Glyceroltrioleate/glyceroltrierucate therapy in 16 patients with X-chromosomal adrenoleukodystrophy/adrenomyeloneuropathy: Effect on clinical, biochemical and neurophysiological parameters View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

1995-01

AUTHORS

G. C. Korenke, D. H. Hunneman, J. Konler, S. Stöckler, K. Landmark, F. Hanefeld

ABSTRACT

We have investigated the effect of glyceroltrioleate/glyceroltrierucate (GTO/GTE) therapy on X-chromosomal adrenoleukodystrophy in 16 patients with adrenoleukodystrophy (n = 6), adrenomyeloneuropathy (n = 3), Addison disease without neurological involvement (n = 2), and neurologically and endocrinologically asymptomatic patients (n = 5). Therapy was carried out for 19.4 +/- 10 months. All patients showed a normalization of C 26:0 plasma fatty acid concentrations. None of the seven neurologically asymptomatic patients developed neurological symptoms. Somatosensory evoked potentials of the tibialis nerve was the most sensitive electrophysiological parameter, showing a slight improvement in neurologically asymptomatic patients during therapy. In none of the patients with normal cranial MRI at start of therapy (n = 6) has MRI deterioration been observed whilst on therapy. Follow up of the neurologically asymptomatic children supports the hypothesis that GTO/GTE therapy might prevent the development of neurological symptoms. Six of the nine neurologically symptomatic patients deteriorated to varying degrees whilst on therapy. MRI alterations have worsened in all patients with clinical deterioration. CONCLUSION: GTO/GTE treatment should be initiated in all neurological asymptomatic boys before first neurological symptoms develop. To discover these patients very long-chain fatty acid determination should be performed in all family members at risk when adrenoleukodystrophy or adrenomyeloneuropathy is diagnosed. More... »

PAGES

64-70

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/bf01972976

DOI

http://dx.doi.org/10.1007/bf01972976

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1040780407

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/7895759


Indexing Status Check whether this publication has been indexed by Scopus and Web Of Science using the SN Indexing Status Tool
Incoming Citations Browse incoming citations for this publication using opencitations.net

JSON-LD is the canonical representation for SciGraph data.

TIP: You can open this SciGraph record using an external JSON-LD service: JSON-LD Playground Google SDTT

[
  {
    "@context": "https://springernature.github.io/scigraph/jsonld/sgcontext.json", 
    "about": [
      {
        "id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/1103", 
        "inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/", 
        "name": "Clinical Sciences", 
        "type": "DefinedTerm"
      }, 
      {
        "id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/11", 
        "inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/", 
        "name": "Medical and Health Sciences", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Adolescent", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Adrenal Cortex Function Tests", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Adrenoleukodystrophy", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Adult", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Age of Onset", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Child", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Child, Preschool", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Demyelinating Diseases", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Erucic Acids", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Evoked Potentials, Somatosensory", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Fatty Acids", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Humans", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Leukopenia", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Magnetic Resonance Imaging", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Male", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Middle Aged", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Neural Conduction", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Spinal Nerves", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Thrombocytopenia", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Triglycerides", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "Triolein", 
        "type": "DefinedTerm"
      }, 
      {
        "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
        "name": "X Chromosome", 
        "type": "DefinedTerm"
      }
    ], 
    "author": [
      {
        "affiliation": {
          "alternateName": "University of G\u00f6ttingen", 
          "id": "https://www.grid.ac/institutes/grid.7450.6", 
          "name": [
            "Department of Paediatrics and Neuropaediatrics, University of G\u00f6ttingen, Robert-Koch-Allee 40, D-37075, G\u00f6ttingen, Germany"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Korenke", 
        "givenName": "G. C.", 
        "id": "sg:person.0746542075.21", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0746542075.21"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "University of G\u00f6ttingen", 
          "id": "https://www.grid.ac/institutes/grid.7450.6", 
          "name": [
            "Department of Paediatrics and Neuropaediatrics, University of G\u00f6ttingen, Robert-Koch-Allee 40, D-37075, G\u00f6ttingen, Germany"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Hunneman", 
        "givenName": "D. H.", 
        "id": "sg:person.011302621203.40", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.011302621203.40"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "University of G\u00f6ttingen", 
          "id": "https://www.grid.ac/institutes/grid.7450.6", 
          "name": [
            "Department of Paediatrics and Neuropaediatrics, University of G\u00f6ttingen, Robert-Koch-Allee 40, D-37075, G\u00f6ttingen, Germany"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Konler", 
        "givenName": "J.", 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "University of G\u00f6ttingen", 
          "id": "https://www.grid.ac/institutes/grid.7450.6", 
          "name": [
            "Department of Paediatrics and Neuropaediatrics, University of G\u00f6ttingen, Robert-Koch-Allee 40, D-37075, G\u00f6ttingen, Germany"
          ], 
          "type": "Organization"
        }, 
        "familyName": "St\u00f6ckler", 
        "givenName": "S.", 
        "id": "sg:person.0613301056.33", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0613301056.33"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "University of G\u00f6ttingen", 
          "id": "https://www.grid.ac/institutes/grid.7450.6", 
          "name": [
            "Department of Paediatrics and Neuropaediatrics, University of G\u00f6ttingen, Robert-Koch-Allee 40, D-37075, G\u00f6ttingen, Germany"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Landmark", 
        "givenName": "K.", 
        "id": "sg:person.01260630624.00", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01260630624.00"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "University of G\u00f6ttingen", 
          "id": "https://www.grid.ac/institutes/grid.7450.6", 
          "name": [
            "Department of Paediatrics and Neuropaediatrics, University of G\u00f6ttingen, Robert-Koch-Allee 40, D-37075, G\u00f6ttingen, Germany"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Hanefeld", 
        "givenName": "F.", 
        "id": "sg:person.01007645054.53", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01007645054.53"
        ], 
        "type": "Person"
      }
    ], 
    "citation": [
      {
        "id": "https://doi.org/10.1212/wnl.31.10.1241", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1002003042"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1073/pnas.85.20.7647", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1006012275"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1007/bf01799621", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1008516324", 
          "https://doi.org/10.1007/bf01799621"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1007/bf01799621", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1008516324"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1159/000112173", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1011818697"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/0140-6736(93)90295-r", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1013608927"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/0140-6736(93)90295-r", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1013608927"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/0140-6736(93)90984-o", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1016856165"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/0140-6736(93)90984-o", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1016856165"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1056/nejm199006283222607", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1018500669"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1002/ana.410210305", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1020568009"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/s0387-7604(12)80350-0", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1021591919"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1002/ana.410210304", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1029388341"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/0885-4505(91)90010-i", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1030140682"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1007/bf01804228", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1032182181", 
          "https://doi.org/10.1007/bf01804228"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1007/bf01804228", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1032182181"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1002/ana.410340212", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1038072030"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1111/j.1471-4159.1983.tb04748.x", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1043699975"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1056/nejm199309093291101", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1044929584"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1097/00005072-199211000-00007", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1048837194"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1038/361726a0", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1051671006", 
          "https://doi.org/10.1038/361726a0"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1212/wnl.36.3.357", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1064366454"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1212/wnl.39.11.1415", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1064368163"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://app.dimensions.ai/details/publication/pub.1082161346", 
        "type": "CreativeWork"
      }
    ], 
    "datePublished": "1995-01", 
    "datePublishedReg": "1995-01-01", 
    "description": "We have investigated the effect of glyceroltrioleate/glyceroltrierucate (GTO/GTE) therapy on X-chromosomal adrenoleukodystrophy in 16 patients with adrenoleukodystrophy (n = 6), adrenomyeloneuropathy (n = 3), Addison disease without neurological involvement (n = 2), and neurologically and endocrinologically asymptomatic patients (n = 5). Therapy was carried out for 19.4 +/- 10 months. All patients showed a normalization of C 26:0 plasma fatty acid concentrations. None of the seven neurologically asymptomatic patients developed neurological symptoms. Somatosensory evoked potentials of the tibialis nerve was the most sensitive electrophysiological parameter, showing a slight improvement in neurologically asymptomatic patients during therapy. In none of the patients with normal cranial MRI at start of therapy (n = 6) has MRI deterioration been observed whilst on therapy. Follow up of the neurologically asymptomatic children supports the hypothesis that GTO/GTE therapy might prevent the development of neurological symptoms. Six of the nine neurologically symptomatic patients deteriorated to varying degrees whilst on therapy. MRI alterations have worsened in all patients with clinical deterioration.\nCONCLUSION: GTO/GTE treatment should be initiated in all neurological asymptomatic boys before first neurological symptoms develop. To discover these patients very long-chain fatty acid determination should be performed in all family members at risk when adrenoleukodystrophy or adrenomyeloneuropathy is diagnosed.", 
    "genre": "research_article", 
    "id": "sg:pub.10.1007/bf01972976", 
    "inLanguage": [
      "en"
    ], 
    "isAccessibleForFree": false, 
    "isPartOf": [
      {
        "id": "sg:journal.1017354", 
        "issn": [
          "0340-6199", 
          "1432-1076"
        ], 
        "name": "European Journal of Pediatrics", 
        "type": "Periodical"
      }, 
      {
        "issueNumber": "1", 
        "type": "PublicationIssue"
      }, 
      {
        "type": "PublicationVolume", 
        "volumeNumber": "154"
      }
    ], 
    "name": "Glyceroltrioleate/glyceroltrierucate therapy in 16 patients with X-chromosomal adrenoleukodystrophy/adrenomyeloneuropathy: Effect on clinical, biochemical and neurophysiological parameters", 
    "pagination": "64-70", 
    "productId": [
      {
        "name": "readcube_id", 
        "type": "PropertyValue", 
        "value": [
          "c242e1244bc2632bf262df776e0c529f2019c302243d8c2b4a5deb00bc9d1fba"
        ]
      }, 
      {
        "name": "pubmed_id", 
        "type": "PropertyValue", 
        "value": [
          "7895759"
        ]
      }, 
      {
        "name": "nlm_unique_id", 
        "type": "PropertyValue", 
        "value": [
          "7603873"
        ]
      }, 
      {
        "name": "doi", 
        "type": "PropertyValue", 
        "value": [
          "10.1007/bf01972976"
        ]
      }, 
      {
        "name": "dimensions_id", 
        "type": "PropertyValue", 
        "value": [
          "pub.1040780407"
        ]
      }
    ], 
    "sameAs": [
      "https://doi.org/10.1007/bf01972976", 
      "https://app.dimensions.ai/details/publication/pub.1040780407"
    ], 
    "sdDataset": "articles", 
    "sdDatePublished": "2019-04-11T11:23", 
    "sdLicense": "https://scigraph.springernature.com/explorer/license/", 
    "sdPublisher": {
      "name": "Springer Nature - SN SciGraph project", 
      "type": "Organization"
    }, 
    "sdSource": "s3://com-uberresearch-data-dimensions-target-20181106-alternative/cleanup/v134/2549eaecd7973599484d7c17b260dba0a4ecb94b/merge/v9/a6c9fde33151104705d4d7ff012ea9563521a3ce/jats-lookup/v90/0000000355_0000000355/records_53001_00000000.jsonl", 
    "type": "ScholarlyArticle", 
    "url": "http://link.springer.com/10.1007/BF01972976"
  }
]
 

Download the RDF metadata as:  json-ld nt turtle xml License info

HOW TO GET THIS DATA PROGRAMMATICALLY:

JSON-LD is a popular format for linked data which is fully compatible with JSON.

curl -H 'Accept: application/ld+json' 'https://scigraph.springernature.com/pub.10.1007/bf01972976'

N-Triples is a line-based linked data format ideal for batch operations.

curl -H 'Accept: application/n-triples' 'https://scigraph.springernature.com/pub.10.1007/bf01972976'

Turtle is a human-readable linked data format.

curl -H 'Accept: text/turtle' 'https://scigraph.springernature.com/pub.10.1007/bf01972976'

RDF/XML is a standard XML format for linked data.

curl -H 'Accept: application/rdf+xml' 'https://scigraph.springernature.com/pub.10.1007/bf01972976'


 

This table displays all metadata directly associated to this object as RDF triples.

259 TRIPLES      21 PREDICATES      73 URIs      43 LITERALS      31 BLANK NODES

Subject Predicate Object
1 sg:pub.10.1007/bf01972976 schema:about N066e1e3d4cef405e942ac417633b1b82
2 N1a0618e81cdc4ffea7a9254f8b0a2e1e
3 N20043934e3654c9593c9a363606e3250
4 N30806f7ddd394e30bf8c8541cf4c8b98
5 N414d213ad0f5443a9d82ca2162eb0d06
6 N424018a171134e1cb5470073efd7f3bf
7 N4b3f28d1f3914fe79c85d01f2b6b7f67
8 N6a86be70976e484ab17c1ed74511aa3f
9 N6f551396855042eea1381faeb17184ac
10 N746d85a05bec4965b5d9165700ecbaeb
11 N84cf174e9fa54d85906c92babcd99a4e
12 N8c9a40ba35474e888cff52f88410d5a9
13 N9648c3611f094bf8939119a0a3e3a7ac
14 Na4df012e64f44f8aa55d4d12a4e475d3
15 Na4e1f991cea44b2183cc2bfbd54a28e9
16 Na5094c205f7342aab03228fe918c9137
17 Nb6305c2e3b374c0096557561d921bcfd
18 Nd1c9ad9a977b4e8eb2bcef642d39e98a
19 Ne740f47d8feb424c93baf87ad0c04e00
20 Nea740180d7dc4ebdaebf4ab802f9d2b6
21 Nf216452ab9ba4388bdaefc9e964efd9b
22 Nff9e218c81b54f2ca037ec8f6218e01a
23 anzsrc-for:11
24 anzsrc-for:1103
25 schema:author Nf00e862b162a4a52bf5e10c33ae4551f
26 schema:citation sg:pub.10.1007/bf01799621
27 sg:pub.10.1007/bf01804228
28 sg:pub.10.1038/361726a0
29 https://app.dimensions.ai/details/publication/pub.1082161346
30 https://doi.org/10.1002/ana.410210304
31 https://doi.org/10.1002/ana.410210305
32 https://doi.org/10.1002/ana.410340212
33 https://doi.org/10.1007/bf01799621
34 https://doi.org/10.1007/bf01804228
35 https://doi.org/10.1016/0140-6736(93)90295-r
36 https://doi.org/10.1016/0140-6736(93)90984-o
37 https://doi.org/10.1016/0885-4505(91)90010-i
38 https://doi.org/10.1016/s0387-7604(12)80350-0
39 https://doi.org/10.1056/nejm199006283222607
40 https://doi.org/10.1056/nejm199309093291101
41 https://doi.org/10.1073/pnas.85.20.7647
42 https://doi.org/10.1097/00005072-199211000-00007
43 https://doi.org/10.1111/j.1471-4159.1983.tb04748.x
44 https://doi.org/10.1159/000112173
45 https://doi.org/10.1212/wnl.31.10.1241
46 https://doi.org/10.1212/wnl.36.3.357
47 https://doi.org/10.1212/wnl.39.11.1415
48 schema:datePublished 1995-01
49 schema:datePublishedReg 1995-01-01
50 schema:description We have investigated the effect of glyceroltrioleate/glyceroltrierucate (GTO/GTE) therapy on X-chromosomal adrenoleukodystrophy in 16 patients with adrenoleukodystrophy (n = 6), adrenomyeloneuropathy (n = 3), Addison disease without neurological involvement (n = 2), and neurologically and endocrinologically asymptomatic patients (n = 5). Therapy was carried out for 19.4 +/- 10 months. All patients showed a normalization of C 26:0 plasma fatty acid concentrations. None of the seven neurologically asymptomatic patients developed neurological symptoms. Somatosensory evoked potentials of the tibialis nerve was the most sensitive electrophysiological parameter, showing a slight improvement in neurologically asymptomatic patients during therapy. In none of the patients with normal cranial MRI at start of therapy (n = 6) has MRI deterioration been observed whilst on therapy. Follow up of the neurologically asymptomatic children supports the hypothesis that GTO/GTE therapy might prevent the development of neurological symptoms. Six of the nine neurologically symptomatic patients deteriorated to varying degrees whilst on therapy. MRI alterations have worsened in all patients with clinical deterioration. CONCLUSION: GTO/GTE treatment should be initiated in all neurological asymptomatic boys before first neurological symptoms develop. To discover these patients very long-chain fatty acid determination should be performed in all family members at risk when adrenoleukodystrophy or adrenomyeloneuropathy is diagnosed.
51 schema:genre research_article
52 schema:inLanguage en
53 schema:isAccessibleForFree false
54 schema:isPartOf N40ede96cbe0d44afaf668826b942a42a
55 N41386440180540ba8a3063d7a246731c
56 sg:journal.1017354
57 schema:name Glyceroltrioleate/glyceroltrierucate therapy in 16 patients with X-chromosomal adrenoleukodystrophy/adrenomyeloneuropathy: Effect on clinical, biochemical and neurophysiological parameters
58 schema:pagination 64-70
59 schema:productId N101090c0c7864ee58cc30b9a75732427
60 N5cabf2884b4e421ea16b18aaf1b158cc
61 N6689b3d781dd4b60891165af8725d59c
62 Naad115a4ff424aaf88ee24b99a7df89e
63 Nc6e764bef3be4cb493afe34d8e443463
64 schema:sameAs https://app.dimensions.ai/details/publication/pub.1040780407
65 https://doi.org/10.1007/bf01972976
66 schema:sdDatePublished 2019-04-11T11:23
67 schema:sdLicense https://scigraph.springernature.com/explorer/license/
68 schema:sdPublisher N981e5dbe6d5c46b684908d0928ab6f6d
69 schema:url http://link.springer.com/10.1007/BF01972976
70 sgo:license sg:explorer/license/
71 sgo:sdDataset articles
72 rdf:type schema:ScholarlyArticle
73 N05eeea8790304af1b9a21a777c5ab28a rdf:first sg:person.01260630624.00
74 rdf:rest Ne6b95db2fcc14dac8898495b8a6f37bb
75 N066e1e3d4cef405e942ac417633b1b82 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
76 schema:name Adrenal Cortex Function Tests
77 rdf:type schema:DefinedTerm
78 N101090c0c7864ee58cc30b9a75732427 schema:name readcube_id
79 schema:value c242e1244bc2632bf262df776e0c529f2019c302243d8c2b4a5deb00bc9d1fba
80 rdf:type schema:PropertyValue
81 N1a0618e81cdc4ffea7a9254f8b0a2e1e schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
82 schema:name Spinal Nerves
83 rdf:type schema:DefinedTerm
84 N20043934e3654c9593c9a363606e3250 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
85 schema:name X Chromosome
86 rdf:type schema:DefinedTerm
87 N30806f7ddd394e30bf8c8541cf4c8b98 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
88 schema:name Fatty Acids
89 rdf:type schema:DefinedTerm
90 N4067498abbd642b9a6c28d77afbbccdd schema:affiliation https://www.grid.ac/institutes/grid.7450.6
91 schema:familyName Konler
92 schema:givenName J.
93 rdf:type schema:Person
94 N40ede96cbe0d44afaf668826b942a42a schema:volumeNumber 154
95 rdf:type schema:PublicationVolume
96 N41386440180540ba8a3063d7a246731c schema:issueNumber 1
97 rdf:type schema:PublicationIssue
98 N414d213ad0f5443a9d82ca2162eb0d06 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
99 schema:name Adolescent
100 rdf:type schema:DefinedTerm
101 N424018a171134e1cb5470073efd7f3bf schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
102 schema:name Triglycerides
103 rdf:type schema:DefinedTerm
104 N4a157bbe36bb4e65993e6e08ff11c916 rdf:first N4067498abbd642b9a6c28d77afbbccdd
105 rdf:rest Nf5bd907a90be48d08589c8c3dce22f07
106 N4b3f28d1f3914fe79c85d01f2b6b7f67 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
107 schema:name Humans
108 rdf:type schema:DefinedTerm
109 N5cabf2884b4e421ea16b18aaf1b158cc schema:name doi
110 schema:value 10.1007/bf01972976
111 rdf:type schema:PropertyValue
112 N6689b3d781dd4b60891165af8725d59c schema:name pubmed_id
113 schema:value 7895759
114 rdf:type schema:PropertyValue
115 N6a86be70976e484ab17c1ed74511aa3f schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
116 schema:name Demyelinating Diseases
117 rdf:type schema:DefinedTerm
118 N6f551396855042eea1381faeb17184ac schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
119 schema:name Evoked Potentials, Somatosensory
120 rdf:type schema:DefinedTerm
121 N746d85a05bec4965b5d9165700ecbaeb schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
122 schema:name Adrenoleukodystrophy
123 rdf:type schema:DefinedTerm
124 N84cf174e9fa54d85906c92babcd99a4e schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
125 schema:name Neural Conduction
126 rdf:type schema:DefinedTerm
127 N8c9a40ba35474e888cff52f88410d5a9 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
128 schema:name Leukopenia
129 rdf:type schema:DefinedTerm
130 N9648c3611f094bf8939119a0a3e3a7ac schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
131 schema:name Male
132 rdf:type schema:DefinedTerm
133 N981e5dbe6d5c46b684908d0928ab6f6d schema:name Springer Nature - SN SciGraph project
134 rdf:type schema:Organization
135 Na4df012e64f44f8aa55d4d12a4e475d3 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
136 schema:name Triolein
137 rdf:type schema:DefinedTerm
138 Na4e1f991cea44b2183cc2bfbd54a28e9 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
139 schema:name Thrombocytopenia
140 rdf:type schema:DefinedTerm
141 Na5094c205f7342aab03228fe918c9137 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
142 schema:name Child
143 rdf:type schema:DefinedTerm
144 Naad115a4ff424aaf88ee24b99a7df89e schema:name nlm_unique_id
145 schema:value 7603873
146 rdf:type schema:PropertyValue
147 Nb4648498ca6945208f7d95c84204797d rdf:first sg:person.011302621203.40
148 rdf:rest N4a157bbe36bb4e65993e6e08ff11c916
149 Nb6305c2e3b374c0096557561d921bcfd schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
150 schema:name Erucic Acids
151 rdf:type schema:DefinedTerm
152 Nc6e764bef3be4cb493afe34d8e443463 schema:name dimensions_id
153 schema:value pub.1040780407
154 rdf:type schema:PropertyValue
155 Nd1c9ad9a977b4e8eb2bcef642d39e98a schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
156 schema:name Magnetic Resonance Imaging
157 rdf:type schema:DefinedTerm
158 Ne6b95db2fcc14dac8898495b8a6f37bb rdf:first sg:person.01007645054.53
159 rdf:rest rdf:nil
160 Ne740f47d8feb424c93baf87ad0c04e00 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
161 schema:name Age of Onset
162 rdf:type schema:DefinedTerm
163 Nea740180d7dc4ebdaebf4ab802f9d2b6 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
164 schema:name Middle Aged
165 rdf:type schema:DefinedTerm
166 Nf00e862b162a4a52bf5e10c33ae4551f rdf:first sg:person.0746542075.21
167 rdf:rest Nb4648498ca6945208f7d95c84204797d
168 Nf216452ab9ba4388bdaefc9e964efd9b schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
169 schema:name Adult
170 rdf:type schema:DefinedTerm
171 Nf5bd907a90be48d08589c8c3dce22f07 rdf:first sg:person.0613301056.33
172 rdf:rest N05eeea8790304af1b9a21a777c5ab28a
173 Nff9e218c81b54f2ca037ec8f6218e01a schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
174 schema:name Child, Preschool
175 rdf:type schema:DefinedTerm
176 anzsrc-for:11 schema:inDefinedTermSet anzsrc-for:
177 schema:name Medical and Health Sciences
178 rdf:type schema:DefinedTerm
179 anzsrc-for:1103 schema:inDefinedTermSet anzsrc-for:
180 schema:name Clinical Sciences
181 rdf:type schema:DefinedTerm
182 sg:journal.1017354 schema:issn 0340-6199
183 1432-1076
184 schema:name European Journal of Pediatrics
185 rdf:type schema:Periodical
186 sg:person.01007645054.53 schema:affiliation https://www.grid.ac/institutes/grid.7450.6
187 schema:familyName Hanefeld
188 schema:givenName F.
189 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01007645054.53
190 rdf:type schema:Person
191 sg:person.011302621203.40 schema:affiliation https://www.grid.ac/institutes/grid.7450.6
192 schema:familyName Hunneman
193 schema:givenName D. H.
194 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.011302621203.40
195 rdf:type schema:Person
196 sg:person.01260630624.00 schema:affiliation https://www.grid.ac/institutes/grid.7450.6
197 schema:familyName Landmark
198 schema:givenName K.
199 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01260630624.00
200 rdf:type schema:Person
201 sg:person.0613301056.33 schema:affiliation https://www.grid.ac/institutes/grid.7450.6
202 schema:familyName Stöckler
203 schema:givenName S.
204 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0613301056.33
205 rdf:type schema:Person
206 sg:person.0746542075.21 schema:affiliation https://www.grid.ac/institutes/grid.7450.6
207 schema:familyName Korenke
208 schema:givenName G. C.
209 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0746542075.21
210 rdf:type schema:Person
211 sg:pub.10.1007/bf01799621 schema:sameAs https://app.dimensions.ai/details/publication/pub.1008516324
212 https://doi.org/10.1007/bf01799621
213 rdf:type schema:CreativeWork
214 sg:pub.10.1007/bf01804228 schema:sameAs https://app.dimensions.ai/details/publication/pub.1032182181
215 https://doi.org/10.1007/bf01804228
216 rdf:type schema:CreativeWork
217 sg:pub.10.1038/361726a0 schema:sameAs https://app.dimensions.ai/details/publication/pub.1051671006
218 https://doi.org/10.1038/361726a0
219 rdf:type schema:CreativeWork
220 https://app.dimensions.ai/details/publication/pub.1082161346 schema:CreativeWork
221 https://doi.org/10.1002/ana.410210304 schema:sameAs https://app.dimensions.ai/details/publication/pub.1029388341
222 rdf:type schema:CreativeWork
223 https://doi.org/10.1002/ana.410210305 schema:sameAs https://app.dimensions.ai/details/publication/pub.1020568009
224 rdf:type schema:CreativeWork
225 https://doi.org/10.1002/ana.410340212 schema:sameAs https://app.dimensions.ai/details/publication/pub.1038072030
226 rdf:type schema:CreativeWork
227 https://doi.org/10.1007/bf01799621 schema:sameAs https://app.dimensions.ai/details/publication/pub.1008516324
228 rdf:type schema:CreativeWork
229 https://doi.org/10.1007/bf01804228 schema:sameAs https://app.dimensions.ai/details/publication/pub.1032182181
230 rdf:type schema:CreativeWork
231 https://doi.org/10.1016/0140-6736(93)90295-r schema:sameAs https://app.dimensions.ai/details/publication/pub.1013608927
232 rdf:type schema:CreativeWork
233 https://doi.org/10.1016/0140-6736(93)90984-o schema:sameAs https://app.dimensions.ai/details/publication/pub.1016856165
234 rdf:type schema:CreativeWork
235 https://doi.org/10.1016/0885-4505(91)90010-i schema:sameAs https://app.dimensions.ai/details/publication/pub.1030140682
236 rdf:type schema:CreativeWork
237 https://doi.org/10.1016/s0387-7604(12)80350-0 schema:sameAs https://app.dimensions.ai/details/publication/pub.1021591919
238 rdf:type schema:CreativeWork
239 https://doi.org/10.1056/nejm199006283222607 schema:sameAs https://app.dimensions.ai/details/publication/pub.1018500669
240 rdf:type schema:CreativeWork
241 https://doi.org/10.1056/nejm199309093291101 schema:sameAs https://app.dimensions.ai/details/publication/pub.1044929584
242 rdf:type schema:CreativeWork
243 https://doi.org/10.1073/pnas.85.20.7647 schema:sameAs https://app.dimensions.ai/details/publication/pub.1006012275
244 rdf:type schema:CreativeWork
245 https://doi.org/10.1097/00005072-199211000-00007 schema:sameAs https://app.dimensions.ai/details/publication/pub.1048837194
246 rdf:type schema:CreativeWork
247 https://doi.org/10.1111/j.1471-4159.1983.tb04748.x schema:sameAs https://app.dimensions.ai/details/publication/pub.1043699975
248 rdf:type schema:CreativeWork
249 https://doi.org/10.1159/000112173 schema:sameAs https://app.dimensions.ai/details/publication/pub.1011818697
250 rdf:type schema:CreativeWork
251 https://doi.org/10.1212/wnl.31.10.1241 schema:sameAs https://app.dimensions.ai/details/publication/pub.1002003042
252 rdf:type schema:CreativeWork
253 https://doi.org/10.1212/wnl.36.3.357 schema:sameAs https://app.dimensions.ai/details/publication/pub.1064366454
254 rdf:type schema:CreativeWork
255 https://doi.org/10.1212/wnl.39.11.1415 schema:sameAs https://app.dimensions.ai/details/publication/pub.1064368163
256 rdf:type schema:CreativeWork
257 https://www.grid.ac/institutes/grid.7450.6 schema:alternateName University of Göttingen
258 schema:name Department of Paediatrics and Neuropaediatrics, University of Göttingen, Robert-Koch-Allee 40, D-37075, Göttingen, Germany
259 rdf:type schema:Organization
 




Preview window. Press ESC to close (or click here)


...