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Article about Links to Liver disease in CF

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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

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