Gastroparesis in neonatal myotonic dystrophy
โ Scribed by Dr. John B. Bodensteiner; Dr. John E. Grunow
- Publisher
- John Wiley and Sons
- Year
- 1984
- Tongue
- English
- Weight
- 210 KB
- Volume
- 7
- Category
- Article
- ISSN
- 0148-639X
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โฆ Synopsis
Abstract
Myotonic dystrophy in the neonate is commonly accompanied by facial diplegia, generalized muscular hypotonia, talipes equinovarus, and muscular respiratory failure. The gastrointestinal manifestations of this disease include poor sucking, choking, regurgitation, aspiration, and swallowing difficulties. Gastroparesis can be a major contributor to the feeding difficulties experienced by these infants. We report on an infant with congenital myotonic dystrophy in whom a severe gastric motility problem was alleviated by metoclopramide therapy. This smooth muscle manifestation may be an important and potentially remediable source of morbidity in these infants.
๐ SIMILAR VOLUMES
Myotonic dystrophy (DM) is a highly variable multisystemic disease belonging to the rather special class of trinucleotide expansion disorders. DM results from dynamic expansion of a perfect (CTG) n repeat situated in a gene-dense region on chromosome 19q. Based on findings in patient materials or ce