Tutkimme osallistujat mahdollisuuksien mukaan kliinisesti, EKG:lla ja suurresoluutio-EKG:lla, laboratoriokokein sekä sydämen ultraääni- tai magneettitutkimuksella. Keräsimme myös kliinisiä tietoja saatavilla olevista potilasasiakirjoista. TULOKSET JA PÄÄTELMÄT Löysimme perheistä yhteensä viisi homotsygoottia ja yhdeksän heterotsygoottia DSG2-variantin c.1003A > G kantajaa. Kaikki homotsygootit kantajat täyttivät ARVC:n nykyiset 2010-vuoden diagnostiset kriteerit, kun taas yhdelläkään heterotsygooteista kantajista ei ollut sydänlihassairauteen viittaavia oireita tai löydöksiä. Tulokset ovat johdonmukaisia autosomaalisen resessiivisen periytyvyyden ja täydellisen penetranssin kanssa. Lisätutkimus on tarpeen saadaksemme tarkempaa ymmärrystä ARVC:hen liittyvien geenien harvinaisten varianttien merkityksestä taudin syntyyn. Geenivarianttien patogeenisuuden tunteminen auttaa diagnostiikassa kliinisen kuvan ollessa epäselvä ja ohjaa oikea-aikaista perinnöllisyysneuvontaa.BACKGROUND Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disease, involving changes in ventricular myocardial tissue and leading to fatal arrhythmias. Mutations in desmosomal genes are thought to be the main cause of ARVC. However, the exact molecular genetic etiology of the disease still remains largely inconclusive, and this along with large variabilities in clinical manifestations complicate clinical diagnostics. CASE PRESENTATION We report two families (n = 20) in which a desmoglein-2 (DSG2) missense variant c.1003A > G, p.(Thr335Ala) was discovered in the index patients using next-generation sequencing panels. The presence of this variant in probands’ siblings and children was studied by Sanger sequencing. Five homozygotes and nine heterozygotes were found with the mutation. Participants were evaluated clinically where possible, and available medical records were obtained. All patients homozygous for the variant fulfilled the current diagnostic criteria for ARVC, whereas none of the heterozygous subjects had symptoms suggestive of ARVC or other cardiomyopathies. CONCLUSIONS The homozygous DSG2 variant c.1003A > G co-segregated with ARVC, indicating autosomal recessive inheritance and complete penetrance. More research is needed to establish a detailed understanding of the relevance of rare variants in ARVC associated genes, which is essential for informative genetic counseling and rational family member testing

Does ventral rectopexy improve pelvic floor function in the long term?

Abstract Background: Information is needed on long-term functional results, sequelas, and outcome predictors for laparoscopic ventral mesh rectopexy. Objective: The purpose of this study was to evaluate long-term function postventral rectopexy in patients with external rectal prolapse or internal rectal prolapse in a large cohort and to identify the possible effects of patient-related factors and operative technical details on patient-reported outcomes. Design: This was a retrospective review with a cross-sectional questionnaire study. Settings: Data were collated from prospectively collected registries in 2 university and 2 central hospitals in Finland. Patients: All 508 consecutive patients treated with ventral rectopexy for external rectal prolapse or symptomatic internal rectal prolapse in 2005 to 2013 were included. Interventions: A questionnaire concerning disease-related symptoms and effect on quality of life was used. Main outcome measures: Defecatory function measured by the Wexner score, the obstructive defecation score, and subjective symptom and quality-of-life evaluation using the visual analog scale were included. The effects of patient-related factors and operative technical details were assessed using multivariate analysis. Results: The questionnaire response rate was 70.7% (330/467 living patients) with a median follow-up time of 44 months. The mean Wexner scores were 7.0 (SD = 6.1) and 6.9 (SD = 5.6), and the mean obstructive defecation scores were 9.7 (SD = 7.6) and 12.3 (SD = 8.0) for patients presenting with external rectal prolapse and internal rectal prolapse. Subjective symptom relief was experienced by 76% and reported more often by patients with external rectal prolapse than with internal rectal prolapse (86% vs 68%; p &lt; 0.001). Complications occurred in 11.4% of patients, and the recurrence rate for rectal prolapse was 7.1%. Limitations: This study was limited by its lack of preoperative functional data and suboptimal questionnaire response rate. Conclusions: Ventral mesh rectopexy effectively treats posterior pelvic floor dysfunction with a low complication rate and an acceptable recurrence rate. Patients with external rectal prolapse benefit more from the operation than those with symptomatic internal rectal prolapse. See Video Abstract at http://links.lww.com/DCR/A479

Nordic research in ophthalmology.

To access publisher full text version of this article. Please click on the hyperlink in Additional Link fieldNordic ophthalmologists and vision scientists are active in many fields of eye research. This is most evident at the biannual Nordic Congress of Ophthalmology, most recently held in Malmö in June 2004. The authors here review some of the research in vision and ophthalmology presented at this meeting or published recently by Nordic scientists. This paper does not represent a comprehensive review of all Nordic research in the field, but attempts to give an overview of some of the activities underway in eye research in this part of the world