Long-term treatment of primary sclerosing cholangitis in children with oral vancomycin: an immunomodulating antibiotic.

BACKGROUND: Primary sclerosing cholangitis is a rare chronic cholestatic condition of unknown etiology, frequently associated with inflammatory bowel disease and characterized by diffuse fibrosing and inflammatory destruction of the intra- and/or extrahepatic biliary duct system. PATIENTS AND METHODS: The study involved 14 children with primary sclerosing cholangitis confirmed by either liver biopsy, endoscopic retrograde cholangiopancreatography, and/or magnetic resonance cholangiogram. In each of the 14 cases, liver histology showed characteristic features consistent with primary sclerosing cholangitis. Eleven children had intrahepatic biliary beading and strictures (6 by endoscopic retrograde cholangiopancreatography; 5 by magnetic resonance cholangiogram). Biochemical tests of liver function including alanine aminotransferase, aspartate aminotransferase, and gamma-glutamyl transpeptidase and the erythrocyte sedimentation rate were elevated for a mean 17 +/- 22 months before vancomycin treatment was initiated. All of the patients were shown to have inflammatory bowel disease histologically; 13 of those patients had clinical evidence of colitis. Oral vancomycin was given to all 14 patients. RESULTS: All 14 patients showed improvement in their alanine aminotransferase (P = 0.007), gamma-glutamyl transpeptidase (P = 0.005), erythrocyte sedimentation rate (P = 0.008), and clinical symptoms with oral vancomycin treatment. There was less improvement noted in the patients with cirrhosis when compared with the patients without cirrhosis. CONCLUSIONS: Before this study, there has not been an effective long-term treatment for sclerosing cholangitis to prevent the usual progression of this disease to cirrhosis. This study showed that oral vancomycin could be an effective long-term treatment of sclerosing cholangitis in children, especially those without cirrhosis.

The role of esophagogastroduodenoscopy in the initial evaluation of childhood inflammatory bowel disease: a 7-year study.

OBJECTIVES: To assess the role of esophagogastroduodenoscopy in the evaluation of children with suspected inflammatory bowel disease. METHODS: All children with inflammatory bowel disease who underwent esophagogastroduodenoscopy during their initial evaluation at our institution during a 7-year period (December 1993 to November 2000) were included in the study. RESULTS: The study included 115 patients: 81 with Crohn disease (mean age, 11.34 years; 42 males) and 34 with ulcerative colitis (mean age, 11.79 years; 20 males). Abnormal findings on esophagogastroduodenoscopy were noted in 64% of patients with Crohn disease and 50% of children with ulcerative colitis; histologic abnormalities were found in 81.6% and 70.6% of the patients, respectively. Granulomas were found in the upper gastrointestinal tracts of 23 of 81 patients (28.4%), with the most common site being the gastric mucosa. Nine of these 23 patients had granulomas solely in the upper gastrointestinal tract. Additional unsuspected pathology noted included: candidiasis, hiatal hernia, Helicobacter pylori infection, and giardiasis. CONCLUSIONS: Endoscopic and histologic abnormalities were found in the upper gastrointestinal tracts of a significant number of children with inflammatory bowel disease. While the mechanism(s) underlying these abnormalities in patients with ulcerative colitis is unclear, the pathology can contribute to the patient's clinical condition. Pathology in the upper gastrointestinal tract should not exclude a diagnosis of ulcerative colitis. Granulomas, confirming the diagnosis of Crohn disease, were found in the upper gastrointestinal tracts of 28% of our patients with Crohn disease. In some cases, granulomas were found solely in the upper gastrointestinal tracts. Based on our data, esophagogastroduodenoscopy with biopsy should be performed in all pediatric patients with suspected inflammatory bowel disease.