Guest guest Posted October 11, 2002 Report Share Posted October 11, 2002 Liver disease in pediatric patients with cystic fibrosis is associated with glutathione S-transferase P1 polymorphism Liver disease in patients with cystic fibrosis (CF) is inconstant and has not yet been clearly related to any specific risk factor. While the expression of cystic fibrosis transmembrane conductance regulator (CFTR) is restricted to the biliary epithelium in the liver, recent findings indicate that CFTR modulates reduced glutathione (GSH) transport and that CFTR dysfunction creates an imbalance in the antioxidant defense. Among liver detoxifying enzymes, the glutathione S-transferases (GSTs) play a key role in the protection against oxidative stress. Because oxidative injury contributes to the development of liver disease, we hypothesized that 2 members of the GST superfamily, GSTM1 and GSTP1, which are expressed in the biliary epithelium, could influence the hepatic status in patients with CF. The potential impact of GSTM1 and GSTP1 gene polymorphisms was assessed in 106 children with CF (mean age, 11.5 years). Based on polymerase chain reaction/restriction fragment length polymorphism analysis, we found that the frequency of GSTP1-Ile105/Ile105 genotype was significantly higher in patients with CF with liver disease than in those without (P < ..03). Among the youngest patients, aged 6 years, GSTP1-Ile105/Ile105 genotype was associated with an 8-fold increase in the risk of liver disease compared with other GSTP1 genotypes (P = .002). No association between the GSTM1 genotype and liver status was documented. In conclusion, GSTP1-Ile105 -encoding allele contributes to hepatic dysfunction in CF. Identification of this polymorphism may have prognostic value and prompt early treatment in patients with CF with an increased risk of liver disease. (HEPATOLOGY 2002;36:913-917.) October 2002, Volume 36, Number 4 Copyright © 2002 by the American Association for the Study of Liver Diseases. http://www.pulmonologylinx.com/thearts.cfm?artid=432143&specid=14&ok=yes Becki YOUR FAVORITE LilGooberGirl YOUNGLUNG EMAIL SUPPORT LIST www.topica.com/lists/younglung Pediatric Interstitial Lung Disease Society http://groups.yahoo.com/group/InterstitialLung_Kids/ Quote Link to comment Share on other sites More sharing options...
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