SKELETAL-MUSCLE WEAKNESS AND DYSPHAGIA CAUSED BY ACID MALTASE DEFICIENCY - NUTRITIONAL CONSEQUENCES OF COINCIDENT CELIAC SPRUE

Authors
KING TS ANDERSON JR WRAIGHT EP HUNTER JO COX TM
Citation
Ts. King et al., SKELETAL-MUSCLE WEAKNESS AND DYSPHAGIA CAUSED BY ACID MALTASE DEFICIENCY - NUTRITIONAL CONSEQUENCES OF COINCIDENT CELIAC SPRUE, JPEN. Journal of parenteral and enteral nutrition, 21(1), 1997, pp. 46-49
Citations number
20
Categorie Soggetti
Nutrition & Dietetics
Journal title
JPEN. Journal of parenteral and enteral nutrition → ACNP
ISSN journal
01486071
Volume
21
Issue
1
Year of publication
1997
Pages
46 - 49
Database
ISI
SICI code
0148-6071(1997)21:1<46:SWADCB>2.0.ZU;2-W
Abstract
Background: A 30-year-old woman with celiac sprue had progressive weig ht loss, myalgia, limb-girdle weakness, and dysphagia. Methods and Res ults: Barium swallow showed an atonic esophagus, and scintigraphic stu dy confirmed esophageal dysmotility. Skeletal muscle biopsy showed cha racteristic appearances of acid maltase deficiency, which was confirme d by a reduction of leukocyte acid alpha-glucosidase activity. Conclus ions: Nutritional factors may have accelerated the presentation of the lysosomal storage disorder. This is the first reported case of dyspha gia caused by esophageal motor weakness in acid maltase deficiency.