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  • 1
    ISSN: 1432-0533
    Keywords: Isoniazid neuropathy ; Axonal degeneration ; Endoneurial edema ; Axonal swelling ; Dying back
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Morphometric studies of the pathologic changes were carried out on the peripheral nerves, spinal roots, and different levels of the Goll's tract in rats given isoniazid and killed 1, 2, 3, 4, 5, 6, 7, 14, and 30 days after intoxication. In teased fiber preparations, axonal degeneration was the main change present, and this was seen as early as day 2 in the peroneal and distal sural nerves. The frequency of myelinated fibers showing axonal degeneration was higher in the distal than the proximal sural nerve, and in the ventral than the dorsal root. In the group of rats killed on 5, 6, 7, and 14 days, a significant decrease of the myelinated fiber density was observed in the distal and proximal sural nerves, ventral root, and at the third cervical level of the Goll's tract. The degree of fiber degeneration was more severe in the distal than in the proximal sural nerve and in the third cervical than the fifth thoracic levels of the Goll's tract. Preferential decrease of large myelinated fibers was noted in all the affected nerves. No definite abnormalities, however, were seen in nerve cells of the 6th lumbar spinal ganglia and anterior horn cells of the lumbar spinal cord on light microscopy. On 30 days, regeneration at varying degrees was discerned in all the affected nerves with significant increase of small myelinated fibers, particularly in the ventral root. The findings indicate that both centrally and peripherally directed myelinated axons are more affected in the distal than in the proximal segments while the neuronal cell bodies are spatio-temporal evolution of this pattern of change is compatible with the concept of the “dying back” process or centralperipheral distal axonopathy.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0738
    Keywords: Fenitrothion ; Fenitrooxon ; Acute toxicity ; Phenobarbital ; Adrenalectomy ; Diethyl maleate
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The effect of adrenalectomy (Adx), SKF 525-A, phenobarbital (PB), and diethyl maleate (DEM) on the acute toxicity of fenitrothion was investigated in male rats by assessing the degree of plasma cholinesterase activity. PB, 60 mg/kg/day for 3 days, exerted no protective effect on the toxicity of fenitrothion (100 mg/kg, p.o.) given 24 h after the last injection. In adrenalectomized and SKF 525-A-pretreated rats, the toxicity of fenitrothion was lower than that of the controls. Fenitrothion toxicity was increased by administration of DEM (1 ml/kg), which depletes hepatic glutathione (GSH) levels. In vitro, the rates of fenitrothion decomposition and fenitrooxon formation by microsomes were markedly affected by PB, SKF 525-A and Adx. The decomposition of fenitrooxon by the microsomal fraction and GSH-dependent decomposition of fenitrooxon by the soluble fraction were not affected by PB, SKF 525-A and Adx pretreatment. The GSH-dependent decomposition of fenitrothion and fenitrooxon was increased by addition of GSH to the incubation mixture. The present results indicate that the GSH-dependent metabolic pathway plays an important role in the detoxication of fenitrothion.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1459
    Keywords: Adrenoleukodystrophy ; Adrenomyeloneuropathy ; Fatty acids ; High-performance liquid chromatography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Mittels Hochleistungs-Flüssigkeitschromatographie wurden durch Analyse der Fettsäuren in Sphingomyelien Adrenoleukodystrophien und Adrenomyeloneuropathien untersucht. Die oben erwähnte analytische Methode erwies sich als einfach, empfindlich und exakt. Bei der Adrenoleukodystrophie und bei Patienten mit Adrenomyeloneuropathie wurden sehr lange Fettsäureketten (C26:0, C25:0 und C24:0 auf der Basis von C22:0) sowohl in Plasma und den Erythrozytenmembranen als auch in Gesamtblutproben als vermehrt erkannt. Auch in Kulturen von Hautfibroblasten von Sphingomyelien wurde eine Zunahme der langkettigen Fettsäuren nachgewiesen. In einem Fall eines heterozygoten Adrenoleukodystrophiepatienten wurden Werte die zwischen jenen der homozygoten und der Kontrollfälle lagen gefunden. Somit ist die Hochleistungs-Flüssigkeitschromatographie eine brauchbare Methode, um eine Zunahme langkettiger Fettsäuren zwecks Diagnose einer Adrenoleukodystrophie oder einer Adrenomyeloneuropathie festzustellen.
    Notes: Summary Using high-performance liquid chromatography, adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AMN) were diagnosed by the analysis of fatty acids in sphingomyelin. The analytical method was simple, sensitive and accurate. In ALD and AMN patients, very long chain fatty acids (C26:0, C25:0 and C24:0 on the base of C22:0) were elevated not only in plasma and erythrocyte membranes but also in whole blood samples. An increase of long chain fatty acids was also shown in sphingomyelin of cultured skin fibroblasts. One heterozygote for ALD showed intermediate values between homozygotes and controls. Thus, high-performance liquid chromatography is a valuable method to detect increased long chain fatty acids for the diagnosis of ALD or AMN.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1459
    Keywords: Muscle atrophy, neurogenic ; β-Galactosidase deficiency ; β-Glucuronidase deficiency ; Dermatan sulfate ; Mucopolysaccharidosis ; Marfan syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei einem 18jährigen Jüngling hatten sich im Verlauf der Kindheit eine zunehmende geistige Retardierung, eine neurogene Muskelatrophie mit Hyperreflexie, Marfan-artige Besonderheiten und multiple Dysplasien der Epiphysen entwickelt. Im Harn fand sich eine vermehrte Ausscheidung von Dermatansulfat. Es wurde eine verminderte Aktivität lysosomaler Enzyme nachgewiesen, nämlich von β-Galactosidase, β-Glucuronidase und N-acetyl-β-d-Glucosaminidase.
    Notes: Summary An 18-year-old boy showed childhood onset of mental retardation, neurogenic muscle atrophy with hyperreflexia, Marfan-like features, multiple epiphyseal dysplasia, increased urinary excretion of dermatan sulfate, and decreased lysosomal enzyme activities in β-galactosidase, β-glucuronidase, and N-acetyl-β-d-glucosaminidase. This case may be a new syndrome, the combination of neurogenic muscle atrophy with lysosomal enzyme deficiencies.
    Type of Medium: Electronic Resource
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