PEDIATRICS Vol. 82 No. 1 July 1988, pp. 107-111
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Esophageal Motor Abnormalities in Children and Adolescents With Scleroderma and Mixed Connective Tissue Disease

Jonathan A. Flick MD1, John T. Boyle MD1, David N. Tuchman MD1, Balu H. Athreya MD1, and Robert A. Doughty MD1

1 From the Divisions of Gastroenterology and Nutrition, and Rheumatology, Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia

To determine the frequency and nature of esophageal motor abnormalities in children and adolescents with scleroderma syndromes and mixed connective tissue disease, esophageal manometry was performed on seven patients with progressive systemic sclerosis, four patients with mixed connective tissue disease, and two patients with linear scleroderma. A total of 73% of patients with progressive systemic sclerosis and mixed connective tissue disease had symptoms of esophageal dysfunction. A significant association between the presence of Raynaud phenomenon and esophageal symptoms was noted. Esophageal motor abnormalities were detected in 73% of patients with progressive systemic sclerosis and mixed connective tissue disease; these abnormalities were characterized by decreased lower esophageal sphincter pressure and abnormal peristalsis in the distal two thirds of the esophageal body. They resemble those described among adults with progressive systemic sclerosis and mixed connective tissue disease but were not related to disease duration or to the presence of Raynaud phenomenon. Patients with linear scleroderma did not have esophageal symptoms and demonstrated only nonspecific motor abnormalities that did not worsen during several years of follow-up.

Key Words: scleroderma • mixed connective tissue disease • Raynaud phenomenon

Submitted on July 30, 1987
Accepted on November 9, 1987




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