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  1. Article: Optimal Resilience Enhancement of Water Distribution Systems

    Lorenz, Imke-Sophie / Pelz, Peter F

    Water. 2020 Sept. 17, v. 12, no. 9

    2020  

    Abstract: Water distribution systems (WDSs) as critical infrastructures are subject to demand peaks due to daily consumption fluctuations, as well as long term changes in the demand pattern due to increased urbanization. Resilient design of water distribution ... ...

    Abstract Water distribution systems (WDSs) as critical infrastructures are subject to demand peaks due to daily consumption fluctuations, as well as long term changes in the demand pattern due to increased urbanization. Resilient design of water distribution systems is of high relevance to water suppliers. The challenging combinatorial problem of high-quality and, at the same time, low-cost water supply can be assisted by cost-benefit optimization to enhance the resilience of existing main line WDSs, as shown in this paper. A Mixed Integer Linear Problem, based on a graph-theoretical resilience index, is implemented considering WDS topology. Utilizing parallel infrastructures, specifically those of the urban transport network and the water distribution network, makes allowances for physical constraints, in order to adjust the existing WDS and to enhance resilience. Therefore, decision-makers can be assisted in choosing the optimal adjustment of WDS depending on their investment budget. Furthermore, it can be observed that, for a specific urban structure, there is a convergence of resilience enhancement with higher costs. This cost-benefit optimization is conducted for a real-world main line WDS, considering also the limitations of computational expenses.
    Keywords decision making ; topology ; urbanization ; water ; water distribution ; water supply
    Language English
    Dates of publication 2020-0917
    Publishing place Multidisciplinary Digital Publishing Institute
    Document type Article
    Note NAL-AP-2-clean
    ZDB-ID 2521238-2
    ISSN 2073-4441
    ISSN 2073-4441
    DOI 10.3390/w12092602
    Database NAL-Catalogue (AGRICOLA)

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  2. Article ; Online: Missense variant c.1460 T > C (p.L487P) enhances protein degradation of ER mannosyltransferase ALG9 in two new ALG9-CDG patients presenting with West syndrome and review of the literature.

    Himmelreich, Nastassja / Dimitrov, Bianca / Zielonka, Matthias / Hüllen, Andreas / Hoffmann, Georg Friedrich / Juenger, Hendrik / Müller, Herbert / Lorenz, Imke / Busse, Birgit / Marschall, Christoph / Schlüter, Gregor / Thiel, Christian

    Molecular genetics and metabolism

    2022  Volume 136, Issue 4, Page(s) 274–281

    Abstract: ALG9-CDG is a CDG-I defect within the group of Congenital Disorders of Glycosylation (CDG). We here describe the clinical symptoms of two new and unrelated ALG9-CDG patients, both carrying the novel homozygous missense variant c.1460 T > C (p.L487P) in ... ...

    Abstract ALG9-CDG is a CDG-I defect within the group of Congenital Disorders of Glycosylation (CDG). We here describe the clinical symptoms of two new and unrelated ALG9-CDG patients, both carrying the novel homozygous missense variant c.1460 T > C (p.L487P) in the ALG9 gene which led to global developmental delay, psychomotor disability, facial dysmorphisms, brain and heart defects, hearing loss, hypotonia, as well as feeding problems. New clinical symptoms comprised West syndrome with hypsarrhythmia. Quantitative RT-PCR analysis revealed a significantly enhanced ALG9 mRNA transcript level, whereas the protein amount in fibroblasts was significantly reduced. This could be ascribed to a stronger degradation of the mutated ALG9 protein in patient fibroblasts. Lipid-linked oligosaccharide analysis showed an ALG9-CDG characteristic accumulation of Man
    MeSH term(s) Congenital Disorders of Glycosylation/genetics ; Humans ; Infant ; Male ; Mannosyltransferases/genetics ; Membrane Proteins/genetics ; Proteolysis ; Spasms, Infantile/genetics
    Chemical Substances Membrane Proteins ; ALG9 protein, human (EC 2.4.1.-) ; Mannosyltransferases (EC 2.4.1.-)
    Language English
    Publishing date 2022-06-26
    Publishing country United States
    Document type Journal Article ; Review ; Research Support, Non-U.S. Gov't
    ZDB-ID 1418518-0
    ISSN 1096-7206 ; 1096-7192
    ISSN (online) 1096-7206
    ISSN 1096-7192
    DOI 10.1016/j.ymgme.2022.06.005
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: Compliance with recommended immunizations in adolescents.

    Heininger, Ulrich / Loos, Kerstin / Lorenz, Imke / Rascher, Wolfgang

    European journal of pediatrics

    2006  Volume 165, Issue 10, Page(s) 671–676

    Abstract: Introduction: Little is known about the completeness and timely administration of recommended standard immunizations in Germany. The goal of this study was to determine compliance with official standard immunization recommendations in adolescents ... ...

    Abstract Introduction: Little is known about the completeness and timely administration of recommended standard immunizations in Germany. The goal of this study was to determine compliance with official standard immunization recommendations in adolescents attending secondary schools in the city of Erlangen, Germany.
    Methods: Adolescents who were attending 5th grade (at approximately 11 years of age), 8th grade (14 years), or 10th and 11th grade (16-17 years) classes at any of the 13 of 14 schools that had agreed to participate were eligible to be enrolled.
    Results: While coverage for the primary series of diphtheria, tetanus and poliomyelitis immunizations was satisfactory (98%), coverage for measles-mumps-rubella immunizations (dose 1: 89-96%; dose 2: 60-76%) and hepatitis B (doses 1-3: 61%) was suboptimal. Of note, 39% of students had not received any immunization against pertussis. Completion of immunization series generally was significantly delayed. Furthermore, rates for recommended booster doses in adolescence were disappointingly low with 21% for tetanus component vaccines and <10% for the fifth dose of pertussis.
    Conclusions: Significant immunization gaps for all recommended standard immunizations in adolescents were detected. This puts individuals at risk for serious vaccine-preventable diseases, contributes to suboptimal herd immunity in the population under study leaving the potential for future epidemics, and impedes national and international targets of disease reduction or elimination.
    MeSH term(s) Adolescent ; Child ; Communicable Disease Control ; Cross-Sectional Studies ; Female ; Germany/epidemiology ; Humans ; Immunization/utilization ; Immunization Programs ; Immunization Schedule ; Male ; Measles-Mumps-Rubella Vaccine/administration & dosage ; Patient Compliance ; Students/statistics & numerical data ; Viral Vaccines/administration & dosage ; Virus Diseases/epidemiology ; Virus Diseases/prevention & control ; Whooping Cough/epidemiology ; Whooping Cough/prevention & control
    Chemical Substances Measles-Mumps-Rubella Vaccine ; Viral Vaccines
    Language English
    Publishing date 2006-10
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 194196-3
    ISSN 1432-1076 ; 0340-6199 ; 0943-9676
    ISSN (online) 1432-1076
    ISSN 0340-6199 ; 0943-9676
    DOI 10.1007/s00431-006-0161-9
    Database MEDical Literature Analysis and Retrieval System OnLINE

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