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Mild idiopathic biliary ductopenia: lack of familial clustering
Correspondence
References 1. Nishizawa T, Okamoto H, Konishi K, et al. A novel DNA virus (TTV) associated with elevated transaminase levels in posttransfusion hepatitis of unknown etiology. Biochem Biophys Res Commun 1997; 241: 92-l.
2. Okamoto
H, Nishizawa T, Kato N, et al. Molecular cloning and characterization of a novel DNA virus (TTY) associated with posttransfusion hepatitis of unknown etiology. Hepatol Res 1998; 10: 1-16.
Mild idiopathic biliary ductopenia: lack of familial clustering To the Editor:
Idiopathic adulthood ductopenia has been described as a chronic cholestatic liver disease with severe fibrosis or cirrhosis (1). We have recently described a mild form of idiopathic biliary ductopenia in adults without liver disease symptoms (2). These patients have abnormal liver-function test values and all the known causes of liver disease have been excluded. The liver histologic finding was a loss of bile ducts in less than 50% of the portal tracts, with no other cholestatic or inflammatory aspects. There was no correlation between the severity of the disease and the sex or the age of the patients. Classic idiopathic adulthood ductopenia is a severe and progressive disease dissimilar to the mild form of idiopathic biliary ductopenia, in which the disease course seems to be benign. The etiology of this disease remains to be clarified, and whether a family cluster exists. In order to determine whether this mild form of idiopathic biliary ductopenia is intrafamilial, we studied a total of 62 relatives of 18 patients who had agreed to participate in the study: 19 parents, 19 offspring and 24 siblings, with a mean age of 45? 10 years and representing a mean of 3.421.6 relatives per patient (range 1 to 7). A routine liver function test was performed in these 62 relatives. Abnormal liver-function test results were found in only four cases; however, a known cause was demonstrated in all of these. In this context, the son of one patient had a slight increase in alanine aminotransferase with relation to hepatitis C virus infection. The mother of one patient and the parents of another presented elevated levels of gamma-glutamyltransferase; the first case was an epileptic woman who is presently undergoing treatment with phenytoin, which is a known hepatotoxic drug (3). The parents of the second patient had increased cholesterol values. It is therefore probable that the elevated level of gamma-glutamyltransferase is due to a metabolic alteration (4,5). The remaining patient family members (58 subjects) had normal
values of serum alanine and aspartate aminotransferases, or gammaglutamyltransferase. In conclusion, in our study we have observed that patients with mild idiopathic biliary ductopenia, in whom abnormal results of a liver-function test have an unknown etiology, do not have relatives with an unjustified abnormal liver-function test. Therefore, it would not appear that this mild form of idiopathic biliary ductopenia has an intrafamilial association. Margarita Pardo, Inmaculada Castillo and Vicente Carreiio Department of Hepatology, para el Estudio de Hepatitis
Fundacidn Jim&e2 Diaz and Fundacibn Virales, Madrid, Spain
References 1. Ludwig J, Wiesner RH, LaRusso NE Idiopathic adulthood ductopenia: a cause of chronic cholestatic liver disease and biliary cirrhosis. J Hepatol 1988; 7: 193-9. 2. Moreno A, Carreiio V, Cano A, Gonzalez C. Idiopathic biliary ductopenia in adults without symptoms of liver disease. N Engl J Med 1997; 336: 835-8. 3. Haruda E Phenytoin hypersensitivity: 38 cases. Neurology 1979; 29: 1480-5. 4. Pintus F, Mascia P Distribution and population determinant of gamma-glutamyltransferase in a random sample of Sardinian inhabitants. ATS-SARDEGNA Research Group. Eur J Epidemiol 1996; 12: 71-6. 5. Poikolainen K, Vartiainen E. Determinant of gamma-glutamyltransferase: positive interaction with alcohol and body mass index, negative association with coffee. Am J Epidemiol 1997; 146: 1019-24.
685
References 1. Nishizawa T, Okamoto H, Konishi K, et al. A novel DNA virus (TTV) associated with elevated transaminase levels in posttransfusion hepatitis of unknown etiology. Biochem Biophys Res Commun 1997; 241: 92-l.
2. Okamoto
H, Nishizawa T, Kato N, et al. Molecular cloning and characterization of a novel DNA virus (TTY) associated with posttransfusion hepatitis of unknown etiology. Hepatol Res 1998; 10: 1-16.
Mild idiopathic biliary ductopenia: lack of familial clustering To the Editor:
Idiopathic adulthood ductopenia has been described as a chronic cholestatic liver disease with severe fibrosis or cirrhosis (1). We have recently described a mild form of idiopathic biliary ductopenia in adults without liver disease symptoms (2). These patients have abnormal liver-function test values and all the known causes of liver disease have been excluded. The liver histologic finding was a loss of bile ducts in less than 50% of the portal tracts, with no other cholestatic or inflammatory aspects. There was no correlation between the severity of the disease and the sex or the age of the patients. Classic idiopathic adulthood ductopenia is a severe and progressive disease dissimilar to the mild form of idiopathic biliary ductopenia, in which the disease course seems to be benign. The etiology of this disease remains to be clarified, and whether a family cluster exists. In order to determine whether this mild form of idiopathic biliary ductopenia is intrafamilial, we studied a total of 62 relatives of 18 patients who had agreed to participate in the study: 19 parents, 19 offspring and 24 siblings, with a mean age of 45? 10 years and representing a mean of 3.421.6 relatives per patient (range 1 to 7). A routine liver function test was performed in these 62 relatives. Abnormal liver-function test results were found in only four cases; however, a known cause was demonstrated in all of these. In this context, the son of one patient had a slight increase in alanine aminotransferase with relation to hepatitis C virus infection. The mother of one patient and the parents of another presented elevated levels of gamma-glutamyltransferase; the first case was an epileptic woman who is presently undergoing treatment with phenytoin, which is a known hepatotoxic drug (3). The parents of the second patient had increased cholesterol values. It is therefore probable that the elevated level of gamma-glutamyltransferase is due to a metabolic alteration (4,5). The remaining patient family members (58 subjects) had normal
values of serum alanine and aspartate aminotransferases, or gammaglutamyltransferase. In conclusion, in our study we have observed that patients with mild idiopathic biliary ductopenia, in whom abnormal results of a liver-function test have an unknown etiology, do not have relatives with an unjustified abnormal liver-function test. Therefore, it would not appear that this mild form of idiopathic biliary ductopenia has an intrafamilial association. Margarita Pardo, Inmaculada Castillo and Vicente Carreiio Department of Hepatology, para el Estudio de Hepatitis
Fundacidn Jim&e2 Diaz and Fundacibn Virales, Madrid, Spain
References 1. Ludwig J, Wiesner RH, LaRusso NE Idiopathic adulthood ductopenia: a cause of chronic cholestatic liver disease and biliary cirrhosis. J Hepatol 1988; 7: 193-9. 2. Moreno A, Carreiio V, Cano A, Gonzalez C. Idiopathic biliary ductopenia in adults without symptoms of liver disease. N Engl J Med 1997; 336: 835-8. 3. Haruda E Phenytoin hypersensitivity: 38 cases. Neurology 1979; 29: 1480-5. 4. Pintus F, Mascia P Distribution and population determinant of gamma-glutamyltransferase in a random sample of Sardinian inhabitants. ATS-SARDEGNA Research Group. Eur J Epidemiol 1996; 12: 71-6. 5. Poikolainen K, Vartiainen E. Determinant of gamma-glutamyltransferase: positive interaction with alcohol and body mass index, negative association with coffee. Am J Epidemiol 1997; 146: 1019-24.
685