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A review of 19 adult patients with alpha-1-antitrypsin deficiency (A1AT deficiency) and chronic liver disease revealed a late onset of symptomatic hepatic abnormalities in this condition. Thirteen patients (68%) were 60 years or older when the liver disease was discovered. The mean age of the patients with the ZZ, SZ, and MZ phenotypes was 58, 66, and 72.5 years, respectively; this suggested a later onset of the liver disease in the heterozygotes. At the time of diagnosis, the hepatic condition usually was advanced; in eight patients (42%) the survival was less than two years. The most important associated condition was chronic obstructive lung disease which was found in 10 patients (53%). We conclude that advanced age and the high incidence of obstructive lung disease make it unlikely that liver transplantation will become a common therapeutic option for adult patients with A1AT deficiency and associated liver disease. Periodic screening of liver function may be indicated in patients with A1AT deficiency so that chronic liver disease can be diagnosed early, particularly if current attempts to develop effective medical therapy for this condition are successful.

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Although many reports in the literature describe the use of endoscopic retrograde cholangiopancreatography (ERCP) in children, few mention the therapeutic application of the technique in treating pediatric patients with pancreatic or biliary pathology. We report here on our two-year experience, consisting of 42 ERCPs conducted in 25 patients for a variety of pancreaticobiliary indications. Ages ranged from 22 months to 19 years. Five patients had normal studies; the remainder were diagnosed with a variety of pancreatic and or biliary anomalies. Seventeen patients had therapeutic interventions consisting of sphincterotomy (n = 12), dilation (n = 6), or stent placement (n = 9), with many of these procedures being done in combination. Five patients had mild complaints of abdominal pain or vomiting immediately after ERCP, but no major technical or medical complications were encountered. We find that therapeutic ERCP can be safely performed in children, but only by highly experienced endoscopists familiar with these specialized procedures.

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