Abstract
The malabsorption associated with chronic liver disease was studied in 23 children, all except two of whom had evidence of cirrhosis. There were 14 patients with extrahepatic biliary atresia, four with "paucity of intrahepatic bile ducts," and five with post-hepatitic cirrhosis. Steatorrhea (> 5 gm /24 hr) was documented in the 14 patients tested while on a normal diet. No evidence of pancreatic or small bowel mucosal disease could be found. While basal duodenal bile salt concentrations were found to be below the critical micellar concentration (CMC) in only two of the four cases of post-hepatitic cirrhosis tested, a poor response to pancreozymin clearly separated all the four from normal control subjects. The 10 children with end stage cirrhosis excreted significantly (P < .001) more fat (21.4 ± 6.6) in their stools than did the others (10.9 ± 2.1). Even though a medium chain triglyceride (MCT) supplemented low fat diet was associated with a drop in fecal fat, it had no effect on the severe malnutrition of decompensated extrahepatic biliary atresia; whereas, in conjunction with cholestyramine, it led to a resumption of weight gain and a concomitant amelioration of liver function in patients with "paucity of intrahepatic bile ducts" and in those with post-hepatitic cirrhosis.
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