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  1. Book: The vegetable gardener's bible

    Smith, Edward C.

    achieve the most from your vegetable garden utilizing wider, deeper beds and organic methods ; [a comprehensive A - Z of common vegetables and herbs]

    2005  

    Author's details Edward C. Smith
    Language English
    Size IX, 309 S. : zahlr. Ill.
    Edition Repr.
    Publisher David & Charles
    Publishing place Newton Abbot
    Publishing country Great Britain
    Document type Book
    HBZ-ID HT014414164
    ISBN 0-7153-1781-4 ; 978-0-7153-1781-5
    Database Catalogue ZB MED Nutrition, Environment, Agriculture

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    In stock of ZB MED Bonn / Germany

    Shelf markLoan statusLocation
    054/710 available for loan (reading room) Freihandmagazin (U1)

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  2. Book: Aplastic anaemia

    Gordon-Smith, Edward C.

    (Baillière's clinical haematology ; 2,1)

    1989  

    Author's details E. C. Gordon-Smith, guest ed
    Series title Baillière's clinical haematology ; 2,1
    Collection
    Keywords Anemia, Aplastic ; Aplastische Anämie
    Subject Panmyelophthise ; Aleukia haemorrhagica ; Aregeneratorische Panmyelopathie
    Size IX, 194 S. : Ill., graph. Darst.
    Publisher Baillière Tindall
    Publishing place London u.a.
    Publishing country Great Britain
    Document type Book
    HBZ-ID HT003279998
    ISBN 0-7020-1281-5 ; 978-0-7020-1281-5
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    Se 1029 -2,1- verfügbar in Königswinter Königswinter

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  3. Article ; Online: Transmission loss of plates with embedded multi-scale and tuned acoustic black holes.

    Xiong, Yu / Smith, Edward C / Conlon, Stephen C

    The Journal of the Acoustical Society of America

    2021  Volume 150, Issue 3, Page(s) 2282

    Abstract: An acoustic black hole (ABH) plate is a lightweight and high loss panel structure for effective reduction of vibration and radiated sound. It is understood that the high loss local ABH modes can be designed at desired frequencies by changing the size of ... ...

    Abstract An acoustic black hole (ABH) plate is a lightweight and high loss panel structure for effective reduction of vibration and radiated sound. It is understood that the high loss local ABH modes can be designed at desired frequencies by changing the size of the ABH cell(s). The ABH cell diameter (size) and minimum thickness play dominant roles in the performance of the ABH effect. In addition, attaching tuning masses at the center of the ABH cells has been shown to alter the local ABH modes with the result of improved low-frequency performance. In this work, the transmission loss (TL) of an embedded multi-scale ABH plate was investigated. The embedded large and small ABH cells were particularly designed to cut-on below and above the critical frequency of the plate, respectively. The results were compared with a uniform plate and an embedded single-scale ABH plate. Discrete tuning masses were attached at the ABH cells' center to manipulate the ABH cut-on modes to increase the TL further. The results show that the damped multi-scale ABH plate achieved a 10 dB TL increase, flattened the TL curve, and nearly eliminated the plate coincidence dip. Manipulating the high loss ABH modes by adding tuning masses (20 g each) demonstrated a 2 dB increase at low frequencies within the mass-law range. Although damping material was applied, adding some mass, an overall weight advantage was still attained compared to the uniform plate. The damped multi-scale ABH plate is 7% lighter than the uniform plate.
    Language English
    Publishing date 2021-10-01
    Publishing country United States
    Document type Journal Article
    ZDB-ID 219231-7
    ISSN 1520-8524 ; 0001-4966
    ISSN (online) 1520-8524
    ISSN 0001-4966
    DOI 10.1121/10.0006442
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.B 591: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

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  4. Book: Haematological effects of drug therapy

    Gordon-Smith, Edward C.

    (CLINICS IN HAEMATOLOGY ; 9,3)

    1980  

    Series title CLINICS IN HAEMATOLOGY ; 9,3
    Clinics in haematology
    Collection Clinics in haematology
    Keywords DRUG THERAPY / ADVERSE EFFECTS ; HEMATOLOGIC DISEASES / CHEMICALLY INDUCED
    Language English
    Size S. 453 - 691
    Publisher Saunders
    Publishing place London
    Document type Book
    HBZ-ID HT001098371
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    Se 128 -9,3- verfügbar in Königswinter Königswinter

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  5. Article: Screening data from 19 patients with late-onset Pompe disease for a phase I clinical trial of AAV8 vector-mediated gene therapy.

    Hannah, William B / Case, Laura E / Smith, Edward C / Walters, Crista / Bali, Deeksha / Kishnani, Priya S / Koeberl, Dwight D

    JIMD reports

    2023  Volume 64, Issue 5, Page(s) 393–400

    Abstract: Late-onset Pompe disease (LOPD) is a multisystem disorder with significant myopathy. The standard treatment is enzyme replacement therapy (ERT), a therapy that is lifesaving, yet with limitations. Clinical trials have emerged for other potential ... ...

    Abstract Late-onset Pompe disease (LOPD) is a multisystem disorder with significant myopathy. The standard treatment is enzyme replacement therapy (ERT), a therapy that is lifesaving, yet with limitations. Clinical trials have emerged for other potential treatment options, including adeno-associated virus (AAV) gene therapy. We present clinical parameters and AAV antibody titers for 19 individuals with LOPD undergoing screening for a Phase I clinical trial with an AAV serotype 8 vector targeting hepatic transduction (AAV2/8-LSPhGAA). Reported clinical parameters included
    Language English
    Publishing date 2023-08-17
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2672872-2
    ISSN 2192-8312 ; 2192-8304
    ISSN (online) 2192-8312
    ISSN 2192-8304
    DOI 10.1002/jmd2.12391
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Viral-Mediated Gene Replacement Therapy in the Developing Central Nervous System: Current Status and Future Directions.

    Uchitel, Julie / Kantor, Boris / Smith, Edward C / Mikati, Mohamad A

    Pediatric neurology

    2020  Volume 110, Page(s) 5–19

    Abstract: The past few years have witnessed rapid developments in viral-mediated gene replacement therapy for pediatric central nervous system neurogenetic disorders. Here, we provide pediatric neurologists with an up-to-date, comprehensive overview of these ... ...

    Abstract The past few years have witnessed rapid developments in viral-mediated gene replacement therapy for pediatric central nervous system neurogenetic disorders. Here, we provide pediatric neurologists with an up-to-date, comprehensive overview of these developments and note emerging trends for future research. This review presents the different types of viral vectors used in viral-mediated gene replacement therapy; the fundamental properties of viral-mediated gene replacement therapy; the challenges associated with the use of this therapy in the central nervous system; the pathway for therapy development, from translational basic science studies to clinical trials; and an overview of the therapies that have reached clinical trials in patients. Current viral platforms under investigation include adenovirus vectors, adeno-associated viral vectors, lentiviral/retroviral vectors, and herpes simplex virus type 1 vectors. This review also presents an in-depth analysis of numerous studies that investigated these viral platforms in cultured cells and in transgenic animal models for pediatric neurogenetic disorders. Viral vectors have been applied to clinical trials for many different pediatric neurogenetic disorders, including Canavan disease, metachromatic leukodystrophy, neuronal ceroid lipofuscinosis, mucopolysaccharidosis III, spinal muscular atrophy, and aromatic l-amino acid decarboxylase deficiency. Of these diseases, only spinal muscular atrophy has a viral-mediated gene replacement therapy approved for marketing. Despite significant progress in therapy development, many challenges remain. Surmounting these challenges is critical to advancing the current status of viral-mediated gene replacement therapy for pediatric central nervous system neurogenetic disorders.
    Language English
    Publishing date 2020-04-24
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 639164-3
    ISSN 1873-5150 ; 0887-8994
    ISSN (online) 1873-5150
    ISSN 0887-8994
    DOI 10.1016/j.pediatrneurol.2020.04.010
    Database MEDical Literature Analysis and Retrieval System OnLINE

    Full text online

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 2260: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  7. Article ; Online: Ultrasound of cervical roots and brachial plexus in neonates.

    Grant, Stuart A / Smith, Edward C

    Muscle & nerve

    2015  Volume 51, Issue 4, Page(s) 626

    MeSH term(s) Brachial Plexus/diagnostic imaging ; Cervical Vertebrae/diagnostic imaging ; Female ; Humans ; Male ; Spinal Nerve Roots/ultrastructure ; Ultrasonography
    Language English
    Publishing date 2015-04
    Publishing country United States
    Document type Letter ; Comment
    ZDB-ID 438353-9
    ISSN 1097-4598 ; 0148-639X
    ISSN (online) 1097-4598
    ISSN 0148-639X
    DOI 10.1002/mus.24518
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1449: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)
    Zs.MO 551: Show issues

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  8. Article ; Online: The severity of MUSK pathogenic variants is predicted by the protein domain they disrupt.

    Cocanougher, Benjamin T / Liu, Samuel W / Francescatto, Ludmila / Behura, Alexander / Anneling, Mariele / Jackson, David G / Deak, Kristen L / Hornik, Chi D / ElMallah, Mai K / Pizoli, Carolyn E / Smith, Edward C / Tan, Khoon Ghee Queenie / McDonald, Marie T

    HGG advances

    2024  Volume 5, Issue 3, Page(s) 100288

    Abstract: Biallelic loss-of-function variants in the MUSK gene result in two allelic disorders: (1) congenital myasthenic syndrome (CMS; OMIM: 616325), a neuromuscular disorder that has a range of severity from severe neonatal-onset weakness to mild adult-onset ... ...

    Abstract Biallelic loss-of-function variants in the MUSK gene result in two allelic disorders: (1) congenital myasthenic syndrome (CMS; OMIM: 616325), a neuromuscular disorder that has a range of severity from severe neonatal-onset weakness to mild adult-onset weakness, and (2) fetal akinesia deformation sequence (OMIM: 208150), a form of pregnancy loss characterized by severe muscle weakness in the fetus. The MUSK gene codes for muscle-specific kinase (MuSK), a receptor tyrosine kinase involved in the development of the neuromuscular junction. Here, we report a case of neonatal-onset MUSK-related CMS in a patient harboring compound heterozygous deletions in the MUSK gene, including (1) a deletion of exons 2-3 leading to an in-frame MuSK protein lacking the immunoglobulin 1 (Ig1) domain and (2) a deletion of exons 7-11 leading to an out-of-frame, truncated MuSK protein. Individual domains of the MuSK protein have been elucidated structurally; however, a complete MuSK structure generated by machine learning algorithms has clear inaccuracies. We modify a predicted AlphaFold structure and integrate previously reported domain-specific structural data to suggest a MuSK protein that dimerizes in two locations (Ig1 and the transmembrane domain). We analyze known pathogenic variants in MUSK to discover domain-specific genotype-phenotype correlations; variants that lead to a loss of protein expression, disruption of the Ig1 domain, or Dok-7 binding are associated with the most severe phenotypes. A conceptual model is provided to explain the severe phenotypes seen in Ig1 variants and the poor response of our patient to pyridostigmine.
    Language English
    Publishing date 2024-04-01
    Publishing country United States
    Document type Journal Article
    ISSN 2666-2477
    ISSN (online) 2666-2477
    DOI 10.1016/j.xhgg.2024.100288
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Efficacy and Safety of Viltolarsen in Boys With Duchenne Muscular Dystrophy: Results From the Phase 2, Open-Label, 4-Year Extension Study.

    Clemens, Paula R / Rao, Vamshi K / Connolly, Anne M / Harper, Amy D / Mah, Jean K / McDonald, Craig M / Smith, Edward C / Zaidman, Craig M / Nakagawa, Tomoyuki / Hoffman, Eric P

    Journal of neuromuscular diseases

    2023  Volume 10, Issue 3, Page(s) 439–447

    Abstract: Background: Duchenne muscular dystrophy (DMD) is caused by DMD gene mutations, resulting in absence of functional dystrophin protein. Viltolarsen, an exon 53 skipping therapy, significantly increased dystrophin levels in patients with DMD. Presented ... ...

    Abstract Background: Duchenne muscular dystrophy (DMD) is caused by DMD gene mutations, resulting in absence of functional dystrophin protein. Viltolarsen, an exon 53 skipping therapy, significantly increased dystrophin levels in patients with DMD. Presented here are completed study results of > 4 years of functional outcomes in viltolarsen-treated patients compared to a historical control group (Cooperative International Neuromuscular Research Group Duchenne Natural History Study [CINRG DNHS]).
    Objective: To evaluate the efficacy and safety of viltolarsen for an additional 192 weeks in boys with DMD.
    Methods: This phase 2, open-label, 192-week long-term extension (LTE) study (NCT03167255) evaluated the efficacy and safety of viltolarsen in participants aged 4 to < 10 years at baseline with DMD amenable to exon 53 skipping. All 16 participants from the initial 24-week study enrolled into this LTE. Timed function tests were compared to the CINRG DNHS group. All participants received glucocorticoid treatment. The primary efficacy outcome was time to stand from supine (TTSTAND). Secondary efficacy outcomes included additional timed function tests. Safety was continuously assessed.
    Results: For the primary efficacy outcome (TTSTAND), viltolarsen-treated patients showed stabilization of motor function over the first two years and significant slowing of disease progression over the following two years compared with the CINRG DNHS control group which declined. Viltolarsen was well tolerated, with most reported treatment-emergent adverse events being mild or moderate. No participants discontinued drug during the study.
    Conclusions: Based on the results of this 4-year LTE, viltolarsen can be an important treatment strategy for DMD patients amenable to exon 53 skipping.
    MeSH term(s) Male ; Humans ; Muscular Dystrophy, Duchenne/genetics ; Dystrophin/genetics ; Oligonucleotides/adverse effects ; Glucocorticoids/therapeutic use
    Chemical Substances Dystrophin ; viltolarsen (SXA7YP6EKX) ; Oligonucleotides ; Glucocorticoids
    Language English
    Publishing date 2023-04-08
    Publishing country Netherlands
    Document type Clinical Trial, Phase II ; Journal Article
    ISSN 2214-3602
    ISSN (online) 2214-3602
    DOI 10.3233/JND-221656
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Parental perspectives of episodic irritability in an ultra-rare genetic disorder associated with NACC1.

    Schoch, Kelly / McConkie-Rosell, Allyn / Walley, Nicole / Bhambhani, Vikas / Feyma, Timothy / Pizoli, Carolyn E / Smith, Edward C / Tan, Queenie K-G / Shashi, Vandana

    Orphanet journal of rare diseases

    2023  Volume 18, Issue 1, Page(s) 269

    Abstract: Background: A recurrent de novo variant (c.892C>T) in NACC1 causes a neurodevelopmental disorder with epilepsy, cataracts, feeding difficulties, and delayed brain myelination (NECFM). An unusual and consistently reported feature is episodic extreme ... ...

    Abstract Background: A recurrent de novo variant (c.892C>T) in NACC1 causes a neurodevelopmental disorder with epilepsy, cataracts, feeding difficulties, and delayed brain myelination (NECFM). An unusual and consistently reported feature is episodic extreme irritability and inconsolability. We now characterize these episodes, their impact on the family, and ascertain treatments that may be effective. Parents of 14 affected individuals provided narratives describing the irritability episodes, including triggers, behavioral and physiological changes, and treatments. Simultaneously, parents of 15 children completed the Non-communicating Children's Pain Checklist-Revised (NCCPC-R), a measure to assess pain in non-verbal children.
    Results: The episodes of extreme irritability include a prodromal, peak, and resolving phase, with normal periods in between. The children were rated to have extreme pain-related behaviors on the NCCPC-R scale, although it is unknown whether the physiologic changes described by parents are caused by pain. Attempted treatments included various classes of medications, with psychotropic and sedative medications being most effective (7/15). Nearly all families (13/14) describe how the episodes have a profound impact on their lives.
    Conclusions: NECFM caused by the recurrent variant c.892C>T is associated with a universal feature of incapacitating episodic irritability of unclear etiology. Further understanding of the pathophysiology can lead to more effective therapeutic strategies.
    MeSH term(s) Child ; Humans ; Brain ; Cataract ; Hypnotics and Sedatives ; Pain/genetics ; Parents ; Rare Diseases ; Neoplasm Proteins ; Repressor Proteins
    Chemical Substances Hypnotics and Sedatives ; NACC1 protein, human ; Neoplasm Proteins ; Repressor Proteins
    Language English
    Publishing date 2023-09-04
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 2225857-7
    ISSN 1750-1172 ; 1750-1172
    ISSN (online) 1750-1172
    ISSN 1750-1172
    DOI 10.1186/s13023-023-02891-3
    Database MEDical Literature Analysis and Retrieval System OnLINE

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