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  1. Book ; Conference proceedings: Brain metabolism revisited - concepts and treatments

    Klepper, Jörg

    metabolic epileptic encephalopathies

    (Symposia proceedings)

    2004  

    Event/congress International Symposium Focus on Neuropediatrics (1, 2004, Fulda)
    Author's details J. Klepper (ed.)
    Series title Symposia proceedings
    Keywords Brain Diseases, Metabolic / metabolism ; Epilepsy / metabolism ; Brain / metabolism ; Brain Diseases, Metabolic / therapy ; Epilepsy / therapy ; Infant ; Kind ; Hirnfunktionsstörung ; Neurophysiologie ; Epilepsie ; Ketogene Kost ; Stoffwechselkrankheit
    Subject Ketogene Diät ; Ketogene Ernährung ; Stoffwechsel ; Stoffwechselerkrankung ; Kindheit ; Kindesalter ; Kindschaft ; Kinder ; Fallsucht ; Nervenphysiologie ; Nervensystem ; Cerebrale Insuffizienz ; Cerebrale Dysfunktion ; Hirninsuffizienz ; Hirnleistungsstörung ; Zerebrale Insuffizienz
    Language English
    Size 133 S. : Ill., graph. Darst.
    Edition 1. Aufl
    Publisher SPS Publ
    Publishing place Heilbronn
    Publishing country Germany
    Document type Book ; Conference proceedings
    Note Literaturangaben
    HBZ-ID HT014495163
    ISBN 3-936145-18-0 ; 978-3-936145-18-2
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    2005 A 3922 order for loan Magazin

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  2. Article ; Online: Diagnosis and treatment of growth hormone deficiency in children on the ketogenic diet: A case series.

    Groveman, Sue / Klepper, Joerg / Liesenkötter, Klaus-Peter / Grimberg, Adda / Bergqvist, A G Christina

    Epilepsia open

    2024  

    Abstract: The ketogenic diet (KD) can have a negative impact on the linear growth and body composition of children. The aims of this study were to review two centers' experience with children who developed height deceleration on the KD and determine if the height ... ...

    Abstract The ketogenic diet (KD) can have a negative impact on the linear growth and body composition of children. The aims of this study were to review two centers' experience with children who developed height deceleration on the KD and determine if the height deceleration was secondary to growth hormone deficiency (GHD), and if growth hormone therapy (GHT) would be effective and safe (not altering ketosis or seizure frequency). Retrospective chart reviews were performed on patients with KD referred to Endocrinology between 2013 and 2018. Seventeen children were identified. Data reviewed included: demographics, growth velocity, KD ratio, protein/calorie intake, lab results, GH dosage, Tanner stage, and seizure frequency, and endocrine recommendations. Descriptive statistics were performed. Of the 17 children referred to the Endocrine Division, seven children were growth hormone deficient and began GHT. Data were provided for six patients (2 males, 4 females; age 2-7 years at the start of KD) on the KD for >6 years and on GHT for >4 years. Growth for all patients stabilized or increased. IGF-1 z-scores normalized. GHT did not affect seizure frequency or ketosis. GHT in those with GHD can be an appropriate option allowing better growth while still maintaining ketogenic therapy and seizure control. PLAIN LANGUAGE SUMMARY: The KD can be an effective treatment for difficult-to-control epilepsy and some disorders of carbohydrate metabolism. The KD can adversely affect the linear growth (height) of children. This case series reviewed six patients who had slow linear growth. It was found that all six children had growth hormone deficiency, grew better with growth hormone treatments, and that their seizures and ketone levels were not affected.
    Language English
    Publishing date 2024-04-20
    Publishing country United States
    Document type Journal Article
    ISSN 2470-9239
    ISSN (online) 2470-9239
    DOI 10.1002/epi4.12942
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: GLUT1 deficiency syndrome and ketogenic diet therapies: missing rare but treatable diseases?

    Klepper, Joerg

    Developmental medicine and child neurology

    2015  Volume 57, Issue 10, Page(s) 896–897

    MeSH term(s) Carbohydrate Metabolism, Inborn Errors/diet therapy ; Carbohydrate Metabolism, Inborn Errors/genetics ; Diet, Ketogenic ; Epilepsy/diet therapy ; Epilepsy/genetics ; Female ; Glucose Transporter Type 1/genetics ; Humans ; Male ; Monosaccharide Transport Proteins/deficiency
    Chemical Substances Glucose Transporter Type 1 ; Monosaccharide Transport Proteins
    Language English
    Publishing date 2015-10
    Publishing country England
    Document type Journal Article ; Comment
    ZDB-ID 80369-8
    ISSN 1469-8749 ; 0012-1622
    ISSN (online) 1469-8749
    ISSN 0012-1622
    DOI 10.1111/dmcn.12807
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.B 5: Show issues Location:
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  4. Article ; Conference proceedings: FV 236. Twenty-five Years Glut1 Deficiency in Neuropediatrics—The Importance for Adult Neurology

    Klepper, Jörg

    Neuropediatrics

    2018  Volume 49, Issue S 02

    Event/congress Abstracts of the 44th Annual Meeting of the Society for Neuropediatrics, Berlin, 2018-10-30
    Language English
    Publishing date 2018-10-01
    Publisher Georg Thieme Verlag KG
    Publishing place Stuttgart ; New York
    Document type Article ; Conference proceedings
    ZDB-ID 573291-8
    ISSN 1439-1899 ; 0174-304X
    ISSN (online) 1439-1899
    ISSN 0174-304X
    DOI 10.1055/s-0038-1675939
    Database Thieme publisher's database

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 728: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  5. Article ; Online: GLUT-1 deficiency syndrome in Norway--yet another piece of the puzzle.

    Klepper, Joerg

    Developmental medicine and child neurology

    2013  Volume 55, Issue 5, Page(s) 400–401

    MeSH term(s) Carbohydrate Metabolism, Inborn Errors/diet therapy ; Diet, Carbohydrate-Restricted/methods ; Diet, Ketogenic/methods ; Female ; Humans ; Male ; Monosaccharide Transport Proteins/deficiency
    Chemical Substances Monosaccharide Transport Proteins
    Language English
    Publishing date 2013-05
    Publishing country England
    Document type Journal Article ; Comment
    ZDB-ID 80369-8
    ISSN 1469-8749 ; 0012-1622
    ISSN (online) 1469-8749
    ISSN 0012-1622
    DOI 10.1111/dmcn.12104
    Database MEDical Literature Analysis and Retrieval System OnLINE

    Full text online

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    In stock of ZB MED Cologne/Königswinter

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  6. Article ; Online: Absence of SLC2A1 mutations does not exclude Glut1 deficiency syndrome.

    Klepper, Joerg

    Neuropediatrics

    2013  Volume 44, Issue 4, Page(s) 235–236

    Abstract: Increasingly, the absence of SLC2A1 mutations causes pediatricians to abandon the diagnosis of Glut1 deficiency. For several reasons this is not justified. Potential disease mechanisms in SLC2A1-negative Glut1 deficiency are discussed. ...

    Abstract Increasingly, the absence of SLC2A1 mutations causes pediatricians to abandon the diagnosis of Glut1 deficiency. For several reasons this is not justified. Potential disease mechanisms in SLC2A1-negative Glut1 deficiency are discussed.
    MeSH term(s) Carbohydrate Metabolism, Inborn Errors/genetics ; Genetic Predisposition to Disease/genetics ; Glucose Transporter Type 1/genetics ; Humans ; Monosaccharide Transport Proteins/deficiency ; Monosaccharide Transport Proteins/genetics ; Mutation/genetics
    Chemical Substances Glucose Transporter Type 1 ; Monosaccharide Transport Proteins ; SLC2A1 protein, human
    Language English
    Publishing date 2013-08
    Publishing country Germany
    Document type Letter
    ZDB-ID 573291-8
    ISSN 1439-1899 ; 0174-304X
    ISSN (online) 1439-1899
    ISSN 0174-304X
    DOI 10.1055/s-0033-1336015
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 728: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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    ab Jg. 2022: Lesesaal (EG)

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  7. Article: Glucide metabolism disorders (excluding glycogen myopathies).

    Klepper, Joerg

    Handbook of clinical neurology

    2013  Volume 113, Page(s) 1689–1694

    Abstract: Glucide metabolism comprises pathways for transport, intermediate metabolism, utilization, and storage of carbohydrates. Defects affect multiple organs and present as systemic diseases. Neurological symptoms result from hypoglycemia, lactic acidosis, or ... ...

    Abstract Glucide metabolism comprises pathways for transport, intermediate metabolism, utilization, and storage of carbohydrates. Defects affect multiple organs and present as systemic diseases. Neurological symptoms result from hypoglycemia, lactic acidosis, or inadequate storage of complex glucide molecules in neurological tissues. In glycogen storage disorders hypoglycemia indicates hepatic involvement, weakness and muscle cramps muscle involvement. Hypoglycemia is also the leading neurological symptom in disorders of gluconeogenesis. Disorders of galactose and fructose metabolism are rare, detectable by neonatal screening, and manifest following dietary intake of these sugars. Rare defects within the pentose metabolism constitute a new area of inborn metabolic disorders and may present with neurological symptoms. Treatment of these disorders involves the avoidance of fasting, dietary treatment eliminating specific carbohydrates, and enzyme replacement therapy in individual glycogen storage diseases.GLUT1 deficiency syndrome, a specific disorder of glucose transport into brain, results in global developmental delay, early-onset epilepsy, and a complex movement disorder. Treatment with a high-fat, low-carbohydrate ketogenic diet provides ketones as an alternative fuel to the brain and restores brain energy metabolism. Recently paroxysmal exertion-induced dyskinesia and stomatin-deficient cryohydrocytosis have been identified as an allelic disorder to GLUT1 deficiency equally responding to a ketogenic diet.
    MeSH term(s) Carbohydrate Metabolism, Inborn Errors/diagnosis ; Carbohydrate Metabolism, Inborn Errors/genetics ; Carbohydrate Metabolism, Inborn Errors/metabolism ; Glucose Transporter Type 1/genetics ; Humans ; Infant, Newborn
    Chemical Substances Glucose Transporter Type 1
    Language English
    Publishing date 2013
    Publishing country Netherlands
    Document type Journal Article ; Review
    ISSN 0072-9752
    ISSN 0072-9752
    DOI 10.1016/B978-0-444-59565-2.00036-8
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Conference proceedings: Sudden Uncontrolled Laughter - Behavioral Disorder or Serious Neurological Symptom?

    Greulich, Nicola / Klepper, Joerg

    Neuropediatrics

    2019  Volume 50, Issue S 02

    Event/congress Abstracts of the 45th Annual Meeting of the Society for Neuropediatrics, München, 2019-09-11
    Language English
    Publishing date 2019-09-01
    Publisher Georg Thieme Verlag KG
    Publishing place Stuttgart ; New York
    Document type Article ; Conference proceedings
    ZDB-ID 573291-8
    ISSN 1439-1899 ; 0174-304X
    ISSN (online) 1439-1899
    ISSN 0174-304X
    DOI 10.1055/s-0039-1698160
    Database Thieme publisher's database

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    In stock of ZB MED Cologne/Königswinter

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    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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  9. Article ; Online: GLUT1 deficiency syndrome in clinical practice.

    Klepper, Joerg

    Epilepsy research

    2012  Volume 100, Issue 3, Page(s) 272–277

    Abstract: GLUT1 deficiency syndrome (GLUT1DS) is caused by impaired glucose transport into brain and is effectively treated by means of a ketogenic diet. In clinical practice the diagnosis of GLUT1DS often is challenging due to the increasing complexity of ... ...

    Abstract GLUT1 deficiency syndrome (GLUT1DS) is caused by impaired glucose transport into brain and is effectively treated by means of a ketogenic diet. In clinical practice the diagnosis of GLUT1DS often is challenging due to the increasing complexity of symptoms, diagnostic cut-offs for hypoglycorrhachia and genetic heterogeneity. In terms of treatment alternative ketogenic diets and their long-term side effects as well as novel compounds such as alpha-lipoic acid and triheptanoin have raised a variety of issues. The current diagnostic and therapeutic approach to GLUT1DS is discussed in this review in view of these recent developments.
    MeSH term(s) Animals ; Brain/metabolism ; Brain/physiopathology ; Carbohydrate Metabolism, Inborn Errors/diagnosis ; Carbohydrate Metabolism, Inborn Errors/diet therapy ; Diet, Ketogenic ; Epilepsy/diet therapy ; Glucose/metabolism ; Glucose Transporter Type 1/deficiency ; Glucose Transporter Type 1/metabolism ; Humans ; Monosaccharide Transport Proteins/deficiency
    Chemical Substances Glucose Transporter Type 1 ; Monosaccharide Transport Proteins ; Glucose (IY9XDZ35W2)
    Language English
    Publishing date 2012-07
    Publishing country Netherlands
    Document type Journal Article ; Review
    ZDB-ID 632939-1
    ISSN 1872-6844 ; 0920-1211
    ISSN (online) 1872-6844
    ISSN 0920-1211
    DOI 10.1016/j.eplepsyres.2011.02.007
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article: Timing of Ketogenic Dietary Therapy (KDT) Introduction and Its Impact on Cognitive Profiles in Children with Glut1-DS-A Preliminary Study.

    Barthold, Martina / Jurkutat, Anne / Goetz, Regina / Schubring, Lucia / Spiegler, Juliane / Fries, Ann-Sophie / Kiesel, Lucia / Klepper, Joerg

    Children (Basel, Switzerland)

    2023  Volume 10, Issue 4

    Abstract: The aim of this research was to characterize cognitive abilities in patients with Glut1-Deficiency syndrome (Glut1DS) following ketogenic diet therapy (KDT).: Methods: The cognitive profiles of eight children were assessed using the Wechsler ... ...

    Abstract The aim of this research was to characterize cognitive abilities in patients with Glut1-Deficiency syndrome (Glut1DS) following ketogenic diet therapy (KDT).
    Methods: The cognitive profiles of eight children were assessed using the Wechsler Intelligence Scale (WISC-IV). The effect of ketogenic diet therapy (KDT) on individual subareas of intelligence was analyzed considering the potential influence of speech motor impairments.
    Results: Patients with Glut1DS showed a wide range of cognitive performance levels. Some participants showed statistically and clinically significant discrepancies between individual subdomains of intelligence. Both variables, KDT initiation as well as duration, had a positive effect on the overall IQ score. Significant correlations were partially found between the time of KDT initiation and the level of IQ scores, depending on the presence of expressive language test demands of the respective subtests of the WISC-IV. Accordingly, the participants benefited les in the linguistic cognitive domain. The discrepancies in cognitive performance profiles of patients with Glut1DS can be attributed to the possibility of a negative distortion of the results due to the influence of speech motor impairments.
    Conclusions: The individual access skills of test persons should be more strongly considered in test procedures for the assessment of intelligence to reduce the negative influence of motor deficits on test performance. Specific characterization and systematization of the speech disorder are indispensable for determining the severity of speech motor impairment in Glut1DS. Therefore, a stronger focus on dysarthria during diagnosis and therapy is necessary.
    Language English
    Publishing date 2023-04-03
    Publishing country Switzerland
    Document type Case Reports
    ZDB-ID 2732685-8
    ISSN 2227-9067
    ISSN 2227-9067
    DOI 10.3390/children10040681
    Database MEDical Literature Analysis and Retrieval System OnLINE

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