Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome)
| ISSN: |
1432-0533
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|---|---|
| Keywords: |
Cerebro-hepato-renal syndrome ; Zellweger's Syndrome ; Polymicrogyria ; Dysmyelination ; Axonal dystrophy ; Metabolic abnormality
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| Source: |
Springer Online Journal Archives 1860-2000
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| Topics: |
Medicine
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| Notes: |
Summary The nervous system in patients with cerebro-hepato-renal syndrome appeared to be affected at various tissue levels. There was evidence of a migrational disorder manifested by polymicrogyria and lack of normal neuronal maturation. There was dysmyelination of the white matter associated with accumulation of neutral fat in astrocytes. Within the peripheral nerves, masses of tangled neurofilaments producing dystrophic axons were demonstrated by electron microscopy. These findings could be explained on the basis of a genetic metabolic defect, one that involved particularly the amino acids. The defect may have interfered with the normal intercellular reaction during embryogenesis resulting in the malformation of multiple organs. The same metabolic abnormality could have caused the hepatic damage and disturbance in normal myelination during the neonatal period.
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| Type of Medium: |
Electronic Resource
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| URL: |
| _version_ | 1798295396130226177 |
|---|---|
| autor | Liu, H. Mei Bangaru, Babu S. Kidd, James Boggs, Joseph |
| autorsonst | Liu, H. Mei Bangaru, Babu S. Kidd, James Boggs, Joseph |
| book_url | http://dx.doi.org/10.1007/BF00684662 |
| datenlieferant | nat_lic_papers |
| hauptsatz | hsatz_simple |
| identnr | NLM203576918 |
| issn | 1432-0533 |
| journal_name | Acta neuropathologica |
| materialart | 1 |
| notes | Summary The nervous system in patients with cerebro-hepato-renal syndrome appeared to be affected at various tissue levels. There was evidence of a migrational disorder manifested by polymicrogyria and lack of normal neuronal maturation. There was dysmyelination of the white matter associated with accumulation of neutral fat in astrocytes. Within the peripheral nerves, masses of tangled neurofilaments producing dystrophic axons were demonstrated by electron microscopy. These findings could be explained on the basis of a genetic metabolic defect, one that involved particularly the amino acids. The defect may have interfered with the normal intercellular reaction during embryogenesis resulting in the malformation of multiple organs. The same metabolic abnormality could have caused the hepatic damage and disturbance in normal myelination during the neonatal period. |
| package_name | Springer |
| publikationsjahr_anzeige | 1976 |
| publikationsjahr_facette | 1976 |
| publikationsjahr_intervall | 8024:1975-1979 |
| publikationsjahr_sort | 1976 |
| publisher | Springer |
| reference | 34 (1976), S. 115-123 |
| schlagwort | Cerebro-hepato-renal syndrome Zellweger's Syndrome Polymicrogyria Dysmyelination Axonal dystrophy Metabolic abnormality |
| search_space | articles |
| shingle_author_1 | Liu, H. Mei Bangaru, Babu S. Kidd, James Boggs, Joseph |
| shingle_author_2 | Liu, H. Mei Bangaru, Babu S. Kidd, James Boggs, Joseph |
| shingle_author_3 | Liu, H. Mei Bangaru, Babu S. Kidd, James Boggs, Joseph |
| shingle_author_4 | Liu, H. Mei Bangaru, Babu S. Kidd, James Boggs, Joseph |
| shingle_catch_all_1 | Liu, H. Mei Bangaru, Babu S. Kidd, James Boggs, Joseph Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome) Cerebro-hepato-renal syndrome Zellweger's Syndrome Polymicrogyria Dysmyelination Axonal dystrophy Metabolic abnormality Cerebro-hepato-renal syndrome Zellweger's Syndrome Polymicrogyria Dysmyelination Axonal dystrophy Metabolic abnormality Summary The nervous system in patients with cerebro-hepato-renal syndrome appeared to be affected at various tissue levels. There was evidence of a migrational disorder manifested by polymicrogyria and lack of normal neuronal maturation. There was dysmyelination of the white matter associated with accumulation of neutral fat in astrocytes. Within the peripheral nerves, masses of tangled neurofilaments producing dystrophic axons were demonstrated by electron microscopy. These findings could be explained on the basis of a genetic metabolic defect, one that involved particularly the amino acids. The defect may have interfered with the normal intercellular reaction during embryogenesis resulting in the malformation of multiple organs. The same metabolic abnormality could have caused the hepatic damage and disturbance in normal myelination during the neonatal period. 1432-0533 14320533 Springer |
| shingle_catch_all_2 | Liu, H. Mei Bangaru, Babu S. Kidd, James Boggs, Joseph Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome) Cerebro-hepato-renal syndrome Zellweger's Syndrome Polymicrogyria Dysmyelination Axonal dystrophy Metabolic abnormality Cerebro-hepato-renal syndrome Zellweger's Syndrome Polymicrogyria Dysmyelination Axonal dystrophy Metabolic abnormality Summary The nervous system in patients with cerebro-hepato-renal syndrome appeared to be affected at various tissue levels. There was evidence of a migrational disorder manifested by polymicrogyria and lack of normal neuronal maturation. There was dysmyelination of the white matter associated with accumulation of neutral fat in astrocytes. Within the peripheral nerves, masses of tangled neurofilaments producing dystrophic axons were demonstrated by electron microscopy. These findings could be explained on the basis of a genetic metabolic defect, one that involved particularly the amino acids. The defect may have interfered with the normal intercellular reaction during embryogenesis resulting in the malformation of multiple organs. The same metabolic abnormality could have caused the hepatic damage and disturbance in normal myelination during the neonatal period. 1432-0533 14320533 Springer |
| shingle_catch_all_3 | Liu, H. Mei Bangaru, Babu S. Kidd, James Boggs, Joseph Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome) Cerebro-hepato-renal syndrome Zellweger's Syndrome Polymicrogyria Dysmyelination Axonal dystrophy Metabolic abnormality Cerebro-hepato-renal syndrome Zellweger's Syndrome Polymicrogyria Dysmyelination Axonal dystrophy Metabolic abnormality Summary The nervous system in patients with cerebro-hepato-renal syndrome appeared to be affected at various tissue levels. There was evidence of a migrational disorder manifested by polymicrogyria and lack of normal neuronal maturation. There was dysmyelination of the white matter associated with accumulation of neutral fat in astrocytes. Within the peripheral nerves, masses of tangled neurofilaments producing dystrophic axons were demonstrated by electron microscopy. These findings could be explained on the basis of a genetic metabolic defect, one that involved particularly the amino acids. The defect may have interfered with the normal intercellular reaction during embryogenesis resulting in the malformation of multiple organs. The same metabolic abnormality could have caused the hepatic damage and disturbance in normal myelination during the neonatal period. 1432-0533 14320533 Springer |
| shingle_catch_all_4 | Liu, H. Mei Bangaru, Babu S. Kidd, James Boggs, Joseph Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome) Cerebro-hepato-renal syndrome Zellweger's Syndrome Polymicrogyria Dysmyelination Axonal dystrophy Metabolic abnormality Cerebro-hepato-renal syndrome Zellweger's Syndrome Polymicrogyria Dysmyelination Axonal dystrophy Metabolic abnormality Summary The nervous system in patients with cerebro-hepato-renal syndrome appeared to be affected at various tissue levels. There was evidence of a migrational disorder manifested by polymicrogyria and lack of normal neuronal maturation. There was dysmyelination of the white matter associated with accumulation of neutral fat in astrocytes. Within the peripheral nerves, masses of tangled neurofilaments producing dystrophic axons were demonstrated by electron microscopy. These findings could be explained on the basis of a genetic metabolic defect, one that involved particularly the amino acids. The defect may have interfered with the normal intercellular reaction during embryogenesis resulting in the malformation of multiple organs. The same metabolic abnormality could have caused the hepatic damage and disturbance in normal myelination during the neonatal period. 1432-0533 14320533 Springer |
| shingle_title_1 | Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome) |
| shingle_title_2 | Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome) |
| shingle_title_3 | Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome) |
| shingle_title_4 | Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome) |
| sigel_instance_filter | dkfz geomar wilbert ipn albert fhp |
| source_archive | Springer Online Journal Archives 1860-2000 |
| timestamp | 2024-05-06T09:35:32.512Z |
| titel | Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome) |
| titel_suche | Neuropathological considerations in cerebro-hepato-renal syndrome (Zellweger's syndrome) |
| topic | WW-YZ |
| uid | nat_lic_papers_NLM203576918 |