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Previous Volume 336:835-838 March 20, 1997 Number 12 Next

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ABSTRACT

Background Idiopathic adulthood ductopenia is a severe cholestatic liver disease of unknown cause characterized by loss of the interlobular bile ducts in more than 50 percent of the portal tracts. In most reported cases, cirrhosis and liver failure develop.

Methods We studied 24 adults with abnormal results on liver-function tests but no symptoms of liver disease. All had liver biopsies that showed a lack of bile ducts in many of the portal tracts.

Results The 17 women and 7 men had a mean age of 41 years (range, 27 to 57). All were asymptomatic and had high serum -glutamyltransferase concentrations (mean [±SD], 179±84 U per liter); 75 percent also had abnormal serum alanine aminotransferase concentrations. The proportion of portal tracts that had bile ducts was 62±7 percent (range, 55 to 78 percent). Three patients had a second liver biopsy three to nine years after the first; there were no changes over time. In four of the five patients treated with 600 to 900 mg of ursodiol two to three times daily, results of liver-function tests returned to normal.

Conclusions Idiopathic biliary ductopenia, with an apparently nonprogressive clinical course, can occur in adults who have no symptoms of biliary disease.

Source Information

From the Liver Unit, Hospital Ramón y Cajal (A.M., A.C.), and the Department of Hepatology, Fundación Jiménez Díaz (V.C., C.G.), both in Madrid.

Address reprint requests to Dr. Carreño at the Department of Hepatology, Fundación Jiménez Díaz, Avda. Reyes Católicos, 2, 28040 Madrid, Spain.

Full Text of this Article

Related Letters:

Idiopathic Biliary Ductopenia
Perez-Atayde A. R., Bartolomé J., Carreño V.
Extract | Full Text  
N Engl J Med 1997; 337:280-281, Jul 24, 1997. Correspondence

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• Khanlou, H., Sass, D., Rothstein, K., Manzarbeitia, C., Reich, D., Jacobson, L., Fleischer, D., Munoz, S. J. (2000). Idiopathic Adulthood Ductopenia: Case Report and Review of the Literature. Arch Intern Med 160: 1033-1036 [Abstract] [Full Text]  
• Perez-Atayde, A. R., Bartolome, J., Carreno, V. (1997). Idiopathic Biliary Ductopenia. NEJM 337: 280-281 [Full Text]