Balanços metabólicos do enxofre em pacientes com leucodistrofia metacromática

Canelas, Horacio M.; Jorge, Francisco B. de; Tognola, Waldir A.

doi:10.1590/S0004-282X1968000400003

Resumos

Foram estudados os balanços metabólicos do enxofre em dois pacientes com a forma juvenil da leucodistrofia metacromática. Foi verificado, em ambos os casos, um balanço positivo desse metaloide, aparentemente não influenciado pelo tipo de dieta (geral ou vegetal). Este resultado está de acordo com a atual concepção patogênica dessa moléstia, que consiste, essencialmente, em uma sulfatidose com diminuição da atividade das sulfatases.

The metabolic balances of sulfur in two cases of the late juvenile form of metachromatic leucodystrophy were studied. A positive balance of sulfur was found in both patients, apparently not influenced by the type of diet (either mixed or vegetarian). This finding is in accordance with the current views on the pathogenesis of the disease, namely a sulfatidosis with low sulfatase activity.

Metabolic balances of sulfur in patients with metachromatic leucodystrophy

Balanços metabólicos do enxofre em pacientes com leucodistrofia metacromática

Horacio M. Canelas^I; Francisco B. de Jorge^II; Waldir A. Tognola^III

^IAssociate Professor; Departments of Neurology and Medicine; University of São Paulo School of Medicine, São Paulo, Brazil

^IIChief of Laboratory; University of São Paulo School of Medicine, São Paulo, Brazil

^IIInstructor; Departments of Neurology and Medicine; University of São Paulo School of Medicine, São Paulo, Brazil

SUMMARY

The metabolic balances of sulfur in two cases of the late juvenile form of metachromatic leucodystrophy were studied. A positive balance of sulfur was found in both patients, apparently not influenced by the type of diet (either mixed or vegetarian).

This finding is in accordance with the current views on the pathogenesis of the disease, namely a sulfatidosis with low sulfatase activity.

RESUMO

Foram estudados os balanços metabólicos do enxofre em dois pacientes com a forma juvenil da leucodistrofia metacromática. Foi verificado, em ambos os casos, um balanço positivo desse metaloide, aparentemente não influenciado pelo tipo de dieta (geral ou vegetal).

Este resultado está de acordo com a atual concepção patogênica dessa moléstia, que consiste, essencialmente, em uma sulfatidose com diminuição da atividade das sulfatases.

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Departamento de Neurologia Faculdade de Medicina, Universidade de São Paulo Caixa Postal, 3461 São Paulo, SP Brasil.

1. AUSTIN, J. H. Metachromatic form of diffuse cerebral sclerosis. Diagnosis during life by urine sediment examination. Neurology (Minneapolis) 7:415-426, 1957.
2. AUSTIN, J. H. Metachromatic form of diffuse cerebral sclerosis. Significance of sulfatide and other lipid abnormalities in white matter and kidney. Neurology (Minneapolis) 10:470-483, 1960.
3. AUSTIN, J. H. Metachromatic form of diffuse cerebral sclerosis. Low sulfatase activity in the urine of nine living patients with metachromatic leukodystrophy (MLD). Arch. Neurol. (Chicago) 12:447-455, 1965.
4. AUSTIN, J. H. Metachromatic form of diffuse cerebral sclerosis. The nature and significance of low sulfatase activity: a controlled study of brain, liver and kidney in four patients with metachromatic leukodystrophy (MLD). Arch. Neurol. (Chicago) 13:593-614, 1965.
5. CANELAS, H. M.; ESCALANTE, O. D.; IRIYA, K. & De JORGE, F. B. Leucodistrofia metacromática: estudo clinico-laboratorial de cinco casos na mesma família. Rev. paul. Med. 63:355-376, 1963.
6. De JORDE, F. B. & CINTRA, A. B. U. Metabolismo do enxofre. Balanços metabólicos em adultos normais sob dieta geral e sob dieta exclusivamente vegetal. Rev. paul. Med. 69:25-30, 1966.
7. De JORGE, F. B.; SILVA, A. G. & CINTRA, A B. U. Determinaçăo quantitativa do enxofre nos materiais biológicos. Rev. bras. Med. 21:491-494, 1964.
8. JATZKEWITZ, H. The role of cerebroside sulphuric esters in leucodystrophy and a new method for the quantitative ultramicrodetermination of the brain sphingolipids. In J. Folch-Pi & H. J. Bauer: Brain Lipids and Lipoproteins, and the Leucodystrophies, pp. 147-152. Elsevier, Amsterdam, 1963.
9. MOSER, H. W.; MOSER, A. B. & McKHANN, G. M. The dynamics of a lipidosis: turnover of sulfatide, steroid sulfate, and polysaccharide sulfate in metachromatic leukodystrophy. Arch. Neurol. (Chicago) 17:494-511, 1967.
10. SVENNERHOLM, L. Some aspects of the biochemical changes in leucodystrophy. InJ. Folch-Pi & H. J. Bauer: Brain Lipids and Lipoproteins, and the Leucodystrophies, pp. 104-119. Elsevier, Amsterdam, 1963.

Datas de Publicação

Publicação nesta coleção
13 Maio 2013
Data do Fascículo
Dez 1968

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. AUSTIN, J. H. Metachromatic form of diffuse cerebral sclerosis. Diagnosis during life by urine sediment examination. Neurology (Minneapolis) 7:415-426, 1957.

[2] 2. AUSTIN, J. H. Metachromatic form of diffuse cerebral sclerosis. Significance of sulfatide and other lipid abnormalities in white matter and kidney. Neurology (Minneapolis) 10:470-483, 1960.

[3] 3. AUSTIN, J. H. Metachromatic form of diffuse cerebral sclerosis. Low sulfatase activity in the urine of nine living patients with metachromatic leukodystrophy (MLD). Arch. Neurol. (Chicago) 12:447-455, 1965.

[4] 4. AUSTIN, J. H. Metachromatic form of diffuse cerebral sclerosis. The nature and significance of low sulfatase activity: a controlled study of brain, liver and kidney in four patients with metachromatic leukodystrophy (MLD). Arch. Neurol. (Chicago) 13:593-614, 1965.

[5] 5. CANELAS, H. M.; ESCALANTE, O. D.; IRIYA, K. & De JORGE, F. B. Leucodistrofia metacromática: estudo clinico-laboratorial de cinco casos na mesma família. Rev. paul. Med. 63:355-376, 1963.

[6] 6. De JORDE, F. B. & CINTRA, A. B. U. Metabolismo do enxofre. Balanços metabólicos em adultos normais sob dieta geral e sob dieta exclusivamente vegetal. Rev. paul. Med. 69:25-30, 1966.

[7] 7. De JORGE, F. B.; SILVA, A. G. & CINTRA, A B. U. Determinaçăo quantitativa do enxofre nos materiais biológicos. Rev. bras. Med. 21:491-494, 1964.

[8] 8. JATZKEWITZ, H. The role of cerebroside sulphuric esters in leucodystrophy and a new method for the quantitative ultramicrodetermination of the brain sphingolipids. In J. Folch-Pi & H. J. Bauer: Brain Lipids and Lipoproteins, and the Leucodystrophies, pp. 147-152. Elsevier, Amsterdam, 1963.

[9] 9. MOSER, H. W.; MOSER, A. B. & McKHANN, G. M. The dynamics of a lipidosis: turnover of sulfatide, steroid sulfate, and polysaccharide sulfate in metachromatic leukodystrophy. Arch. Neurol. (Chicago) 17:494-511, 1967.

[10] 10. SVENNERHOLM, L. Some aspects of the biochemical changes in leucodystrophy. InJ. Folch-Pi & H. J. Bauer: Brain Lipids and Lipoproteins, and the Leucodystrophies, pp. 104-119. Elsevier, Amsterdam, 1963.