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Abstract. 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. [ABSTRACT FROM AUTHOR]

To determine whether the acidifying effect of malabsorbed carbohydrate on fecal pH differed between methane-excreting and nonexcreting individuals, we administered the poorly absorbed disaccharide lactulose to five CH4 excretors and six CH4 nonexcretors. Lactulose, 20 g twice daily for 1 wk, significantly lowered fecal pH among CH4 nonexcretors as compared with CH4-excreting individuals (5.38 ± 0.66 vs 6.90 ± 0.61, p < 0.01). To determine whether this observation was due to differences in acid production from bacterial carbohydrate fermentation, feces from each subject were incubated with lactulose. There were no differences in in vitro acid or hydrogen production between groups. We conclude that malabsorbed carbohydrate fails to induce in vivo colonic acidification in CH4 excretors. In contrast, in vitro fecal carbohydrate fermentation is similar among CH4 excretors and nonexcretors. These results suggest differences between these two groups in the colonic absorption of fermentation products. [ABSTRACT FROM AUTHOR]