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Encoprésie révélatrice d’une dystrophie myotonique de Steinert : à propos de 2 observations
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- Author(s): Avez-Couturier, J.1; Michaud, L.1 ; Cuisset, J.-M.2; Lamblin, M.-D.3; Dolhem, P.4; Turck, D.1; Vallée, L.2; Gottrand, F.1
- Source:
Archives de Pédiatrie. May2009, Vol. 16 Issue 5, p430-434. 5p.- Subject Terms:
- Source:
- Additional Information
- Alternate Title: Encopresis revealing myotonic dystrophy in 2 children
- Abstract: Summary: Gastrointestinal symptoms are very frequent in myotonic dystrophy but largely unrecognized. They can be the revealing factors of the disease. We report 2 cases of 10 and 17-year-old children with persistent encopresis starting at the age of 3 and 5 years in spite of laxative treatment. Neurological examination and anorectal manometry provided the diagnosis of myotonic dystrophy. Procainamide treatment was introduced and the digestive symptoms improved. Any child with encopresis should have complete evaluation to rule out the diagnosis of myotonic dystrophy and physicians should look for upper and/or lower gastrointestinal symptoms in every patient with myotonic dystrophy. [Copyright &y& Elsevier]
- Abstract: Résumé: Les signes digestifs de la dystrophie myotonique de Steinert sont fréquents mais peu connus. Ils peuvent être le mode de révélation de la maladie. Nous rapportons les observations de 2 enfants âgés de 10 et 17ans qui avaient consulté pour une encoprésie qui persistait, malgré les traitements médicaux appropriés. L’examen neurologique, ainsi que la manométrie anorectale ont permis de faire le diagnostic de dystrophie myotonique de Steinert, puis de débuter un traitement par procaïnamide, qui a permis de diminuer les phénomènes myotoniques et d’améliorer l’encoprésie. Le diagnostic de cette affection doit être évoqué chez tout enfant présentant une encoprésie associée à une béance anale ou à un retard mental. Il est également important de rechercher régulièrement des signes d’atteinte digestive haute ou basse chez ces patients.
- Abstract:
Copyright of Archives de Pédiatrie is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.) - Abstract:
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