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B Vignes

Publications and source records attributed to B Vignes.

At least 19 recordsLinked to original sources

de Toni-Fanconi-Debré syndrome with Leigh syndrome revealing severe muscle cytochrome c oxidase deficiency.

We describe a patient with severe muscle cytochrome c oxidase deficiency who had de Toni-Fanconi-Debré syndrome and acute neurologic deterioration resembling Leigh syndrome, without clear evidence of muscle abnormality. Metabolic investigations revealed elevated cerebrospinal fluid lactate values contrasting with normal blood lactate, and high 3-hydroxybutyrate/acetoacetate ratio with normal lactate/pyruvate ratio. This case emphasizes the importance of performing metabolic and biochemical investigations in every patient with Leigh syndrome, even in the absence of hyperlactatemia or myopathy.

Brain Diseases, Metabolic

[Toxoplasmosis and lupus. A review of the literature apropos of 4 cases].

The authors report 4 cases of toxoplasmosis in patients with acute disseminated lupus erythematosus (ADLE). In one case, a pregnant patient with serology indicative of chronic infection, infected the neonate who died of subacute toxoplasmosis. Although ADLE is a classical cause of immunodepression, toxoplasmosis is a rare complication; only 5 cases were found in a review of the literature. Toxoplasmosis infection may resemble an exacerbation of lupus; an accurate diagnosis is essential as the treatment of the two conditions is radically different. The problems of diagnosis of toxoplasmosis in immunodepressed patients are reviewed and the therapeutic approach, especially in pregnant patients, is discussed. In ADLE, the authors recommend checking toxoplasmosis serology before starting and during treatment with corticosteroids. Special attention should be paid to pregnant women with apparently chronic serological changes as neonatal infection may occur.

Adolescent

[Smallpox].

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Nursing

[Diphtheria].

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Diphtheria

[Scarlet fever].

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Nursing

[Influenza].

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Humans

[Chickenpox].

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Chickenpox

[Tetanus].

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Nursing

[Mumps].

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Mumps